Boards Flashcards

1
Q

Hereditary angioedema is the result of:

A

C1-INH esterase deficiency

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2
Q

Chipmunk facies

A

Beta thalassemia

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3
Q

Name the procedure for removing thyroglossal duct cyst

A

sistrunk procedure

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4
Q

DX?

Syndromic association?

A

Hollman miller sign: Anterior bowing of the posterior wall of the maxillary sinus that occurs as a result of nasopharyngeal angiofibroma

Can be associated with Gardner’s syndrome

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5
Q

Conditions that cause elevated vanillylmandelic acid (VMA) (3)

A

*note ALL neuroectodermally derived 1. Melanotic neuroectodermal tumor of infancy 2. Olfactory neuroblastoma 3. Pheochromocytoma

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6
Q

Burton line

A

Bluish pigmentation of the marginal gingiva that results from lead poisoning

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7
Q

3 Fun facts about chordomas

A
  1. most often seen involving the clivus
  2. contains physaliferous cells
  3. stains positive with brachyury
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8
Q

Dermatofibroma staining vs dermatofibrosarcoma protuberans (DFSP)

A

DF: CD34- F13A+ DFSP: CD34+ F13A -

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9
Q

Intestinal type sinonasal adenocarcinoma is associated with what professions

A

leather and woodworking

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10
Q

What are 4 risk factors of sinonasal adenocarcinoma (not intestinal type)

A
  1. Southeast Asia
  2. EBV infection
  3. high salt fish diet (chinese male population greatly affected)
  4. vitamin C deficiency
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11
Q

Warthin-Finkeldey giant cells are seen in what 5 conditions?

A
  1. measles
  2. lymphoma
  3. Kimura disease
  4. AIDs associated lymphoproliferative disorders
  5. Lupus erythematosus
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12
Q

DX?

Name the two cell types indicated by the arrows

What is the causitive organism?

A

Rhinoscleroma caused by : Klebsiella rhinoscleromatis (gram negative bacteria)

Mikulicz cells(foamy histiocyte), Russell bodies (eosinophilic inclusions in plasma cells)

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13
Q

Depicted entity:

  • Chronic and localized infection of the mucus membranes
  • Lesions present clinically as polypoid, soft masses (sometimes pedunculated) of the nose, throat, ear, and even the genitalia in both sexes.

What is the infectous organism?

A

Rhinosporiodosis- caused by Rhinosporidium seeberi (seems to be a fungus when you google it) -clinical presentation is an enlarged nostril/end of the nose

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14
Q

Ascher syndrome features (3)

A
  1. Double lip 2. Goiter 3. upper eyelid edema (blepharochalasia)
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15
Q

Paramedian lip pit associated syndromes (3)

A
  1. Van der woude syndrome 2. Kabuki syndrome 3. Popliteal pterygium syndrome
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16
Q

Premature exfoliation of the mandibular incisors may be seen in

A

hypophosphatasia

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17
Q

Beaten metal/copper skull is seen in which 3 conditions?

A

-Crouzan Syndrome (beaten-metal) -Apert syndrome (beaten metal) -Hypophosphatasia (beaten-copper)

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18
Q

“Teeth floating in air” + skin plantar/palmar hyperkeratosis

A

Papilon-Lefevre syndrome

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19
Q

Name that condition: Vertical bone trabeculae, Missing premolar, Retained primary molar

-List 2 other clinical findings

A

Segmental odontomaxillary dysplasia

Gingival overgrowth in the area of missing teeth

Becker nevus: hyperpigmentation with hypertrichosis

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20
Q

Ghost teeth on radiology enameloid conglomerates on histo

A

regional odontodysplasia

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21
Q

Black coated tongue cause

A

Bismuth staining

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22
Q

Skull radiology: best diagnosis for punched out radiolucent lesions in a child vs an older adult

A

child: LCH adult: multiple myeloma

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23
Q
A

Hydroxychloroquine pigmentation (antimalarial medication also given to lupus patients)

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24
Q

Lateral Calcifications seen on PAN DDX (5)

A
  1. tonsilith
  2. sialolith
  3. phlebolith
  4. dystrophic acne
  5. calcified lymph node
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25
Q

Globodontia (aka globe shaped teeth), hearing loss, and ocular colobomas are classic of what syndrome?

A

otodental syndrome

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26
Q

Papillary lesion of the soft palate DDX

A
  1. squamous papilloma 2. verruciform xanthoma 3. Giant cell fibroma 4. Sialadenoma papilliferum
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27
Q

Munro’s abscesses

A

psoriasis

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28
Q

Pautriers abscesses

A

Mycosis Fungoides

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29
Q

HLA-Cw6

A

psoriasis

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30
Q

Tissue culture pattern on histology

A

nodular fasciitis

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31
Q

DDX for mixed radiolucent-radiopaque lesion (6) (of course site and appearance play a factor)

A

CEOT

Ossifying fibroma

COC

odontoma

ameloblastic fibro-odontoma

AOT

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32
Q

Late stage florid COD- how do you rule out Pagets disease?

A

There should be no expansion in COD and ALK phos levels are normal (elevated in Pagets)

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33
Q

variant of ameloblastoma with mixed radiolucent/radiopaque appearance that resembles BFOL

A

desmoplastic ameloblastoma

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34
Q

A patient is diagnosed with basal cell carcinoma of the gingiva, what is the more likely diagnosis

A

a misdiagnosed ameloblastoma

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35
Q

-unerupted tooth, usually canine -radiolucency frequently extends below the CEJ of the tooth -snowflake calcifications

A

AOT

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36
Q

PTCH1 gene mutation occurs in what 2 entities?

A

OKC/Gorlin syndrome and CEOT

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37
Q

driven snow calcification appearance

A

CEOT

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38
Q

Amyloid-like acellular amorphous material occurring in CEOT is what?

A

odontogenic ameloblast-associated protein (ODAM)

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39
Q

Clear cell histology DDX (5)

A
  1. Clear cell odontogenic carcinoma
  2. hyalinizing clear cell carcinoma
  3. Intraosseous mucoep
  4. clear cell variant of CEOT
  5. Metastatic dz: renal, breast, melanoma
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40
Q

A defect or groove in the palate may be caused by what odontogenic neoplasm

A

central odontogenic fibroma

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41
Q

Ground substance in an odontogenic myxoma is composed of

A

GAGs: predominantly hyaluronic acid and chondroitin sulfate

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42
Q

Clinical hallmarks: blue sclerae, hearing loss, joint hyperextensibility, contractures, triangular facies, frontal bossing, macrocephaly, flattened vertex and skull base, prominent occiput (back of the head)

3 conditions with blue sclera?

A

Osteogenesis imperfecta (brittle bone disease)

-Osteogenesis imperfecta, ehlers-danlos, marfan syndrome

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43
Q

4 Radiographic hallmarks of osteogenesis imperfecta

A
  1. osteopenia
  2. bowing of long bones
  3. multiple fractures
  4. increased number of wormian bones
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44
Q

Increased number of intrasutural bones (wormian bones) in what two conditions

A

-cleidocranial dysplasia -osteogenesis imperfecta

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45
Q

What disease: Spontaneous and progressive destruction of 1 or more bones (give the 5 names please)

What are the histo findings?

A

1.massive osteolysis, 2.vanishing bone disesae, 3.gorham disease, 4.gorham-stout disease, 5.phantom bone disease

Histo: Destroyed bone –> vascular proliferation first → dense fibrous tissue

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46
Q

Many basophilic reversal lines within bone creating a characteristic jigsaw puzzle/mosaic appearance is characteristic histology for what?

A

paget’s disease (osteitis deformans)

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47
Q

10 disorders with Multiple giant cell lesions (6 high yield)…What 2 entities have been associated with central giant cell granulomas in the jaw?

A
  • Cherubism
  • Noonan-like/multiple giant cell lesion syndrome
  • noonan syndrome
  • ramon syndrome
  • jaffe-campanacci syndrome

neurofibromatosis type 1

  • schimmelpenning syndrome
  • oculo-ectodermal syndrome

-pagets disease (giant cell tumors)

-hyperparathyroidism (brown tumors)

JAW lesions assoc w central giant cell granulomas:

  1. Aneurysmal bone cyst
  2. central odontogenic fibromas
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48
Q

What cell lines are affected by early post zygotic activating mutation in GNAS

A

-Melanocytes, endocrine cells, osteoblasts *if late in embryogenesis only osteoblasts are affected

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49
Q

Monostotic fibrous dysplasia that affects the maxilla and adjacent bones is termed

A

cranio-facial fibrous dysplasia

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50
Q

3 Syndromes associated with polyostotic fibrous dysplasia

A
  1. Jaffe-lichtenstein
  2. Mazabraud
  3. McCune Albright
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51
Q

early presenting sign of polyostotic fibrous dysplasia is pain and long bone deformity- the deformity is specifically called what? (3 names)

A

deformity of the proximal femur = shepards crook deformity, coxa vara, hockey stick deformity (causes leg bowing)

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52
Q

Clinical: anterior mandibular COD with progressive growth

A

expansive osseous dysplasia

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53
Q

WHat diseasE?

  • Diffuse fibro-osseous lesions of the jaws with prominent psammoma bodies
  • bone fragility
  • bowing/cortical sclerosis of the long bones
A

Gnatho-diaphyseal dysplasia GDD1 mutation

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54
Q

What disease?

  • Parathyroid adenoma or carcinoma
  • Ossifying fibromas of the jaws
  • Renal cysts
  • Wilms tumors
A

Hyperparathyroidism jaw tumor syndrome HRPT2 mutation

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55
Q

-Multifocal osteomas -Supernumerary teeth, impacted teeth, odontomas -adenomatous polyps

A

Gardner’s syndrome APC gene chromo 5

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56
Q

Name 4 less frequently mentioned manifestations of Gardners syndrome

A
  1. desmoid tumors- usually at the abdominal scar left after the patient receives colectomy
  2. pigmented lesions of the ocular fundus: congenital hypertrophy of the retinal pigment epithelium
  3. epidermoid cysts
  4. thyroid carcinoma, pancreatic adeno, etc
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57
Q

-Radiology: radiolucent nidus with possible central opacity “target-like” -Clinical: nocturnal pain relieved by NSAIDs

A

osteoid osteoma (should also be less than 1.5-2cm in size)

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58
Q

Clinical: Genital lesions, conjunctivitis, arthritis, geographic tongue What condition? What HLA? Etiology?

A

-Reiter’s syndrome (reactive arthritis) -HLA-B27 -occurs following venereal disease or dysentery

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59
Q

Multiple chondromas are associated with what 2 diseases/syndromes

A
  1. Ollier disease: sporadic chondromatosis with unilateral tendency
  2. Maffucci syndrome: sporadic chondromatosis and soft tissue angiomas
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60
Q

What syndromes increase the risk for osteosarcoma

A

Paget’s disease

Li-fraumeni syndrome

Hereditary retinoblastoma

Rothmund-thomson syndrome

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61
Q

19 year old male

Bx: Non specific vascular proliferation with fibrous CT and chronic inflammatory cells

A

Massive osteolysis

Vanishing bone disease

phantom bone disease

gorham/gorham-stout disease

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62
Q

25 year old male

HX of veneral disease and arthritis

What is the term for associated genital lesions?

associated HLA?

A

Balanitis circinata

HLA-B27

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63
Q

5 year old female

Other findings: Bone surrounding the teeth is lower denisty, hyperplasia of the soft tissue in the area

DX?

Histo findings?

A

Regional odontodysplasia (ghost teeth)

Histo: enameloid conglomerates (also seen in amelogenesis imperfecta)

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64
Q

Clinical: 20 year old female; Tower skull(acrobrachycephaly) appearance

  • Diagnosis?
  • What is this image depicting?
  • In more severe cases of this syndrome what type of skull deformity may occur?

-

A
  • Apert syndrome
  • Syndactyly
  • Cloverleaf skull
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65
Q

55 year old male recently with new diagnosis and management of HTN

  • DDX?
  • What medications may cause this presentation?
A
  • Medication related gingival hyperplasia vs Leukemic infiltrate
  • (anti-epileptic)Phenyltoin, (immunosuppressant) cyclosporine, calcium channel blockers (amlodipine)
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66
Q

12 year old male

What is the most likely diagnosis?

What are these lesions?

List all of the manifestations of this disease

Mutation?

A

MEN2B

Mucosal neuromas

  • Marfanoid body habitus, pheochromocytoma, medullary thyroid carcinoma
  • 95% germline mutation at codon 918(M918T) of RET proto-oncogene (smaller subset have A883F)
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67
Q
  • What is the term for the lesion shown?
  • What % of patients with this lesion have an associated syndrome?
  • What syndrome?
  • What mutation and inheritance pattern?
  • What other lesions would a patient with this syndrome have?
A
  • Port wine stain (nevus flammeus)
  • 8-10%
  • Sturge Weber syndrome
  • GNAQ chromo 9; not inherited
  • Leptomeningeal angiomas, intellectual disability, contralateral hemiplegia, seizures
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68
Q
  • Most likely diagnosis?
  • What is the cause of the lesions?
  • Associated genetics?
  • Histologic buzzword?
A
  • multifocal epithelial hyperplasia (Heck’s disease)
  • HPV 13 and 32
  • HLA DR4
  • Mitosoid cell
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69
Q

5 year old male with multiple rough itchy papules

  • Diagnosis?
  • Etiology?
  • Patients with what 3 conditions are prone to prolonged or florid disease?
  • Histo buzz words?
A
  • Molluscum contagiosum
  • poxvirus infection MCV
  • HIV/AIDS, darier disease, atopic dermatitis
  • Henderson paterson bodies
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70
Q
  • What condition is the image depicting?
  • Associated somatic mutation? (what other lesion has the same one?)
  • If similar appearing lesions were throughout the body with associated pruritis what might you be concerned for?
  • If the patient scratched one of these lesons before biopsy what is the charactertistic term for the histologic appearance?
A
  • Dermatosis papulosa nigra
  • FGFR3, PIK3CA (actinic lentigo)
  • Leser-Trelat sign: rare phenomenon associated with internal malignancy
  • Irritated sebK aka Inverted follicular keratosis of Helwig
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71
Q

Biopsy of one of these lesions shows sebaceous hyperplasia

  • Name the associated syndrome
  • Name 3 other findings in this syndrome
A

Muirre-Torre Syndrome

-visceral malignancies, sebaceous adenomas and carcinomas (shown in the image), keratoacanthomas

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72
Q

What is the most likely diagnosis?

  • What other manifestations of disease would you expect?
  • If the condition was limited to skin manifestations what is your DDX
A
  • Noonan syndrome with multiple lentigenes (formerly LEOPARD syndrome)
  • L:multiple lentigenes, E:electrocardiographic conduction defects, O: Ocular hypertelorism, P: pulmonary stenosis, A: abnormalities of the genitalia, R)etarded (slowed) growth resulting in short stature, D: deafness
  • DDX: Lentiginosis profusa, Peutz Jeghers, LEOPARD
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73
Q
  • The lesion seen is fully excised, what is the recurrence rate?
  • what if it was verrucous?
  • Transformation potential is this is mild vs moderate vs severe?
A

-10-35% for typical leukoplakia, 83% for verruciform variants

Mild: ~1-7%

Moderate: ~4-11%

Severe: 20-43%

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74
Q

This patient has been using smokeless tobacco for 10 years.

  • What histologic features would be seen on biopsy?
  • Clinical recommendations?
A
  1. hyperkeratotic and acanthotic
    - intraceullar vacuoles
    - parakeratin chevrons
    - amorphous subepithelial material in the superficial CT

*dysplasia is uncommon

  1. Habit cessation- lesion should go away in ~ 2 weeks, if persists 6 weeks+ then it should be biopsied
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75
Q
  • Name 3 inherited syndromes associated with multiple of the lesion depicted in this image
  • Name one acquired cause
A

Multiple keratoacanthomas:

Inherited:

Muir-Torre (sebaceous adenomas/carcinomas, KAs, GI carcinoma)

Ferguson-Smith syndrome (self-healing squamous epitheliomas, many KAs, Scottish descent)

Witten-Zak syndrome, multiple familial keratoacanthoma

Acquired:

Grybowski syndrome (hundreds/thousands small papules skin/GI– may be associated with internal malignancy)

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76
Q

Multiple basal cell carcinomas are common in what inherited genetic disorders?

A
  1. nevoid basal cell carcinoma syndrome
  2. xeroderma pigmentosum
  3. Albinism
  4. Rombo syndrome
  5. Bazex-Christol-Dupre syndrome
  6. Dowling-Meara subtype of epidermolysis bullosa simplex
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77
Q

DX?

A

Laugier–Hunziker syndrome is a rare acquired disorder characterized by diffuse hyperpigmentation of the oral mucosa and longitudinal melanonychia in adults. They appear as macular lesions less than 5 mm in diameter.

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78
Q

Most commonly associated syndrome with this clinical presentation?

What other syndromes also have this lesion?

A

Port wine stain (nevus flammeus)

  • Sturge Weber syndrome
  • Beckwith-Weidemann syndrome
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79
Q

What is the term for this clinical presentation?

What are 5 causes?

A
  1. Bell’s Palsy (idiopathic, viral, Lyme disease)
  2. Ramsey Hunt Syndrome (VZV infection)
  3. Heerdfort syndrome (Acute sarcoidosis: Uveitis, Parotid swelling, Fever, Facial paralysis)
  4. Melkerson Rosenthal Syndrome (Orofacial Granulomatosis: lip swelling, facial nerve paralysis, fissured tongue)
  5. Perineural/neural invasion of tumor affecting CN VII
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80
Q

Most likely diagnosis?

  • Key radiographic features
  • Key non-radiographic features
  • What may have a similar presentation and confuse you
A

Segmental odontomaxillary dysplasia

  • missing premolar, vertical oriented coarse bony trabeculae, smaller maxillary sinus
  • Becker nevus, gingival hyperplasia in the area
  • Fibrous dysplasia
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81
Q

All lesions with BRAF mutations

A

Some ameloblastomas

50% of Langerhan cell histiocytosis

Melanoma

Acquired melanocytic nevus

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82
Q

The HLAs:

  • Cw6
  • B27
  • DR4
  • DR3/B8
  • DRw52
  • B51
  • DRB1
A

Cw6= psoriasis (geographic tongue)

B27= Reiters syndrome

DR4= Hecks disease

DR3/B8= primary sjogrens

DRw52=primary or secondary sjogrens

B51= Behcets

BRB1=Rheumatoid Arthritis

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83
Q

Mikulicz cells

Mikulicz disease

Mikulicz syndrome

Mikulicz aphthae

A

Mikulicz cells: rhinoscleroma (klebsiella rhinoscleromatis)

Mikulicz disease: IgG4 disease Bilateral painless swelling of lacrimal and salivary glands which show an intense chronic inflammatory infiltrate

Mikulicz syndrome: Similar parotid and lacrimal enlargement but due to TB, sarcoid, lymphoma etc

Mikulicz apthae: minor aphthous ulcers

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84
Q

Things to see with a wood’s lamp

A
  • Ash leaf spots of Tuberous sclerosis
  • Red fluorescence of the teeth in Congenital Erythropoietic porphyria
  • Give tetracycline 48 hours before a debridement for osteomyelitis: alive bone fluroescense with woods lamp and dead bone does not
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85
Q

Some serologies:

Diffuse cutaneous systemic sclerosis

Limited cutaneous systemic sclerosis (includes CREST)

Paget’s disease

Lupus Erythematous

A

DCSS: antitopoisomerase-1 antibodies also called anti-scl70

LCSS: anticentromeric antibodies

Paget’s: elevated ALK phos

Lupus: antibodies against dsDNA, antibodies against Sm(smith), elevated ANAs, elevated RF

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86
Q

DX?

A

Lichen planus

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87
Q

2 clinical syndromes of Acute Sarcoidosis

A

Lofgren syndrome:

Erythema nodosum

Bilateral hilar lymphadenopathy

Arthralgia

Heerfordt syndrome (uveoparotid fever)

Parotid enlargement

Anterior uveitis

Facial paralysis

Fever

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88
Q

What is the term for this histologic finding?

Diagnosis?

A

Asteroid body of sarcoidosis

-entrapped fragments of collagen within the granuloma

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89
Q

DX?

Name of the entity depicted?

A

Sarcoid granuloma

-Schumann body

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90
Q

What is the official / “more appropriate” name for the pattern seen on sialography of Sjogren’s Syndrome?

A

punctate sialectasia (fruit-laden branchless tree)

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91
Q

Most likely diagnosis?

What is the etiology?

What is the clinical term applied to these lesions?

A

Bullous impetigo

Typically staph aureus only

Lacquer

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92
Q

What is the most likely diagnosis?

What are two other conditions might you consider in your DDX?

What is the clinical term sometimes used to describe the appearance of this entity?

A

Non bullous impetigo

DDX: HSV, exoliative chelitis(though not really in this picture)

Corn flakes glued to the surface of the lesion

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93
Q

What is the most likely diagnosis?

What is the etiology?

What two characteristic clinical terms are applied to this lesion?

A

Erysipelas

Group A beta hemolytic strep infection

peau d’orange & St. Anthony’s fire

**St anthonys fire is alos applied to ergotism aka ergot poisoning

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94
Q

This lesion is positive for staph aureus and strep pyogenes.

This is a rare clinical presentation of what disease?

What is the clinical term applied to this specific lesion?

What differentiates this particular clinical pattern from other clinical patterns of this same disease?

A

Impetigo

Ecthyma

The lesion heals with a permanent scar- scarring is not expected in other lesions of impetigo either bullous or nonbullous

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95
Q

List two conditions that can result in the lesion depicted

If this patient had chronic intraoral lesions what is the most likely diagnosis?

If this patient had chronic widespread involvement of oral, nasal, ocular, and laryngeal mucosa what autoantibody is likely?

If lesions associated with this disease process were isolated to the oral cavity what autoantibody is likely?

A
  1. two conditions possible: Mucosal membrane pemphigoid (aka cicatricial pemphigoid) AND erythema multiforme
  2. CHRONIC= Mucous membrane pemphigoid
  3. widespread involvement by MMP is associated with autoantibody to epiligrin
  4. Oral only involvement of MMP is associated with autoantibodies to alpha-6-integrin
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96
Q

Name that outdated and terrifying testing method:

Sterilized sarcoid tissue is injected into the skin of a patient

A

Kveim test

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97
Q

Olfactory neuroblastoma:

  1. Name for low grade pseudo-rosette
  2. Name for high grade true rosette
A
  1. pseudo-rosette: Homer-Wright
  2. true-rosette: Flexner-Wintersteiner
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98
Q

Diagnosis?

A

Congenital syphilis

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99
Q

Pupils that accomodate when patient looks at a close up object, but do not constrict with light are called _____ _______ pupils. This is seen in what condition?

A

Argyll Robertson pupils

Tertiary syphillis

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100
Q

Loss of coordination of movement in tertiary syphillis is termed?

A

tabes dorsalis

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101
Q

What is the diagnosis?

What other 2 clinical findings are part of the Hutchinson Triad?

A

Hutchinson teeth of Congenital syphilis

-Eigth nerve deafness, interstitial keratitis

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102
Q

BUGS summary:

Name the (most common) bug for each disease (keep scrolling):

  • Diptheria
  • Tuberculosis
  • Syphilis
  • Rhinoscleroma
  • Rhinosporodosis
  • Leprosy
  • Gonorrhea
  • Measles
  • German measles
  • Mumps
  • Scarlet fever
  • Impetigo
  • Erysipelas
  • Actinomycosis
  • Cat scratch disease
  • Histoplasmosis
  • Blastomycosis
  • Paracoccidomycosis
  • Coccidioidomycosis
  • Cryptococcosis
  • Mucormycosis
  • Toxoplasmosis
  • Chicken pox
  • Shingles
  • Infectious mononucleosis
  • NOMA
A

Diptheria = Corynebacteria Diphtheriae aka klebs loffler bacillus

Tuberculosis = Mycobacterium tuberculosis

Syphilis = Treponema Pallidum

Rhinoscleroma = Klebsiella rhinoscleromatis

Rhinosporodosis = Rhinosporidum seeberi

Leprosy = Mycobacterium leprae

Gonorrhea = Niesseria gonorrhoeae

Measles aka Rubeola = Paramyxoviridae, genus Morbillivirus.

German measles aka Rubella = Togavirus, genus Rubivirus

Mumps = Paramyxoviridae, genus Rubulavirus

Scarlet fever = Group A Beta hemolytic strep

Impetigo = staph aureus +/- strep pyogenes (aka group A, b-hemo)

Erysipelas = beta hemolytic strep (usually strep pryogenes)

Actinomycosis = actinomyces israelii

Cat scratch disease = Bartonella henselae

Histoplasmosis = Histoplasma capsulatum

Blastomycosis = Blastomyces dermatitidis

Paracoccidomycosis = Paracoccidioides brasiliensis

Coccidoidomycosis = coccidioides immitis &/or posadasii

Cryptococcosis = Cryptoccocus neoformans

Mucormycosis = Mucoromycotina absidia/mucor/rhizopus

Toxoplasmosis = toxoplasma gondii

Chicken pox = Varicella

Shingles = Herpes zoster

Infectious mononucleosis = epstein barr virus; HHV-4

NOMA = Fusobacterium necrophorum and Prevotella intermedia

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103
Q

This patient has anesthesia and loss of sweating of the overlying skin in these lesions. Biopsy demonstrated well formed granulomatous inflammation

Diagnosis?

A

Tuberculoid aka paucibacillary leprosy

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104
Q

Diagnosis?

What is the term for the characteristic facies?

What would you expect to see on biopsy?

A

Lepromatous (multibacillary) leprosy

  • Leonine facies
  • Sheets of lymphocytes with vacuolated histiocytes (aka lepra cells)
  • No well formed granulomas
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105
Q

What is the general term for this clinical presentation?

What is the differential diagnosis?

A

Macrocheilia

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106
Q

Children with red teeth DDX

A

Congenital erythropoietic porphyria aka Gunther disease

Leprosy

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107
Q

What is the name for the cells depicted in this image?

What is the diagnosis?

A

Lepra cells of lepromatous leprosy- they are vacuolated macrophages

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108
Q

Other manifestations: fever, exanthematous rash, erythematous cheeks

What is the term for the clinical lesions depicted?

Diagnosis?

What oral lesions do you expect to see?

A

Pastia lines

Scarlet fever

-White strawberry tongue then red strawberry tongue

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109
Q

Bacterial species of chronic periodontitis red complex

A
  • Treponema denticola
  • Tannerella forsythensis
  • Porphyromonas gingivalis
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110
Q

The area feels indurated or “wooden” to palpation

On incision of this lesion yellow granular material is seen.

DDX?

If histology shows club shaped filamentous bacteria in a radiating rosette pattern what is the diagnosis?

A
  • Actinomycosis
  • mimicked by Botryomycosis (usually caused by staph aureus)
  • Definitive diagnosis is Actino
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111
Q

This patient also has hypoparathyroidism and hypoadrenocorticism.

Diagnosis?

Associated genetic mutation?

A

APECED: autoimmune polyendocrinpathy-candidasis-ectodermal dystrophy syndrome

  • mucocutaneous candidiasis, nail dystrophy, corneal keratopathy, hypoparathyroid/hypoparathyroid
  • childhood onset
  • Mutation in AIRE gene
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112
Q

These are two different presentations of the same disease.

What is the terminology used to describe each image?

What is the diagnosis?

A

Luetic glossitis = atrophic form

Interstitial glossitis = gummatous form

Tertiary syphilis

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113
Q

Patient has a fetid odor eminating from their mouth. Past medical history is signficant for smoking. What is the best diagnosis?

  • What bug is implicated in this process?
  • Risk factors associated with this process?
  • If there was no associated odor what else might you consider on the differential?
  • If this disease process spreads from the gingiva to the adjacent soft tissue what term would then apply?
  • If this disease process becomes chronic and causes the loss of alveolar bone what term would apply?
  • If this disease spread through multiple planes of soft tissue and onto the skin of the face what term would then apply?
A

Necrotizing ulcerative gingivitis

  • Fusobacterium nucleatum, prevotella intermedia, porphyromonas gingivalis, treponema spp., Selenomonas spp.
  • Smoking, Stress, Poor nutrition, AIDS, immunosuppresion, Poor nutrition
  • Neisseria gonorrhea infection
  • Necrotizing ulcerative mucositis
  • Necrotizing ulcerative periodontitis
  • Cancrum oris (NOMA)
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114
Q

PMH: Healthy 35 year old male, areas have been present since adolescence. Most likely diagnosis?

  • In a 55 year old male with a clinical history significant for heart transplant what diagnosis is more likely?
  • Name 3 associated familial syndromes
  • TX?
A

-Gingival Fibromatosis

  • Medication induced gingival hyperplasia probably 2/2 cyclosporine and/or CCB
  • Byars-Jurkiewicz syndrome, Costello syndrome, Cross syndrome, Infantile systemic hyalinosis, Jones-Hartsfield syndrome, Ramon syndrome and more
  • Excellent oral hygiene +/- surgical revision based on severity (better outcome when you wait until after all permnanent teeth erupted)
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115
Q

High yield genetic disorders associated with periodontitis in kids/young adults

What is the specific terminology applied to this?

A

Papilon Lefevre & Haim munk

Acrodynia (pink disease of mercury poisoning)

Ehlers Danlos

Hypophosphatasia

Trisomy 21

Others: Acatalasia, Chediak Higashi syndrome, Cohen syndrome, AIDS, DM, Kindler syndrome

-Periodontal disease as a manifestation of systemic disease

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116
Q

What is the predominant bacteria of localized aggressive periodontitis?

This bacteria has also been found prevalently in the oral disease of patients with what disease?

A

-A. Actinomycetemcomitans (Aggregatibacter actinomycetemcomitans)

-Papilon Lefevre

Bacteria types expand when patients have generalized type and include the red complex

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117
Q

What is the term for the histologic feature seen in this image?

What conditions/diseases can this be found in?

A

Hamazaki-Wesenberg Bodies aka yellow-brown bodies

myriad medical conditions: appendicitis, cirrhosis, lymphoid tumours, colon carcinoma and numerous others, most famously sarcoidosis

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118
Q

Viral/Fungal/Bacterial Buzz words, name that disease:

  • Koplik spots
  • Mickey mouse ears/Mariners wheel histology
  • Forchheimer sign
  • Signficant cause of death in AIDS pts
  • Doubly refractile cell wall
  • Nine banded armadillo carries this
  • How about a regular armadillo carrier
  • Frequent overlying pseudoepitheliomatous hyperplasia on bx
  • Female hormones are protective against?
  • Hypersensitivity reaction to this is termed valley fever
  • Cutaneous form of disease has lesions that resemble a volcano
  • Mulberry-like ulcerations
  • Prominent mucopolysaccharide capsule (stain w/mucicarmine)
  • Pts taking deferoxamine or who are ketoacidotic at higher risk
  • Fruiting bodies
  • Obligate intracellular parasite
  • Black fever aka kala-azar
A

Koplik spots = Measles (Rubeola) (Paramyxoviridae, Morbillivirus)

Mickey/Mariner = Paracoccidioidomycosis (Paracoccidioides brasiliensis)

Forchheimer sign = German Measles (Rubella) (Toga virus Rubivirus)

Signif COD in AIDs pts = cryptococcosis (Cryptococcus neoformans)

Doubly refractile cell wall = Blastomycosis (Blastomyces dermatitidis)

Nine banded armadillo = Paracoccidioidomycosis (Paracoccidioides brasiliensis)

Regular armadillo = Leprosy (Mycobacterium leprae) (Hansen disease)

Freq pseudoepi hyper = Blastomycosis (Blastomyces dermatitidis)

Female hormones protective = Paracoccidioidomycosis (Paracoccidioides brasiliensis)

Hypersensitivity rxn to coccidioidomycosis = valley fever (Coccidioides immitis or posadasii)

Cutaneous volcano = Leishmaniasis (species Leishmania)

Mulberry ulcerations = Paracoccidioidomycosis (Paracoccidioides brasiliensis)

Prom mucopolysaccharide capsule= cryptococcosis (Cryptococcus neoformans)

Increased iron = increased risk of mucormycosis (Mucormycotina Rhizopus)

Fruiting bodies = aspergillosis (Aspergillus fumigatus)

Obligate intracellular parasite = leishmaniasis (species Leishmania)

Black fever = visceral leishmaniasis (species Leishmania)

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119
Q

Direct immunofluorescence pattern for Chronic ulcerative stomatitis?

Indirect immunofluorescence finding?

A
  • speckled pattern of IgG deposits in the basal one-third of the epithelium
  • Indirect: presence of stratified epithelium specific anti-nuclear antigen (SES-ANA)
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120
Q

What is the characteristic cell depicted here?

What is this diagnostic of?

A

Floret cell

Pleomorphic lipoma

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121
Q

In addition to the clinical finding below this patient is also experiencing joint pain.

What is the most likely diagnosis?

List the features of this condition

A

SAPHO syndrome

Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis

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122
Q

Multiple bone involvement by primary chronic osteomyelitis in children is termed

A

CRMO: Chronic recurrent multifocal osteomyelitis

thought to be a widespread variant of primary chronic osteomyelitis

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123
Q

This finding is present on both the hands and feet in addition to a pruritic skin rash.

DDX?

  • If this was a 5 year old patient with red eyes and cracked lips in addition to these findings, what diagnosis would you favor?
  • If this patient had HTN and neurologic symptoms what diagnosis would you favor? What is the term used for the neurologic symptoms?

*What oral manifestations would you expect in the second case?

A
  • Acrodynia, Kawasaki disease, Polycythemia Vera (erythromelalgia)
  • Kawaski disease
  • Acrodynia; neurologic symptoms are termed Erethism-excitability, tremors, memory loss, delirium
  • Oral symptoms of acrodynia: excessive salivation (also excessive sweating) and premature tooth loss

-

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124
Q

34 year old male, HIV+, lesions are also present on the palms and soles of the feet

DDX for this case?

-What conditions cause a rash that includes the palms and soles of the feet?

A

Secondary syphilis

Psoriasis (specifically guttate)

Erythema multiforme (should expect more oral manifestations not shown)

Palms and soles of the feet inclusive rashes: syphilis, psoriasis, EM, coxsackie viruses, rocky mountain spotted fever, mycosis fungoides, tinea

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125
Q

DX?

A

Calcific metamorphosis and aseptic pulpal necrosis

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126
Q

What are the two major clinical findings?

What two conditions produce these radiographic findings?

A
  • Thistle tube/flame shaped pulp chambers & pulp stones
  • Dentin dysplasia type 2 and pulpal dysplasia
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127
Q

What termed is applied to this radiographic finding?

What is the diagnosis?

What is the histologic description of the root of these teeth?

A

Rootless teeth

Dentin dysplasia type 1

Histo buzz:

  • whorls of tubular dentin
  • “stream flowing around boulders”
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128
Q

What two pathoses can cause a “bull neck” appearance

A

Diptheria

Ludwigs angina

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129
Q

Whats my keratin?

Epidermolysis Bullosa simplex

White sponge nevus

Pachyonychia congenita

Oral lesions of pachyonychia congenita

Pachyonychia congenita with neonatal teeth

A

EBS: Keratins 5 & 14

WSN: Keratin 4 & 13

Pachyonychia congenita: 6a, 6b, 16, 17

Pachyonychia with oral lesions: Keratin 6a

PC neonatal teeth: Keratin 17

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130
Q

At least 4 conditions associated with premature tooth loss

A

Acrodynia (pink disease) mercury exposure children

Hypophosphatasia

Dentin Dysplasia type 1 (rootless teeth)

Papilon Lefevre

Haim Munk

Acatalasia

Cohen Syndrome

Ehlers-Danlos

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131
Q

What clinical features are depicted in this image?

What is the most likely diagnosis?

What mutation and inheritance pattern is associated with this condition?

A

-bowing of long bones with a break (fragility of the bones), diffuse fibro-osseous lesions of the jaws (on histology they have prominent psamomma bodies)

MUT: GDD1, auto dom

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132
Q

Time to dominate Lipid Reticuloendothelioses….

What are the three diseases & their subtypes?

A
  1. Goucher (Type 1 - least severe, Ashkenazi Jewish, Types 2/3 CNS invovment, severe)
  2. Neimann Pick (ABC: A,C=severe, B=less severe)
  3. Tay-Sach (Mild to severe: mild can live to adulthood, severe do not)
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133
Q

What is the image depicting?

What disease is this associated with?

What is another important maxillofacial feature?

What is the primary histological buzz phrase?

What enzyme is lacking? What accumulates?

A

Erlymeyer Flask Deformity (EFD) of the longbones

Gaucher Disease

Multiple RLs of the mandible (filled with Gaucher cells)

Gaucher cells (macrophages) with wrinkled silk cytoplasm

Glucocerebrosidase missing so accumulation of glucosylceramide

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134
Q

Niemann Pick Disease:

What enzyme is missing? What accumulates as a result?

Of the types, which are more severe?

What is the genetic mutation?

What is the characteristic cell?

A

Sphingomyelinase missing, so sphingomyelin accumulates

A,C are most severe…Type B survives into adulthood

NPC1, NPC2

“Sea-Blue” Histiocyte seen on bone marrow aspirate

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135
Q

Tay-Sachs:

How does it compare to the other lipoidendoreticuloses?

What enzyme is missing? What accumulates?

What cell type is affected?

A

Most severe of the lipoidendoreticuloses…only mild cases survive to adult hood

Beta-hexosaminidase A missing, ganglioside accumulates

Neurons (hense the severe nature as opposed to Gaucher and NP where accumulation happens in macrophages)

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136
Q

Infant with bilateral facial swellings, fever, leukocytosis, and hyperostosis of his facial bones. Blood cultures negatve. What’s the most probable diagnosis?

A

Caffey Disease (AD, a COL1A1 mutation has been found)

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137
Q

Name the skin lesion…What systemic condition is it associated with?

A

Lupus pernio. Sarcoidosis

Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.

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138
Q

What are the 3 main components of RAMON syndrome?

A
  1. Cherubism
  2. Gingival fibromatosis
  3. seizures
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139
Q

What is the most likely diagnosis for for lesion depicted?

What syndrome is associated with these lesions?

What are the major components of this syndrome?

A
  • Multiple large congenital nevi
  • Neurocutaneous melanosis
  • Potentially fatal condition in which patients have multiple or large congenital nevi + melanocytic neoplasms of the CNS (melanomas)
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140
Q

What is the term for areas indicated by the yellow arrows?

What diagnosis is this feature specific for?

What mutation is associated?

A
  • Kamino bodies
  • Spitz nevus
  • HRAS
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141
Q
  • What is the most likely diagnosis?
  • What is the most common mutation associated with this lesion overall?
  • What is a common mutation associated with this lesion at this specific site?
  • What are the 4 clinicopathologic subtypes of this entity?

which subtype is most common in the oral cavity?

which subtype is most common in Blacks?

which subtype is most common on the skin?

A
  • Melanoma
  • BRAF 50%
  • Mucosal melanomas are also associated with KIT mutations
  • Clincopathologic subtypes:

superficial spreading, nodular, acral lentigenous, lentigo maligna melanoma (lentigo maligna aka hutchinson freckle is the precursor lesion and is just melanoma in situ)

  • Oral cavity = acral lentigenous
  • Blacks = acral lentigenous
  • Skin = superficial spreading (70%)
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142
Q

A 75 year old male presents with complaints of right sided jaw and neck pain. IOE is WNL. EOE is positive for the image shown.

  • What is the most likely diagnosis?
  • What would you see on biopsy?
  • What other symptoms are classic for this condition?
A
  • Temporal arteritis aka giant cell arteritis aka Horton disease
  • transmural and perivascular infiltrate of lymphocytes, histiocytes and giant cells +/- granulomas. Elastic layer is fragmented
  • blurry vision, headaches, jaw pain
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143
Q

This biopsy is from the posterior lateral tongue.

Diagnosis?

  • is this a neoplastic process?
A
  • Sub-gemmal neurogenous plaque
  • This is part of normal anatomy. Plexus underlies taste buds on the posterior lateral tongue. Taste buds are CK 8/18+
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144
Q

This is a biopsy of an enlarged cervical lymph node.

On higher power the following are seen: Karryorhexis, fibrin deposits, plasmacytoid monocytes

  • Most likely diagnosis?
  • What other entities are considered on the differential? How would you exlcude them?
A
  • Kikuchi disease
  • Cat scratch disease: there should be a history of cat exposure and a skin papule. You also typically see follicular hyperplasia and more neutrophils
  • Lymphoma with necrosis: should see more atypia and no plasmacytoid monocytes
  • Lupus lymphadenitis: architecture of the lymph node is presevered, necrosis is in the paracortical area
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145
Q

Patient presented with a well-defined, oval, red nodule of the skin.

Diagnosis?

  • Is this process benign, malignant, or reactive/nonneoplastic?
  • What are the 3 histologic components of this entity?
A

Intravascular papillary endothelial hyperplasia (Massons tumor)

  • Non neoplastic/reactive process
  • Dilated vascular spaces, fibrin deposition, endothelial cell proliferation
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146
Q

6 year old patient, Diagnosis?

  • Characteristic finding on electron microscopy?
  • stains?

What classification system is currently used?

A
  • LCH
  • Birbeck granules
  • CD1a, langerin (CD207)

CLASSIFICATION:

  1. Single organ: usually bone or skin, can be multiple lesions but only of one system
  2. Multiorgan w/o organ dysfunction
  3. Multiorgan w/ organ dysfuction low risk: skin, LN, pituitary, bone
  4. Multiorgan w/ organ dysfunction high risk: lung, liver, spleen, BM
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147
Q

This clinical presentation is characteristic for what?

What is the responsible genetic mutation?

The mutation results in overproduction of what product?

What is the inheritance pattern?

What 2 other conditions/diseases can produce a similar appearance of the body habitus?

A
  • Marfan syndrome
  • Fibrillin-1
  • TGF-Beta
  • Autosomal dominant
  • MEN2B, NBCCS
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148
Q

A 20 year old patient present with a well-defined radiolucency of the mandible. All teeth in the area are vital. A biopsy is performed and shows the following.

  • What is the histologic buzzword for this presentation?
  • What is this characteristic (but not specific) for?
  • What stains would you perform for confirmation?
  • What is the classic clinical presentation for this disease?
  • What is the prognosis/outcome for the majority of patients?
A
  • Emperipolesis: the active penetration of one cell by another which remains intact
  • Rosai-Dorfman disease aka sinus histiocytosis with massive LAD
  • S100 and CD1a: S100 positive (same as langerhan cell histiocytosis), CD1a negative (to rule out langerhan histiocytes)
  • Massive painless cervical LAD in a younger patient, of unknown etiology
  • 80% of cases spontaneous resolve, this is not a clonal process
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149
Q
A
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150
Q

Diagnosis?

Term for the foot manifestation?

Oral findings?

A
  • Arsenic poisoning
  • Black foot disease
  • necrotizing ulcerative stomatitis, excessive salivation,
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151
Q

This patient was previously treated with gold.

-What is the term for manifestation seen in this photo and what is insighting event?

What is a common oral complication in patients treated with gold?

A

Chrysiasis: when patients are treated with gold therapy they can develop this slate blue pigmentation in areas exposed to sunlight

-Oral mucositisi

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152
Q

What bacteria is most commonly responsible for Lemierre syndrome?

A

Fusobacterium necrophorum

  • usally occurs in teens
  • can be life threatening
  • basically an oropharyngeal infection that can lead to sepsis
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153
Q

A newborn is born with jaundice. If the baby is not treated these may develop ______ a dangerous accumulation of bilirubin in the brain

A

Kernicterus

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154
Q

DDX?

  • This patient had peripheral blood eosinophilia, what is the diagnosis?
  • A biopsy is performed of the lymph node, what would you expect to see?
A
  • DDX: Branchial cleft cyst, Unicentric castleman disease, kikuchi disease, kimura disease, metastatic (HPV+ SCC) carcinoma
  • Kimura disease
  • eosinophils, eosinophilic microabscesses, vessels with hyalinzation, follicular hyperplasia
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155
Q

Diagnosis?

A

-Cholesteatoma

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156
Q

A patient complains of profuse sweating near her right ear when eating spicy and sour foods. PMH signficant for previous excision of a pleomorphic adenoma in the parotid glad. WHat is the most likely diagnosis? What test could be used to better demonstrate the patients symptoms?

A
  • Frey’s syndrome: results from injury of the salivary gland
  • Starch iodine test
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157
Q

Local causes of delayed tooth eruption

Systemic causes of delayed tooth eruption

A

Local causes: gingival fibromatosis, clefts, ROD, SOD, supernumeraries, tumors

Systemic: anemia, celiac, cerebral palsy, chemotherapy, endocrine disturbances, vit D resistant rickets

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158
Q

DX?

What intraoral finding is found in 92% of these patients

A

Rubinstein-Taybi

Talon cusp

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159
Q
A

Dens en dente aka dens invaginatus

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160
Q

DX?

What might this be caused by?

A

buccal bifurcation cyst

-cervical enamel extension

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161
Q

What syndrome would you suspect?

What are the other associated findings?

A

PHACES

P: posterior fossa brain anomalies

H: Hemangioma

A: arterial anomalies

C: Cardic defects

E: Eye anomalies

S: sternal cleft, supraumbilical raphe

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162
Q

What is the term for this finding?

Name an associated syndrome?

A

sternal cleft

PHACES

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163
Q

What is the term for this radiographic finding?

What is it characteristic of?

A

tram-track or tram-line calcifications

Sturge weber syndrome

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164
Q

Salt fish diet –>

Leather and wood-working –>

A

salt fish = sinonasal adenocarcinoma

leather and wood-working = intestinal type sinonasal adenocarcinoma

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165
Q

The child has had multiple episodes of swelling

There is no pus on palpation of the duct

DX?

TX?

Is this condition the most common cause worldwide of parotid swelling?

A

Juvenile recurrent parotitis

Usually happens around 3-6 yrs old, multiple episodes, seld resolves by puberty

-Worldwide: Mumps, in the USA: Juvenile recurrent parotitis

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166
Q

DX?

A

Cheilitis glandularis

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167
Q

What conditions can produce complaints of excess drooling?

If there is no obvious cause, and they get episodic sialorrhea, what is the term?

A
  • cerebral palsy, down syndrome, ALS, parkinsons, heavy metal poisoning, rabies
  • idiopathic paroxysmal sialorrhea
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168
Q

Rose bengal dye test is used for?

What other test is used for the same purpose?

A
  • diagnosis of keratoconjunctivitis sicca
  • Schirmer test
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169
Q

Difference between Epstein Pearl vs Bohn Nodule vs Ging Cyst of Newborn

A

Epstein pearls: traped epithelium along fusion MIDLINE

Bohns Nodule: minor salivary glands, scattered over hard palate, near soft palate junction

Gingival cyst of newborn: dental lamina, on alveolar ridge

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170
Q

Conditions that affect the nails (8)

A

Lichen planus: thinned with longitudinal ridging and fissuring

Hand foot mouth disease: Beau lines, Onychomadesis(spontaneous separation of nail from matrix)

Plummer-vinson: Koilonychia aka spoon shaped nails

APECED/mucocutaneous candidiasis

Pachyonychia congenita: Nail thickening

Dyskeratosis congenita: nail dystrophy

Dariers disease: candy-cane nails (alternating red and white bands)

Laugier-Hunziker: longitudinal pigmented band on the nail

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171
Q

Large radiolucency is seen in a skull film of a patient with elevated alk phos and bone pain. What is the clinical term for this lesion?

A

osteoporosis circumscripta- occurs in early stage disease

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172
Q

What 3 disease cause Forcheimer spots

A

German measles (rubella)

Scarlet fever

Mono

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173
Q

Lesion is firm

BX demonstrates benign appearing bone and cartilage

A

Cutright lesion AKA Reactive osseous and chondromatous metaplasia

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174
Q

Diagnosis?

Recommended therapy?

A

DX: Perioral dermatitis

  1. “zero therapy” tell the patient to discontinue anything they have been applying in the area
  2. topical pimecrolimus or topical erythromycin.
  3. Oral tetracycline (though not in children or pregnant patients)
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175
Q

DX?

Tx?

A

DX: Exfoliative chelitis

TX:

  1. Elimination of habit
  2. Tx of any superimposed infection: antiobiotic and or antifungal
  3. topical tacrolimus
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176
Q

DX?

TX?

A

DX: transient ligual papillitis

TX: self resolving though you can give topical corticosteroids or analgesics for discomfort

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177
Q

Differential DX?

Ancillary testing to confirm dx?

A

Behcet’s syndrome, IgA deficiency, cyclic neutropenia, IBD, Sweet syndrome, MAGIC syndrome

Ancillary test for Behcets = positive pathergy test

cyclic neutropenia = concurrent blood tests, biopsy that shows little neutrophil acitivity in the ulcer

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178
Q

Describe the finding being depicted in this image. What systemic condition(s) is this typical of?

A

Loss of lamina dura- generalized

  • Hyperparathyroidism, osteomalacia, pagets disease, fibrous dysplasia
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179
Q

In addition to the finding depicted this male child has the following bloodwork:

  • Hypophosphatemia
  • Low serum calcium

What is the most likely diagnosis?

What characteristic finding is seen on histology of one of these teeth?

A

The picture is showing an abscess which frequently results from pulpal extensions to the surface in Vitamin D resistent rickets

**teeth on histology demonstrate globular dentin

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180
Q

What is this image depicting and where is this commonly found?

A

Hyaline body aka pulse granuloma

Walls of inflammatory cysts

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181
Q

Metastasis to the mandible that is PAX8 and TTF1:

A

thyroid

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182
Q

Cherry-red spot on the macule is associated with:

A

Tay-Sachs
(Lysosomal storage disorders, retinal ischemia, and retinal infarction)

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183
Q

Negri bodies are associated with:

A

rabies

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184
Q

T1 SCC with tumor thickness < 3mm, which may have you do a neck dissection and radiation:

A
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185
Q

Which of the following is associated with NUT-something:

A

NUT midline

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186
Q

PAX-FOX01 seen in:

A

alveolar rhabdomyosarcoma

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187
Q

EWS is paired with which gene in Ewing sarcoma:

A

FLI1

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188
Q

EWSR1-ATF1 is seen in which two:

A

clear cell sarcoma and clear cell odontogenic carcinoma

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189
Q

What is the female equivalent of a seminoma:

A

dysgerminoma

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190
Q

Which subtype of rhabdo has a specific translocation:

A

alveolar

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191
Q

Most common nasal septum papilloma:

A

fungiform

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192
Q

Nasal papilloma most likely to be malignant:

A

inverted

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193
Q

Nasal papilloma that is destructive and malignant:

A

inverted

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194
Q

Most common nasal disease seen in family practice:

A

rhinitis

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195
Q

Patient with red face and bumps that goes away with tetracycline:

A

rosacea

196
Q

STAT6 in which soft tissue tumor:

A

solitary fibrous tumor

197
Q

Most likely to have Flexner-Wintersteiner rosettes:

A

olfactory neuroblastoma

198
Q

Chromosome for nevoid basal cell carcinoma syndrome:

A

9

199
Q

Pierre-Robin is seen with:

A

Velocardiofacial syndrome (Digeorge)
Stickler syndrome

200
Q

Most common lesion in a person with Beckwith-Wiedemann

A

Wilms
(Wilms tumor, adrenal carcinoma, hepatoblastoma, rhabdomyosarcoma, neuroblastoma )

201
Q

Best site for FNA:

A

soft palate swelling (other answers were epithelial)

202
Q

Which amelogenesis imperfecta is scattered in primary:
(scattered pits?)

A

Hypoplastic?
Other variants - hypocacification, hypomaturation and AI with taurodontism

203
Q

Which symptom associated with DSPP mutation

A

Hearing loss
(vs dysosmia etc)

204
Q

Old male patient with renal failure, lumbar pain, and IgG spike

A

multiple myeloma

205
Q

Which is true about thyroglossal duct cyst

A

midline presentation below the hyoid
Midline
Adjacent to hyoid bone
Any age - peds-first 2 decades of life most common
Respiratory lining most often, cuboidal or even small intestine epithelium occasionally

206
Q

Which has perinuclear eosinophilic condensation

A

white sponge nevus

207
Q

Genodermatosis with autosomal recessive and problems with DNA repair

A

xeroderma pigmentosum

208
Q

Most common salivary neoplasm on lower lip

A

??
Mucoep

209
Q

Most common thyroid carcinoma in ectopic thyroid sites

A

PTC

210
Q

Dental manifestation of oral-dento-facial-cardio syndrome

A

radiculomegaly, oligodontia, retained primary teeth, and malocclusion
root dilacerations, malocclusion, and cataracts
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6466113/#B1-ijerph-16-00928

211
Q

OKCs in NBCCS have upregulation of

A

p53

212
Q

Most common location for lateral periodontal cyst

A

mandibular canine/premolar
(Mandibular premolar-canine-lateral incisor area)

213
Q

Most common presentation of orthokeratinizing odontogenic cyst

A

unerupted molar

214
Q

Most common odontogenic cyst in anterior mandible

A

glandular odontogenic cyst

215
Q

Most likely variant of ameloblastoma to present as RL/RO

A

desmoplastic

216
Q

Best stain for peripheral ameloblastoma or intraoral BCC

A

Ber-EP4

217
Q

Second “hit” of double-hit in colorectal cancer

A

One copy of a tumour suppressor gene (TSG) is inactivated by a nonsense or frameshift mutation, and the other copy is inactivated by a similar mutation or by loss of heterozygosity (LOH)
it has been shown that the earliest event of sporadic
colorectal cancers is the inactivation ofthe APC gene,1,2 or other genes involved in the
Wnt pathway.3,4 The APC gene inhibits members of the Wnt signaling pathway, which promote the expression of β-catenin. In its turn, β-catenin acts as an enhancer of cell division.

218
Q

Which is most likely to be trisomy

A

Patau
Down Syndrome (Trisomy 21), Edwards Syndrome (Trisomy 18), Patau Syndrome (Trisomy 13), Klinefelter Syndrome (XXY Syndrome)

219
Q

Most common thing with Chtovek sign:

A

hypocalcemia

220
Q

Facial paralysis, fever, and serum/urine calcium; which chronic granulomatous most likely with these

A

sarcoid

221
Q

Best test for lingual thyroid

A

99-technitium scan

222
Q

Which is not a required feature for Sjogren

A

scarring alopecia

223
Q

Parabasal/basal and speckled DIF

A

chronic ulcerative stomatitis

224
Q

Which antibody for chronic ulcerative stomatitis

A

delta-NP63-alpha

225
Q

Chicken-wire pattern of DI

A

pemphigus

226
Q

Shaggy deposition of fibrinogen at basement membrane with cytoid bodies:

A

lichen planus

227
Q

Lichen planus can sometimes be seen in association with:

A

superficial mucoceles

228
Q

GFAP staining peripheral of which salivary neoplasm

A

PA

229
Q

Which is associated with HLA-DR4:

A

prurigo (Hecks and giant cell arteritis not on the list)

230
Q

Laryngeal papillomatosis is associated with:

A

HPV6, 11

231
Q

Osteogenic osteomalacia is most often seen with:

A

phosphaturic mesenchymal tumor

232
Q

Which are associated with HPV:

A

E7 (E6 not on the list)

233
Q

Which is true regarding AOT:

A

well-circumscribed RL (the others made less sense)

234
Q

Lyre is associated with:

A

carotid body paraganglioma

235
Q

Most likely site for saddle embolism to come from:

A

leg/lower extremities

236
Q

Odontohypophosphatasia is most often associated with:

A

stress fractures and premature loss of adult dentition

237
Q

USP6 can be used to identify which spindle cell tumor:

A

nodular fasciitis

238
Q

USP6 is rearranged in which bone tumor:

A

aneursymal bone cyst

239
Q

Example of transient neoplastic disease:

A

nodular fasciitis

240
Q

Most common soft tissue location for metastatic tumor:

A

gingiva

241
Q

Which is associated with NKX3.1:

A

prostate

242
Q

Which site is most likely to have dysplasia or early invasive carcinoma on biopsy:

A

floor of mouth?
high risk sites - lateral tongue?

243
Q

Most common soft tissue tumor in children:

A

rhabdomyosarcoma

244
Q

Leser-Trelat sign is associated with onset of multiple:

A

seborrheic keratoses

245
Q

Most common head and neck site for osteoma:

A

frontal sinus

246
Q

Loose bodies are seen in:

A

synovial chondromatosis

247
Q

Rhomboid bodies seen in:

A

gout
(buzzword: needle shaped crystals)

248
Q

Patient with trismus, no radiographic changegs, acellular dense fibrous tissue

A

oral submucous fibrosis (scleroderma on differentiatial)

249
Q

Bartonella causes cat scratch

A

bacillary angiomatosis

250
Q

Lyme disease is caused by

A

Borrelia burgdorferi
Rarely, Borrelia mayonii

251
Q

Most common mutation in melanoma:

A

BRAF

252
Q

Which is true regarding PGCG:

A

occurs on ridge

253
Q

Fibrous dysplasia may mimic which type of osteosarcoma:

A

fibroblastic?

254
Q

Cemento-osseous dysplasia is often seen with:

A

simple bone cyst?

255
Q

Which of the following has an autosomal dominant pattern of inheritance with genetic imprinting:

A

paraganglioma

256
Q

Which of the following defines epigenetic

A

methylation of DNA

257
Q

Which of the following stains calcium

A

von Kossa
Calcium salts appear as dark blue to purple, brittle deposits on H&E stain and can be stained with alizarin red (more specific) or von Kossa stains (black).

258
Q

Cochineal beetles are related to oral pathology because

A

eosin dye

259
Q

Which stains oncocytomas

A

PTAH

260
Q

False positives in TB testing:

A

vaccination with BCG

261
Q

Who will most likely get parotid lymphoepithelial cysts

A

HIV patients

262
Q

Which is true about giant cell arteritis

A

Sudden blindness (alt: seen in women or men)
Can also have tongue necrosis

263
Q

Which odontogenic neoplasm can have clear cell changes

A

CEOT

264
Q

Spheroid bodies with Maltese cross

A
265
Q

Hereditary angioedema is the result of:

A

C1-INH enterase deficiency

266
Q

Metastasis to the mandible that is PAX8 and TTF1

A

Thyroid

267
Q

Cherry-red spot on the macule is associated with

A

Tay-Sachs

268
Q

Negri bodies associated with

A

rabies

269
Q

T1 SCC with tumor thickness <3mm, which may have you do a neck dissection with radiation?

A

Not sure what they’re going for here. Just look up SCC staging

270
Q

Which of the following is associated with NUT-something?

A

NUT midline

271
Q

PAX-FOX01 seen in

A

alveolar rhabdomyosarcoma

272
Q

EWS paired with which gene in Ewing Sarcoma?

A

FLI1

273
Q

EWSR1-ATF1 seen in which 2 entities?

A

clear cell sarcoma and clear cell odontogenic carcinoma

274
Q

Female equivalent of seminoma

A

dysgerminoma

275
Q

which subtype of rhabdomyosarcoma has a specific translocation?

A

alveolar

276
Q

most common nasal septum papilloma

A

fungiform

277
Q

nasal papilloma most likely to be malignant

A

inverted

278
Q

nasal papilloma that is destructive and malignant

A

inverted

279
Q

most common nasal disease seen in family practice?

A

rhinitis

280
Q

Patient with red face bumps that go away with tetracycline?

A

rosacea

281
Q

STAT6 for which soft tissue tumor

A

solitary fibrous tumor

282
Q

Flexner-Wintersteiner rosettes

A

olfactory neuroblastoma

283
Q

chromosome for nevoid basal cell carcinoma syndrome? mutation?

A

9…patch PTCH1

284
Q

Pierre-Robin sequence seen with which syndrome?

A

velocardial facial syndrome

285
Q

Most common lesion in a person with Beckwith-Wiedemann syndreome?

A

Wilms tumor

286
Q

Best site for an FNA

A

Soft palate swelling (other answers were epithelial)

287
Q

Which amelogenesis imperfecta is scattered in primary

A

Hypoplastic AI often affects both primary and permanent, but can affect primary only, so maybe they are referring to this?

288
Q

Which symptom associated with DSPP mutation?

A

hearing loss

289
Q

Old dude with renal failure, lumbar pain, IgG spike

A

multiple myeloma

290
Q

True about thyroglossal duct cyst:

A

midline presentation below the hyoid

291
Q

Perinuclear eosinophilic condensation

A

white sponge nevus

292
Q

Genodermatosis with autosomal recessive and problems with DNA repair

A

xeroderma pigmentosum

293
Q

most common salivary neoplasm on lower lip

A

mucoepidermoid carcinoma (pg443 neville)

294
Q

Most common thyroid carcinoma in ectopic thyroid sites

A

PTC

295
Q

5 Most common dental maifestation in oculo-facio-cardio-dental syndrome

A
  1. radiculomegaly 2. oligodontia 3. root dilacerations 4 malocclusion 5 delayed eruption
296
Q

OKCs in NBCCS have up regulation of which gene

A

p53

297
Q

Most common location for lateral periodontal cyst

A

mandibular canine / premolar

298
Q

most common presentation of orthokeratinizing odontogenic cyst

A

unerupted molar

299
Q

Most common odontogenic cyst in the anterior mandible

A

glandular odontogenic cyst

300
Q

Most likely variant of ameloblastoma to present as mixed RL/RO

A

desmoplastic ameloblastoma

301
Q

Best stain for a peripheral ameloblastoma or intraoral BCC

A

BerEP4

302
Q

Second hit of double hit colorectal cancer

A

According to Robbins: Methylation abnormalities, inactivation of normal alleles: APC and B-catinin

303
Q

Which is most likely to be trisomy?

A

Patau (trisomy 13)

304
Q

Most common thing with Chtovek sign

A

hypocalcemia

305
Q

Facial paralysis, fever, serum/urine calcium; which chronic granulomatous condition is most likely associated with these?

A

Sarcoid

306
Q

Best test for lingual thyroid

A

99-technitium scan

307
Q

What is NOT a required feature of sjogren?

A

scarring alopecia

308
Q

Parabasal/basal and speckled DIF

A

CUS chronic ulcerative stomatitis

309
Q

Which antibody is used for chronic ulcerative stomatitis?

A

delta-NP63-alpha

310
Q

CHichen-wire pattern DIF

A

pemphigus

311
Q

Shaggy deposition of fibrinogen at basement membrane with cytoid bodies

A

lichen planus

312
Q

lichen planus can sometimes be seen in association with

A

superfical mucoceles

313
Q

GFAP stainin peripheral of which salivary gland neoplasm?

A

PA

314
Q

Which is associated with HLA-DR4

A

prurigo (Hecks and Giant cell arteritis not options

315
Q

Laryngeal papillomatosis is associated with which HPV strains

A

6,11

316
Q

Osteogenic osteosarcoma is most often seen with which type of tumor?

A

phosphturic mesenchymal tumor

317
Q

which proteins are associated with HPV

A

E7 (E6 not an option)

318
Q

Which is true regarding AOT:

A

well-circumscribed RL

319
Q

Lyre is associated with

A

Carotid body paraganglioma )lyre sign = splaying of the internal and external carotid arteries

320
Q

Most likely site for a saddle embolism to originate

A

leg

321
Q

Odontohypophosphatasia is most often associated with

A

stress fractures and premature loss of adult dentition

322
Q

USP6 can be used to identify which spindle cell tumor?

A

nodular fasciitis

323
Q

USP6 is rearranged in which bone tumor?

A

Aneurysmal bone cyst

324
Q

Example of transient neoplastic disease

A

nodular fasciitis

325
Q

Most common soft tissue location for metastatic tumor

A

gingiva

326
Q

Which is associated with NKX3.1

A

Prostate

327
Q

Most specific marker for differentiating adrenal cortical carcinoma from a met?

A

SF1?

In the past, SF-1 has been suggested as a marker to differentiate between tumors of adrenocortical and nonadrenocortical origin [34]. Recent studies have demonstrated overexpression of SF-1 in most cases of childhood adrenocortical tumors [11]. Moreover, Sbiera et al. [35] confirmed that SF-1 is a highly valuable immunohistochemical marker to determine the adrenocortical origin of an adrenal mass and demonstrated that SF-1 expression is of stage-independent prognostic value in patients with ACC.

328
Q

Which site is most likely to have dysplasia or early invasive carcinoma on biopsy

A

floor of mouth

329
Q

Most common soft tissue tumor in children

A

rhabdomyosarcoma

330
Q

Leser-Trelat sign is associated with onset of multiple what?

A

seborrheic keratoses

331
Q

Most common head and neck site for an osteoma

A

frontal sinus

332
Q

loose bodies are seen in

A

synovial chondromatosis

333
Q

rhomboid bodies are seen in:

A

gout

334
Q

Patient with trisumus, no radiographic changes, acellular dense fibrous tissue

A

oral submucous fibrosis (but scleroderma also option)

335
Q

Bartonella causes cat scratch and

A

bacilliary angiomatosis

336
Q

Lyme disease is caused by

A

borrella burgdorferi

337
Q

Most common mutation in melanoma

A

BRAF

338
Q

Which is true regarding PGCG?

A

Occurs on the ridge

339
Q

Fibrous dysplasia may mimic which type of osteosarcoma?

A

fibroblastic?

340
Q

Cemento-osseous dysplasia is often seen with

A

simple bone cyst

341
Q

Which of the following has an autosomal dominant pattern of inheritance with genetic imprinting?

A

Paraganglioma

342
Q

Which of the following defines epigenetic?

A

methylation of DNA

343
Q

Which of the following stains calcium

A

von Kossa

344
Q

Cochineal beetles are related to oral pathology because

A

eosin dye

345
Q

Which stains oncocytomas?

A

PTAH

346
Q

False positives in TB testing

A

vaccination with BCG

347
Q

Who will most likely get parotid lymphoepithelial cysts?

A

HIV

348
Q

Which is true about giant cell arteritis?

A

sudden blindness (or seen in women or men - women are affected 2x more)

349
Q

Which odontogenic neoplasms can have clear cell changes?

A

CEOT

350
Q

Spheroid bodies with Maltese cross

A

High urinary protein nephrotic syndrome /Fabry disease vs babesiosis aka babesia microti (tick borne illness)

351
Q

Ferruginous bodies seen in

A

asbestos fibers

352
Q

Electric cautery of acinar cells in parotid glands can make them seem like

A

spindle cells

353
Q

Increased risk of oral cancer can be seen in

A

dyskeratosis congenita

354
Q

Wernicke-Korsakoff in alcoholics caused by deficiency in

A

thiamine (B1)

355
Q

Vitamin A deficiency associated with

A

blindness

356
Q

Which is true about a blue nevus

A

tyndall effect

357
Q

Which is true about a halo nevus

A

hyperpigmented lesion surrounded by hypopigmentations

358
Q

McCune-Albright genetic mutation

A

GNAS1

359
Q

BRAF inhibitors may be used in the treatment of which odontogenic lesion

A

ameloblastoma

360
Q

mutations in SH3BP2 seen in

A

cherubism

361
Q

Which syndrome presents with cherubism-like CGCG-like leisons

A

Noonan syndrome

362
Q

Which is the most common complication of a middle ear infection?

A

Cholesteatoma or acute mastoiditis (I’m going to favor mastoiditis thanks to google)

363
Q

What is used in reverse transcriptase PCR?

A

cDNA

364
Q

HHV8 associated with

A

KS

365
Q

Hyaline globules associated with

A

KS

366
Q

Kikuchi-Fujimoto disease AKA

A

necrotizing histiocytic lymphadenitits

367
Q

Emperipolesis is seen in

A

massive sinus histiocytosis with lymphadenopathy (Rosai Dorfman)

368
Q

Sarcomatoid differentiation can be seen in which salivary neoplasm

A

salivary duct carcinoma

369
Q

Most common salivary duct carcinoma cell type

A

apocrine

370
Q

What part of the body controls sleep and appetitie?

A

hypothalamus

371
Q

Which is associated with myasthenia gravis

A

thymoma

372
Q

Stage III and IV oropharyngeal carcinoma which have prognostic implications

A

both HPV and tobacco use

373
Q

If a child has gingival bleeding and arthralia, consider:

A

vitamin c deficiency

374
Q

MDM2 therapy and radiation therapy is novel treatment for

A

rhabdomyosarcoma

375
Q

MUC4 is a specific marker for

A

low grade fibromyxoid sarcoma

376
Q

what type of lung cancer is a mid age female non-smoker

A

ALK positive lung cancer (EGFR as well)

About 50% to 60% of lung cancers found in people who never smoked are adenocarcinomas. About 10% to 20% are squamous cell carcinomas. A few (6% to 8%) are small cell lung cancers, and the rest are other types of lung cancer.

377
Q

Extravasated RBCs and fibrin in biopsy seen in

A

plasminogen deficiency

378
Q

Biphasic salivary gland tumor

A

PA, epi-myoepi carcinoma, cribriform (?), Adenoid cystic ca

379
Q

Progressive multifocal leukoencephalopathy is seen in

A

AIDS

380
Q

Hypophosphatasia is associated with reduced levels of

A

alkaline phosphatase

381
Q

Segmental odontomaxillary dysplasia may present with

A

hypertrichosis!

382
Q

Which 2 markers are best for diagnosing a desmoplastic melanoma

A

HMB45 and S100

383
Q

Which stain uses the same target as MART-1

A

Melan-A

384
Q

Which marker is positive in PEComa

A

HMB45

385
Q

Which is true regarding amyloidosis in dialysis

A

beta-2-microglobulin

386
Q

Adult patient presenting for extraction with severe bleeding, which may be an initial sign of:

A

von Willebrand disease

387
Q

Cytoplasmic antineutrophil antibody seen in

A

Wegener (GPA)

388
Q

Nuclear staining with beta catenin seen in

A

desmoid fibromatosis

389
Q

Which of the following is inherited in X-linked fashion

A

Hemophilia A

390
Q

WHich of the following is a manifestation of Crohn disease

A

linear ulceration

391
Q

Which of the following presents with skip lesions of the colon

A

Crohn disease

392
Q

Eosinophilic anucleated squamous like cells on all slides during a day may be from

A

dandruff LOL is this a real question??

393
Q

Formication is meth addicts with

A

thinking there are bugs on them

394
Q

Which has fruiting bodies

A

aspergillosis

395
Q

Demitaceous fungal organisms seen in

A

allergic fungal sinusitis

396
Q

Which of the following has increased risk for mucormycosis

A

hemochromatosis

397
Q

Mycosis fungoides is a disease of which cells

A

Helper T lymphocytes

398
Q

Pautrier abscesses are seen in

A

cutaneous T cell lymphoma (mycosis fungoidies)

399
Q

Facial paralysis in the setting of VZV is called

A

Ramsay Hunt Syndrome

400
Q

Anhydrosis, miosis, enopthalmos in a patient with an apical lung carcinoma is called

A

Horner syndrome

401
Q

Most common CNS lymphoma

A

diffuse large B cell lymphoma

402
Q

Lesion in spleen positive with podoplanin

A

lymphangioma

403
Q

Cardiac myxomas seen in

A

carney complex

404
Q

Bilateral acoustic schwannomas

A

NF2

405
Q

Most common non-odontogenic oral/facial cyst

A

nasopalatine duct cyst?

406
Q

Most common cause of granulomatous gingivitis

A

implantation of dental abrasives

407
Q

Which is true regarding Graves

A

Immunoglobulins against TSH receptors
Graves’ disease, one of the autoimmune thyroid diseases, is caused by the production of IgG autoantibodies directed against the thyrotropin receptor. These antibodies bind to and activate the receptor, causing the autonomous production of thyroid hormones

408
Q

Cell of origin for a granular cell tumor

A

schwann cells

409
Q

Electron micrographs of a granular cell odontogenic tumor show that the granules are

A

lysozymes

410
Q

Reed-Sternberf cell in Hodgkin is what origin?

A

B cell

411
Q

Michaelis-Guttman bodies are seen in

A

malakoplakia

412
Q

Pink teeth during autopsy may be caused by

A

The post mortem pink tooth is a common finding related to cases of asphyxia, such as strangulation, drowning or suffocation

413
Q

Odontogenic tumor most likely at multiple sites

A

squamous odontogenic tumor

414
Q

1st president of the ABOMP

A

so mean: Henry Goldman, DMD?

415
Q

Concept of a globular maxillary cyst is no longer in favor because

A

most have proven to be OKCs

416
Q

SCC occuring in a branchial cleft cyst is most likely

A

met SCC to a cervical LN

417
Q

Paramedian lip pits

A

clefting: kabuki, popliteal-pterygium, and van der woude

418
Q

commisure lip pits also associated with

A

auricular pits

419
Q

tests ordered for suspecting Wegeners (GPA)

A

cANCA (PR3-anca) and pANCA (mPo-anca)

420
Q

Hyaline membrane disease cause

A

deficiency of pulmonary surfactant in an immature lung

421
Q

Swiss cheese holes processing artifact

A

Freezing artifact

422
Q

End point decalcification - determining methods

A

Three methods used to assess decalcification.

Physical method of decalcification (least accurate)
* Visual assessment
* Manual Testing - test for tissue’s flexibility and hardness usinf fine probe

X-ray method method of decalcification (most accurate)

Chemical method of decalcification - use of chemical solutions test for residual calcium (accurate)

422
Q

Drug for pemphigus, pemphigoid, and b cell lymphoma

A

Rituximab, veltuzumab?
CHOP therapy?

423
Q

Rituximab mechanism of action

A

Monoclonal antibody that targets CD20
By targeting CD20, rituximab promotes cell lysis while sparing hematopoietic and plasma cells without this surface antigen
Cell lysis mechanisms - Complement-dependent cytotoxicity (CDC) and antibody-dependent cell-mediated cytotoxicity (ADCC)

424
Q

COVID most common oral manifestation

A

Taste alteration - loss of taste?
Others - ulcer, erosion, bulla, vesicle, pustule, fissured or depapillated tongue, macule, papule, plaque, pigmentation, halitosis, whitish areas, hemorrhagic crust, necrosis, petechiae, swelling, erythema, and spontaneous bleeding

425
Q

Similarity between covid and Kawasaki

A

An increasing number of COVID-19 cases in children have been associated with a new multisystem inflammatory syndrome. Its clinical features and laboratory characteristics are similar to those of Kawasaki disease (KD), KD shock syndrome, and toxic shock syndrome

426
Q

Calceneurin mechanism

A

Calcineurin inhibitors exert their immunosuppressive effects by reducing interleukin-2 (IL-2) production and IL-2 receptor expression, leading to a reduction in T-cell activation

Calcineurin (CaN) is a calcium and calmodulin dependent serine/threonine protein phosphatase (also known as protein phosphatase 3, and calcium-dependent serine-threonine phosphatase). It activates the T cells of the immune system and can be blocked by drugs

427
Q

Mantle zone lymphoma

A

** Cyclin D1 **
Classical immunophenotype of MCL is CD20-positive/ CD5-positive/ cyclin D1-positive/ CD23-negative/ CD10-negative.

428
Q

Imatinib used to treat

A

Certain types of cancer (such as acute lymphoblastic leukemia, chronic myeloid leukemia, gastrointestinal stromal tumors, and myelodysplastic/myeloproliferative diseases)

429
Q

Best pain killer after oral surgery

A

Acetaminophen and Ibuprofen????

430
Q

Oral amyloidosis type of amyloid

A

Light chain

431
Q

Water fixation artifact

A
432
Q

Denosumab (Xgeva)

A

Denosumab prevents RANKL from activating its receptor, RANK, on the surface of osteoclasts and their precursors. Prevention of the RANKL/RANK interaction inhibits osteoclast formation, function, and survival, thereby decreasing bone resorption and increasing bone mass and strength in both cortical and trabecular bone

433
Q

most frequent hematologic manifestations

A

anemia, leukopenia, lymphopenia, thrombocytopenia, lymphadenopathy, and splenomegaly

434
Q

Mucopolysaccharidosis most common oral manifestation

A
  • Oral manifestations vary according to types
    ○ Some degree of macroglossia
    ○ Gingival hyperplasia may be present, particularly in anterior regions, as a result of drying and irritating effects of mouth breathing
    ○ Dental changes = thin enamel with pointed cusps on posterior teeth
    § Seem to be a unique feature to mucopolysaccharidosis type IVA
    ○ Numerous impacted teeth with prominent follicular spaces, possibly caused by accumulation of glycosaminoglycans in follicular connective tissue
    Reports of multiple impacted teeth that are congregated in single large follicle, forming rosette pattern radiographically
435
Q

Glucocerebrosidase deficiency

A

Gaucher disease

436
Q

Erlenmeyer flask and bone pain which disease

A

Gaucher disease

437
Q

Gleason score what the numbers mean

A

Gleason X: The Gleason score cannot be determined.

Gleason 6 or lower: The cells look similar to healthy cells, which is called well differentiated.

Gleason 7: The cells look somewhat similar to healthy cells, which is called moderately differentiated.

Gleason 8, 9, or 10: The cells look very different from healthy cells, which is called poorly differentiated or undifferentiated.

438
Q

Fibrotic bands buccal mucosa but no submucous fibrosis as option

A

Submucosal Fibrotic Bands in Oral Lichen Planus?

439
Q

DSPP which conditions

A

Dentinogesis imperfecta

440
Q

Complication of using immune checkpoint inhibitors

A

Widespread inflammation
Fatigue, headache, arthralgia, rash, pruritus, pneumonitis, diarrhoea and/or colitis, hepatitis and endocrinopathies

441
Q

monotypic salivary gland neoplasm

A

Monophasic?
Polymorphous adenocarcinoma vs Canalicular adenoma

442
Q

Cetuximab mechanism of action

A

Cetuximab is a monoclonal antibody that binds specifically to the EGFR on both normal and tumour cells to competitively inhibit the binding of epidermal growth factor (EGF) and other ligands that are produced by normal and tumour tissue epithelial cells

443
Q

Premalignant oral condition with highest rate of transformation
(oral submucous vs PVL?)

A
444
Q

Which genetics technique includes microwaving

A

microwave irradiation

445
Q

If tumor stage is like rT3N2bM0 what do the lower case letters mean

A

Each of the T, N, and M categories might be written with a lowercase letter in front of it, showing if it’s a clinical or pathological classification:

A clinical stage is noted with “c” (for example, cT1)
A pathological stage is noted with “p” (for example, pN2)
For staging that is done after treatment or after cancer recurrence/progression, a category might also be given another lowercase letter in front of it:

For cancers that are restaged after neoadjuvant therapy (or other therapy), a “y” might be used in front of the category (for example, ycT1 or ypT2)
For cancers that are restaged after recurrence or progression of the cancer, an “r” might be used in front of the category (for example, rcT1 or rpT2)

446
Q

Most common head and neck location for carcinoid tumor

A

Both typical and atypical carcinoid tumours commonly involve the supraglottic compartment of the larynx, whereas small cell neuroendocrine carcinoma typically has a more homogeneous involvement of the larynx

447
Q

How to ddx Merkel cell from other small round blue cell with IHC

A

CK20
“Perinuclear dot” pattern

448
Q

Non-smoking young woman what kind of lung cancer

A

Lung cancer in non-smokers is almost exclusively non-small cell lung cancer, with adenocarcinoma being the most common type

449
Q

Which salivary gland tumor can be ciliated or warthin like

A

warthin-like mucoep or ciliated mucoep

450
Q

Follicular adenoma vs Follicular adenocarcinoma

A

A follicular carcinoma cannot be distinguished from a follicular adenoma based on cytologic features alone. It is distinguished from a follicular adenoma on the basis of capsular invasion, vascular invasion, extrathyroidal tumor extension, lymph node metastases, or systemic metastases

451
Q

Eosinophilic esophagitis findings on endoscopy

A

GERD?

452
Q

Necrotic hepatocytes

A

Hepatic necrosis is defined as death of hepatocytes, which maybe single cell, multiple cells in piecemeal, focal, multifocal, submassive or massive

453
Q

Intrahepatic cause of cirrhosis

A

Intrahepatic causes of portal hypertension include cirrhosis and hepatic fibrosis or scarring
Alcohol abuse,
Hepatitis B and C infections,

454
Q

Most common bladder cancer

A

Urothelial carcinoma, also known as transitional cell carcinoma (TCC), is by far the most common type of bladder cancer.

455
Q

What kind of epithelium is used for dx of paraneoplastic pemphigus

A

rat urinary bladder mucosa (rich expression of plakins)

456
Q

Hodgkin associated with which virus

A

EBV

457
Q

Ddx adenoid cystic from basaloid squamous ca

A

In addition to certain histological features, such as the absence of bi-layered structures, the presence of necrosis, and/or the presence of prominent cellular atypia, diffuse immunopositivity for p63/p40, p53 and Ki-67 and negativity for p16 are useful for differentiating BSCC from AdCC and HMSC

458
Q

GIST

A

Most common primary mesenchymal neoplasm of GI tract
Usually older adults (median age: 60 years)
Stomach and small bowel most common sites
Extremely broad and variable morphologic spectrum
Tumors are spindled, epithelioid, or mixed type
Characteristic CD117 and DOG1 (+)

459
Q

Nasopharyngeal ca which virus

A

EBV

460
Q

Spitz

A

HRAS
Skin of extremities or face during childhood
Solitary, dome-shaped, pink to reddish-brown papule
< 6mm in greatest diameter
Young age and small size
Kamino body

461
Q

HRPT which protein is defective

A

Parathyroid hormone-related protein?
MEN1?

462
Q

Schwannoma histo description

A
463
Q

GNAS1

A

fibrous dysplasia

464
Q

GNAS stands for

A

GNAS stands for Guanine Nucleotide binding protein, Alpha Stimulating activity polypeptide

465
Q

Eosinophilic angiocentric sclerosis

A

Eosinophilic angiocentric fibrosis (EAF) is an exceedingly rare and potentially disfiguring and obstructing benign lesion involving the upper airways
Nasal mass biopsy. Thick collagen bundles with perivascular fibrosis in an “onion-skin” type whorling pattern. Eosinophil-rich inflammatory infiltrate is also present

466
Q

Drugs for hypophosphatasia

A

STRENSIQ® (asfotase alfa) is the first and only prescription medicine used to treat people with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP).
Attempts to treat by infusing alkaline phosphatase have been unsuccessful, presumably because enzyme functions within cell rather than in extracellular environment

467
Q

IHC for Gorham Stout

A

D2-40 stain to highlight lymphatic endothelium

468
Q

Histo description on Paget’s

A
  • Uncontrolled resorption and formation of bone
    • Resorptive phase,
      ○ Numerous hyperactive osteoclasts surround the bone trabeculae (enlarged with increased number of nuclei)
      ○ Highly vascular fibrous connective tissue replaces the marrow
      ○ Gradual conversion from pre-dominantly osteoclastic to osteoblastic activity
      ○ Osteoblasts form osteoid rims around bone trabeculae and bone lacks an organized lamellar pattern
      ○ Basophilic reversal lines, which indicate junction between alternating bone resorption and formation, result in “jigsaw puzzle” or “mosaic” appearance
    • Sclerotic phase
      Large masses of dense bone with prominent reversal lines
469
Q

SOX10 which salivary tumor

A

secretory carcinoma
canalicular adenoma

470
Q

ki-67 increased in which layer of OKC

A

Greater expression of proliferating cell nuclear antigen (PCNA) and Ki-67 in suprabasilar layer

471
Q

Syndromic OKC what’s upregulated

A

OKCs show overexpression of p53 and cyclin D1 (bcl-1) oncoprotein

472
Q

Esophageal varices most common cause

A

Scarring (cirrhosis) of the liver is the most common cause of esophageal varices

473
Q

Multiple trichoepitheliomas what syndrome

A

Brooke-Spiegler syndrome (CYLD cutaneous syndrome)

474
Q

Saponification

A

Saponification is the hydrolysis of an ester to form an alcohol and the salt of a carboxylic acid in acidic or essential conditions. Saponification is usually used to refer to the soap-forming reaction of a metallic alkali (base) with fat or grease

475
Q

Tongue necrosis can be a manifestation of

A

giant cell arteritis

476
Q

Stains for Rosai dorfman

A

S100 (+), CD1a (-), langerin (-)

477
Q

Stains for LCH

A

(+) CD-1a or CD-207 (langerin) or S100

478
Q

VEGF inhibitors

A
479
Q

Dermatitis herpetiformis

A

Dermatitis herpetiformis (DH) is a chronic, intensely itchy, blistering skin manifestation of gluten-sensitive enteropathy, commonly known as celiac disease

480
Q

Oral findings in tuberous sclerosis

A

Developmental enamel pitting on facial aspect of anterior permanent dentition in 50-100% of patients
Multiple fibrous papules
Predominantly on anterior gingival mucosa
Diffuse fibrous gingival enlargement - even those who are not taking phenytoin

481
Q

VHL and endolymphatic sac tumor

A

Associated with Von hippel lindau
Most arise within intraosseous portion of the endolymphatic duct / sac, with precursor lesions present in VHL patients

482
Q

Endolymphatic sac tumor characteristics and location

A

Embryologically, the sac derives from the neuroectoderm and is located on the posteromedial surface of the temporal bone

483
Q

Homer Wright rosettes but no olfactory neuroblastoma or retinoblastoma as options

A

medulloblastoma, supratentorial PNETs and pineoblastomas, Ewing

484
Q

Mdm2 and cdk4 which tumors

A

Osteosarc
Liposarc

485
Q

Which of the following markers is most useful to differentiate tubular / trabecular predominant basal cell adenoma from pleomorphic adenoma?

Beta catenin
Calponin
CMA
p63
S100

A

Beta catenin. Jo et al. reported that nuclear beta catenin staining can be present in 83% of basal cell adenomas. All adenoid cystic carcinomas (0/20) and pleomorphic adenomas (0/20) were negative. 4 of 5 basal cell adenomas had exon 3 CTNNB1 mutations (Am J Surg Pathol 2016;40:1143).