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
Globodontia (aka globe shaped teeth), hearing loss, and ocular colobomas are classic of what syndrome?
otodental syndrome
26
Papillary lesion of the soft palate DDX
1. squamous papilloma 2. verruciform xanthoma 3. Giant cell fibroma 4. Sialadenoma papilliferum
27
Munro's abscesses
psoriasis
28
Pautriers abscesses
Mycosis Fungoides
29
HLA-Cw6
psoriasis
30
Tissue culture pattern on histology
nodular fasciitis
31
DDX for mixed radiolucent-radiopaque lesion (6) (of course site and appearance play a factor)
CEOT Ossifying fibroma COC odontoma ameloblastic fibro-odontoma AOT
32
Late stage florid COD- how do you rule out Pagets disease?
There should be no expansion in COD and ALK phos levels are normal (elevated in Pagets)
33
variant of ameloblastoma with mixed radiolucent/radiopaque appearance that resembles BFOL
desmoplastic ameloblastoma
34
A patient is diagnosed with basal cell carcinoma of the gingiva, what is the more likely diagnosis
a misdiagnosed ameloblastoma
35
-unerupted tooth, usually canine -radiolucency frequently extends below the CEJ of the tooth -snowflake calcifications
AOT
36
PTCH1 gene mutation occurs in what 2 entities?
OKC/Gorlin syndrome and CEOT
37
driven snow calcification appearance
CEOT
38
Amyloid-like acellular amorphous material occurring in CEOT is what?
odontogenic ameloblast-associated protein (ODAM)
39
Clear cell histology DDX (5)
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
40
A defect or groove in the palate may be caused by what odontogenic neoplasm
central odontogenic fibroma
41
Ground substance in an odontogenic myxoma is composed of
GAGs: predominantly hyaluronic acid and chondroitin sulfate
42
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?
Osteogenesis imperfecta (brittle bone disease) -Osteogenesis imperfecta, ehlers-danlos, marfan syndrome
43
4 Radiographic hallmarks of osteogenesis imperfecta
1. osteopenia 2. bowing of long bones 3. multiple fractures 4. increased number of wormian bones
44
Increased number of intrasutural bones (wormian bones) in what two conditions
-cleidocranial dysplasia -osteogenesis imperfecta
45
What disease: Spontaneous and progressive destruction of 1 or more bones (give the 5 names please) What are the histo findings?
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
46
Many basophilic reversal lines within bone creating a characteristic jigsaw puzzle/mosaic appearance is characteristic histology for what?
paget's disease (osteitis deformans)
47
10 disorders with Multiple giant cell lesions (6 high yield)...What 2 entities have been associated with central giant cell granulomas in the jaw?
- **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**
48
What cell lines are affected by early post zygotic activating mutation in GNAS
-Melanocytes, endocrine cells, osteoblasts \*if late in embryogenesis only osteoblasts are affected
49
Monostotic fibrous dysplasia that affects the maxilla and adjacent bones is termed
cranio-facial fibrous dysplasia
50
3 Syndromes associated with polyostotic fibrous dysplasia
1. Jaffe-lichtenstein 2. Mazabraud 3. McCune Albright
51
early presenting sign of polyostotic fibrous dysplasia is pain and long bone deformity- the deformity is specifically called what? (3 names)
deformity of the proximal femur = shepards crook deformity, coxa vara, hockey stick deformity (causes leg bowing)
52
Clinical: anterior mandibular COD with progressive growth
expansive osseous dysplasia
53
WHat diseasE? - Diffuse fibro-osseous lesions of the jaws with prominent psammoma bodies - bone fragility - bowing/cortical sclerosis of the long bones
Gnatho-diaphyseal dysplasia GDD1 mutation
54
What disease? - Parathyroid adenoma or carcinoma - Ossifying fibromas of the jaws - Renal cysts - Wilms tumors
Hyperparathyroidism jaw tumor syndrome HRPT2 mutation
55
-Multifocal osteomas -Supernumerary teeth, impacted teeth, odontomas -adenomatous polyps
Gardner's syndrome APC gene chromo 5
56
Name 4 less frequently mentioned manifestations of Gardners syndrome
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
57
-Radiology: radiolucent nidus with possible central opacity "target-like" -Clinical: nocturnal pain relieved by NSAIDs
osteoid osteoma (should also be less than 1.5-2cm in size)
58
Clinical: Genital lesions, conjunctivitis, arthritis, geographic tongue What condition? What HLA? Etiology?
-Reiter's syndrome (reactive arthritis) -HLA-B27 -occurs following venereal disease or dysentery
59
Multiple chondromas are associated with what 2 diseases/syndromes
1. Ollier disease: sporadic chondromatosis with unilateral tendency 2. Maffucci syndrome: sporadic chondromatosis and soft tissue angiomas
60
What syndromes increase the risk for osteosarcoma
Paget's disease Li-fraumeni syndrome Hereditary retinoblastoma Rothmund-thomson syndrome
61
19 year old male Bx: Non specific vascular proliferation with fibrous CT and chronic inflammatory cells
Massive osteolysis Vanishing bone disease phantom bone disease gorham/gorham-stout disease
62
25 year old male HX of veneral disease and arthritis What is the term for associated genital lesions? associated HLA?
Balanitis circinata HLA-B27
63
5 year old female Other findings: Bone surrounding the teeth is lower denisty, hyperplasia of the soft tissue in the area DX? Histo findings?
Regional odontodysplasia (ghost teeth) Histo: enameloid conglomerates (also seen in amelogenesis imperfecta)
64
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? -
- Apert syndrome - Syndactyly - Cloverleaf skull
65
55 year old male recently with new diagnosis and management of HTN - DDX? - What medications may cause this presentation?
- Medication related gingival hyperplasia vs Leukemic infiltrate - (anti-epileptic)Phenyltoin, (immunosuppressant) cyclosporine, calcium channel blockers (amlodipine)
66
12 year old male What is the most likely diagnosis? What are these lesions? List all of the manifestations of this disease Mutation?
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)
67
- 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?
- Port wine stain (nevus flammeus) - 8-10% - Sturge Weber syndrome - GNAQ chromo 9; not inherited - Leptomeningeal angiomas, intellectual disability, contralateral hemiplegia, seizures
68
- Most likely diagnosis? - What is the cause of the lesions? - Associated genetics? - Histologic buzzword?
- multifocal epithelial hyperplasia (Heck's disease) - HPV 13 and 32 - HLA DR4 - Mitosoid cell
69
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?
- Molluscum contagiosum - poxvirus infection MCV - HIV/AIDS, darier disease, atopic dermatitis - Henderson paterson bodies
70
- 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?
- Dermatosis papulosa nigra - FGFR3, PIK3CA (actinic lentigo) - Leser-Trelat sign: rare phenomenon associated with internal malignancy - Irritated sebK aka Inverted follicular keratosis of Helwig
71
Biopsy of one of these lesions shows sebaceous hyperplasia - Name the associated syndrome - Name 3 other findings in this syndrome
Muirre-Torre Syndrome -visceral malignancies, sebaceous adenomas and carcinomas (shown in the image), keratoacanthomas
72
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
- 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
73
- 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?
-10-35% for typical leukoplakia, 83% for verruciform variants Mild: ~1-7% Moderate: ~4-11% Severe: 20-43%
74
This patient has been using smokeless tobacco for 10 years. - What histologic features would be seen on biopsy? - Clinical recommendations?
1. hyperkeratotic and acanthotic - intraceullar vacuoles - parakeratin chevrons - amorphous subepithelial material in the superficial CT \*dysplasia is uncommon 2. Habit cessation- lesion should go away in ~ 2 weeks, if persists 6 weeks+ then it should be biopsied
75
- Name 3 inherited syndromes associated with multiple of the lesion depicted in this image - Name one acquired cause
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)
76
Multiple basal cell carcinomas are common in what inherited genetic disorders?
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
77
DX?
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.
78
Most commonly associated syndrome with this clinical presentation? What other syndromes also have this lesion?
Port wine stain (nevus flammeus) - Sturge Weber syndrome - Beckwith-Weidemann syndrome
79
What is the term for this clinical presentation? What are 5 causes?
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
80
Most likely diagnosis? - Key radiographic features - Key non-radiographic features - What may have a similar presentation and confuse you
Segmental odontomaxillary dysplasia - missing premolar, vertical oriented coarse bony trabeculae, smaller maxillary sinus - Becker nevus, gingival hyperplasia in the area - Fibrous dysplasia
81
All lesions with BRAF mutations
Some ameloblastomas 50% of Langerhan cell histiocytosis Melanoma Acquired melanocytic nevus
82
**The HLAs:** - Cw6 - B27 - DR4 - DR3/B8 - DRw52 - B51 - DRB1
Cw6= psoriasis (geographic tongue) B27= Reiters syndrome DR4= Hecks disease DR3/B8= primary sjogrens DRw52=primary or secondary sjogrens B51= Behcets BRB1=Rheumatoid Arthritis
83
Mikulicz cells Mikulicz disease Mikulicz syndrome Mikulicz aphthae
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
84
Things to see with a wood's lamp
- 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
85
_Some serologies:_ Diffuse cutaneous systemic sclerosis Limited cutaneous systemic sclerosis (includes CREST) Paget's disease Lupus Erythematous
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
86
DX?
Lichen planus
87
2 clinical syndromes of Acute Sarcoidosis
Lofgren syndrome: Erythema nodosum Bilateral hilar lymphadenopathy Arthralgia Heerfordt syndrome (uveoparotid fever) Parotid enlargement Anterior uveitis Facial paralysis Fever
88
What is the term for this histologic finding? Diagnosis?
Asteroid body of sarcoidosis -entrapped fragments of collagen within the granuloma
89
DX? Name of the entity depicted?
Sarcoid granuloma -Schumann body
90
What is the official / "more appropriate" name for the pattern seen on sialography of Sjogren's Syndrome?
punctate sialectasia (fruit-laden branchless tree)
91
Most likely diagnosis? What is the etiology? What is the clinical term applied to these lesions?
Bullous impetigo Typically staph aureus only Lacquer
92
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?
Non bullous impetigo DDX: HSV, exoliative chelitis(though not really in this picture) Corn flakes glued to the surface of the lesion
93
What is the most likely diagnosis? What is the etiology? What two characteristic clinical terms are applied to this lesion?
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
94
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?
Impetigo Ecthyma The lesion heals with a permanent scar- scarring is not expected in other lesions of impetigo either bullous or nonbullous
95
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?
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
96
Name that outdated and terrifying testing method: Sterilized sarcoid tissue is injected into the skin of a patient
Kveim test
97
Olfactory neuroblastoma: 1. Name for low grade pseudo-rosette 2. Name for high grade true rosette
1. pseudo-rosette: Homer-Wright 2. true-rosette: Flexner-Wintersteiner
98
Diagnosis?
Congenital syphilis
99
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?
Argyll Robertson pupils Tertiary syphillis
100
Loss of coordination of movement in tertiary syphillis is termed?
tabes dorsalis
101
What is the diagnosis? What other 2 clinical findings are part of the Hutchinson Triad?
Hutchinson teeth of Congenital syphilis -Eigth nerve deafness, interstitial keratitis
102
BUGS summary: ## Footnote 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
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
103
This patient has anesthesia and loss of sweating of the overlying skin in these lesions. Biopsy demonstrated well formed granulomatous inflammation Diagnosis?
Tuberculoid aka paucibacillary leprosy
104
Diagnosis? What is the term for the characteristic facies? What would you expect to see on biopsy?
Lepromatous (multibacillary) leprosy - Leonine facies - Sheets of lymphocytes with vacuolated histiocytes (aka lepra cells) - No well formed granulomas
105
What is the general term for this clinical presentation? What is the differential diagnosis?
Macrocheilia
106
Children with red teeth DDX
Congenital erythropoietic porphyria aka Gunther disease Leprosy
107
What is the name for the cells depicted in this image? What is the diagnosis?
Lepra cells of lepromatous leprosy- they are vacuolated macrophages
108
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?
Pastia lines Scarlet fever -White strawberry tongue then red strawberry tongue
109
Bacterial species of chronic periodontitis red complex
- Treponema denticola - Tannerella forsythensis - Porphyromonas gingivalis
110
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?
- Actinomycosis - mimicked by Botryomycosis (usually caused by staph aureus) - Definitive diagnosis is Actino
111
This patient also has hypoparathyroidism and hypoadrenocorticism. Diagnosis? Associated genetic mutation?
APECED: autoimmune polyendocrinpathy-candidasis-ectodermal dystrophy syndrome - mucocutaneous candidiasis, nail dystrophy, corneal keratopathy, hypoparathyroid/hypoparathyroid - childhood onset - Mutation in AIRE gene
112
These are two different presentations of the same disease. What is the terminology used to describe each image? What is the diagnosis?
Luetic glossitis = atrophic form Interstitial glossitis = gummatous form Tertiary syphilis
113
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?
**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)
114
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?
**-Gingival Fibromatosis** ## Footnote - 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)
115
High yield genetic disorders associated with periodontitis in kids/young adults What is the specific terminology applied to this?
**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
116
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. Actinomycetemcomitans (**Aggregatibacter actinomycetemcomitans) **-Papilon Lefevre** Bacteria types expand when patients have generalized type and include the red complex
117
What is the term for the histologic feature seen in this image? What conditions/diseases can this be found in?
Hamazaki-Wesenberg Bodies aka yellow-brown bodies myriad medical conditions: appendicitis, cirrhosis, lymphoid tumours, colon carcinoma and numerous others, most famously sarcoidosis
118
**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
_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)
119
Direct immunofluorescence pattern for Chronic ulcerative stomatitis? Indirect immunofluorescence finding?
- speckled pattern of IgG deposits in the basal one-third of the epithelium - Indirect: presence of stratified epithelium specific anti-nuclear antigen (SES-ANA)
120
What is the characteristic cell depicted here? What is this diagnostic of?
Floret cell Pleomorphic lipoma
121
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
SAPHO syndrome Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis
122
Multiple bone involvement by primary chronic osteomyelitis in children is termed
CRMO: Chronic recurrent multifocal osteomyelitis thought to be a widespread variant of primary chronic osteomyelitis
123
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?
- 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 -
124
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?
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
125
DX?
Calcific metamorphosis and aseptic pulpal necrosis
126
What are the two major clinical findings? What two conditions produce these radiographic findings?
- Thistle tube/flame shaped pulp chambers & pulp stones - Dentin dysplasia type 2 and pulpal dysplasia
127
What termed is applied to this radiographic finding? What is the diagnosis? What is the histologic description of the root of these teeth?
Rootless teeth Dentin dysplasia type 1 Histo buzz: - whorls of tubular dentin - “stream flowing around boulders”
128
What two pathoses can cause a "bull neck" appearance
Diptheria Ludwigs angina
129
**Whats my keratin?** Epidermolysis Bullosa simplex White sponge nevus Pachyonychia congenita Oral lesions of pachyonychia congenita Pachyonychia congenita with neonatal teeth
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
130
At least 4 conditions associated with premature tooth loss
Acrodynia (pink disease) mercury exposure children Hypophosphatasia Dentin Dysplasia type 1 (rootless teeth) Papilon Lefevre Haim Munk Acatalasia Cohen Syndrome Ehlers-Danlos
131
What clinical features are depicted in this image? What is the most likely diagnosis? What mutation and inheritance pattern is associated with this condition?
-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
132
Time to dominate Lipid Reticuloendothelioses.... What are the three diseases & their subtypes?
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)
133
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?
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
134
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?
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
135
Tay-Sachs: How does it compare to the other lipoidendoreticuloses? What enzyme is missing? What accumulates? What cell type is affected?
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)
136
Infant with bilateral facial swellings, fever, leukocytosis, and hyperostosis of his facial bones. Blood cultures negatve. What's the most probable diagnosis?
Caffey Disease (AD, a COL1A1 mutation has been found)
137
Name the skin lesion...What systemic condition is it associated with?
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.
138
What are the 3 main components of RAMON syndrome?
1. Cherubism 2. Gingival fibromatosis 3. seizures
139
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?
- Multiple large congenital nevi - Neurocutaneous melanosis - Potentially fatal condition in which patients have multiple or large congenital nevi + melanocytic neoplasms of the CNS (melanomas)
140
What is the term for areas indicated by the yellow arrows? What diagnosis is this feature specific for? What mutation is associated?
- Kamino bodies - Spitz nevus - HRAS
141
- 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?
- 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%)
142
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?
- 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
143
This biopsy is from the posterior lateral tongue. Diagnosis? - is this a neoplastic process?
- 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+
144
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?
- 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
145
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?
Intravascular papillary endothelial hyperplasia (Massons tumor) - Non neoplastic/reactive process - Dilated vascular spaces, fibrin deposition, endothelial cell proliferation
146
6 year old patient, Diagnosis? - Characteristic finding on electron microscopy? - stains? What classification system is currently used?
- 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
147
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?
- Marfan syndrome - Fibrillin-1 - TGF-Beta - Autosomal dominant - MEN2B, NBCCS
148
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?
- 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
149
150
Diagnosis? Term for the foot manifestation? Oral findings?
- Arsenic poisoning - Black foot disease - necrotizing ulcerative stomatitis, excessive salivation,
151
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?
Chrysiasis: when patients are treated with gold therapy they can develop this slate blue pigmentation in areas exposed to sunlight -Oral mucositisi
152
What bacteria is most commonly responsible for Lemierre syndrome?
Fusobacterium necrophorum - usally occurs in teens - can be life threatening - basically an oropharyngeal infection that can lead to sepsis
153
A newborn is born with jaundice. If the baby is not treated these may develop ______ a dangerous accumulation of bilirubin in the brain
Kernicterus
154
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?
- 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
155
Diagnosis?
-Cholesteatoma
156
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?
- Frey's syndrome: results from injury of the salivary gland - Starch iodine test
157
Local causes of delayed tooth eruption Systemic causes of delayed tooth eruption
Local causes: gingival fibromatosis, clefts, ROD, SOD, supernumeraries, tumors Systemic: anemia, celiac, cerebral palsy, chemotherapy, endocrine disturbances, vit D resistant rickets
158
DX? What intraoral finding is found in 92% of these patients
Rubinstein-Taybi Talon cusp
159
Dens en dente aka dens invaginatus
160
DX? What might this be caused by?
buccal bifurcation cyst -cervical enamel extension
161
What syndrome would you suspect? What are the other associated findings?
PHACES P: posterior fossa brain anomalies H: Hemangioma A: arterial anomalies C: Cardic defects E: Eye anomalies S: sternal cleft, supraumbilical raphe
162
What is the term for this finding? Name an associated syndrome?
sternal cleft PHACES
163
What is the term for this radiographic finding? What is it characteristic of?
tram-track or tram-line calcifications Sturge weber syndrome
164
Salt fish diet --\> Leather and wood-working --\>
salt fish = sinonasal adenocarcinoma leather and wood-working = intestinal type sinonasal adenocarcinoma
165
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?
Juvenile recurrent parotitis Usually happens around 3-6 yrs old, multiple episodes, seld resolves by puberty -Worldwide: Mumps, in the USA: Juvenile recurrent parotitis
166
DX?
Cheilitis glandularis
167
What conditions can produce complaints of excess drooling? If there is no obvious cause, and they get episodic sialorrhea, what is the term?
- cerebral palsy, down syndrome, ALS, parkinsons, heavy metal poisoning, rabies - idiopathic paroxysmal sialorrhea
168
Rose bengal dye test is used for? What other test is used for the same purpose?
- diagnosis of keratoconjunctivitis sicca - Schirmer test
169
Difference between Epstein Pearl vs Bohn Nodule vs Ging Cyst of Newborn
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
170
Conditions that affect the nails (8)
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
171
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?
osteoporosis circumscripta- occurs in early stage disease
172
What 3 disease cause Forcheimer spots
German measles (rubella) Scarlet fever Mono
173
Lesion is firm BX demonstrates benign appearing bone and cartilage
Cutright lesion AKA Reactive osseous and chondromatous metaplasia
174
Diagnosis? Recommended therapy?
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)
175
DX? Tx?
DX: Exfoliative chelitis TX: 1. Elimination of habit 2. Tx of any superimposed infection: antiobiotic and or antifungal 3. topical tacrolimus
176
DX? TX?
DX: transient ligual papillitis TX: self resolving though you can give topical corticosteroids or analgesics for discomfort
177
Differential DX? Ancillary testing to confirm dx?
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
178
Describe the finding being depicted in this image. What systemic condition(s) is this typical of?
Loss of lamina dura- generalized - Hyperparathyroidism, osteomalacia, pagets disease, fibrous dysplasia
179
In addition to the finding depicted this male child has the following bloodwork: ## Footnote - Hypophosphatemia - Low serum calcium What is the most likely diagnosis? What characteristic finding is seen on histology of one of these teeth?
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
180
What is this image depicting and where is this commonly found?
Hyaline body aka pulse granuloma Walls of inflammatory cysts
181
Metastasis to the mandible that is PAX8 and TTF1:
thyroid
182
Cherry-red spot on the macule is associated with:
Tay-Sachs (Lysosomal storage disorders, retinal ischemia, and retinal infarction)
183
Negri bodies are associated with:
rabies
184
T1 SCC with tumor thickness < 3mm, which may have you do a neck dissection and radiation:
185
Which of the following is associated with NUT-something:
NUT midline
186
PAX-FOX01 seen in:
alveolar rhabdomyosarcoma
187
EWS is paired with which gene in Ewing sarcoma:
FLI1
188
EWSR1-ATF1 is seen in which two:
clear cell sarcoma and clear cell odontogenic carcinoma
189
What is the female equivalent of a seminoma:
dysgerminoma
190
Which subtype of rhabdo has a specific translocation:
alveolar
191
Most common nasal septum papilloma:
fungiform
192
Nasal papilloma most likely to be malignant:
inverted
193
Nasal papilloma that is destructive and malignant:
inverted
194
Most common nasal disease seen in family practice:
rhinitis
195
Patient with red face and bumps that goes away with tetracycline:
rosacea
196
STAT6 in which soft tissue tumor:
solitary fibrous tumor
197
Most likely to have Flexner-Wintersteiner rosettes:
olfactory neuroblastoma
198
Chromosome for nevoid basal cell carcinoma syndrome:
9
199
Pierre-Robin is seen with:
Velocardiofacial syndrome (Digeorge) Stickler syndrome
200
Most common lesion in a person with Beckwith-Wiedemann
Wilms (Wilms tumor, adrenal carcinoma, hepatoblastoma, rhabdomyosarcoma, neuroblastoma )
201
Best site for FNA:
soft palate swelling (other answers were epithelial)
202
Which amelogenesis imperfecta is scattered in primary: (scattered pits?)
Hypoplastic? Other variants - hypocacification, hypomaturation and AI with taurodontism
203
Which symptom associated with DSPP mutation
Hearing loss (vs dysosmia etc)
204
Old male patient with renal failure, lumbar pain, and IgG spike
multiple myeloma
205
Which is true about thyroglossal duct cyst
**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
Which has perinuclear eosinophilic condensation
white sponge nevus
207
Genodermatosis with autosomal recessive and problems with DNA repair
xeroderma pigmentosum
208
Most common salivary neoplasm on lower lip
?? Mucoep
209
Most common thyroid carcinoma in ectopic thyroid sites
PTC
210
Dental manifestation of oral-dento-facial-cardio syndrome
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
OKCs in NBCCS have upregulation of
p53
212
Most common location for lateral periodontal cyst
mandibular canine/premolar (Mandibular premolar-canine-lateral incisor area)
213
Most common presentation of orthokeratinizing odontogenic cyst
unerupted molar
214
Most common odontogenic cyst in anterior mandible
glandular odontogenic cyst
215
Most likely variant of ameloblastoma to present as RL/RO
desmoplastic
216
Best stain for peripheral ameloblastoma or intraoral BCC
Ber-EP4
217
Second "hit" of double-hit in colorectal cancer
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
Which is most likely to be trisomy
Patau Down Syndrome (Trisomy 21), Edwards Syndrome (Trisomy 18), Patau Syndrome (Trisomy 13), Klinefelter Syndrome (XXY Syndrome)
219
Most common thing with Chtovek sign:
hypocalcemia
220
Facial paralysis, fever, and serum/urine calcium; which chronic granulomatous most likely with these
sarcoid
221
Best test for lingual thyroid
99-technitium scan
222
Which is not a required feature for Sjogren
scarring alopecia
223
Parabasal/basal and speckled DIF
chronic ulcerative stomatitis
224
Which antibody for chronic ulcerative stomatitis
delta-NP63-alpha
225
Chicken-wire pattern of DI
pemphigus
226
Shaggy deposition of fibrinogen at basement membrane with cytoid bodies:
lichen planus
227
Lichen planus can sometimes be seen in association with:
superficial mucoceles
228
GFAP staining peripheral of which salivary neoplasm
PA
229
Which is associated with HLA-DR4:
prurigo (Hecks and giant cell arteritis not on the list)
230
Laryngeal papillomatosis is associated with:
HPV6, 11
231
Osteogenic osteomalacia is most often seen with:
phosphaturic mesenchymal tumor
232
Which are associated with HPV:
E7 (E6 not on the list)
233
Which is true regarding AOT:
well-circumscribed RL (the others made less sense)
234
Lyre is associated with:
carotid body paraganglioma
235
Most likely site for saddle embolism to come from:
leg/lower extremities
236
Odontohypophosphatasia is most often associated with:
stress fractures and premature loss of adult dentition
237
USP6 can be used to identify which spindle cell tumor:
nodular fasciitis
238
USP6 is rearranged in which bone tumor:
aneursymal bone cyst
239
Example of transient neoplastic disease:
nodular fasciitis
240
Most common soft tissue location for metastatic tumor:
gingiva
241
Which is associated with NKX3.1:
prostate
242
Which site is most likely to have dysplasia or early invasive carcinoma on biopsy:
floor of mouth? high risk sites - lateral tongue?
243
Most common soft tissue tumor in children:
rhabdomyosarcoma
244
Leser-Trelat sign is associated with onset of multiple:
seborrheic keratoses
245
Most common head and neck site for osteoma:
frontal sinus
246
Loose bodies are seen in:
synovial chondromatosis
247
Rhomboid bodies seen in:
gout (buzzword: needle shaped crystals)
248
Patient with trismus, no radiographic changegs, acellular dense fibrous tissue
oral submucous fibrosis (scleroderma on differentiatial)
249
Bartonella causes cat scratch
bacillary angiomatosis
250
Lyme disease is caused by
Borrelia burgdorferi Rarely, Borrelia mayonii
251
Most common mutation in melanoma:
BRAF
252
Which is true regarding PGCG:
occurs on ridge
253
Fibrous dysplasia may mimic which type of osteosarcoma:
fibroblastic?
254
Cemento-osseous dysplasia is often seen with:
simple bone cyst?
255
Which of the following has an autosomal dominant pattern of inheritance with genetic imprinting:
paraganglioma
256
Which of the following defines epigenetic
methylation of DNA
257
Which of the following stains calcium
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
Cochineal beetles are related to oral pathology because
eosin dye
259
Which stains oncocytomas
PTAH
260
False positives in TB testing:
vaccination with BCG
261
Who will most likely get parotid lymphoepithelial cysts
HIV patients
262
Which is true about giant cell arteritis
Sudden blindness (alt: seen in women or men) Can also have tongue necrosis
263
Which odontogenic neoplasm can have clear cell changes
CEOT
264
Spheroid bodies with Maltese cross
265
Hereditary angioedema is the result of:
C1-INH enterase deficiency
266
Metastasis to the mandible that is PAX8 and TTF1
Thyroid
267
Cherry-red spot on the macule is associated with
Tay-Sachs
268
Negri bodies associated with
rabies
269
T1 SCC with tumor thickness <3mm, which may have you do a neck dissection with radiation?
Not sure what they're going for here. Just look up SCC staging
270
Which of the following is associated with NUT-something?
NUT midline
271
PAX-FOX01 seen in
alveolar rhabdomyosarcoma
272
EWS paired with which gene in Ewing Sarcoma?
FLI1
273
EWSR1-ATF1 seen in which 2 entities?
clear cell sarcoma and clear cell odontogenic carcinoma
274
Female equivalent of seminoma
dysgerminoma
275
which subtype of rhabdomyosarcoma has a specific translocation?
alveolar
276
most common nasal septum papilloma
fungiform
277
nasal papilloma most likely to be malignant
inverted
278
nasal papilloma that is destructive and malignant
inverted
279
most common nasal disease seen in family practice?
rhinitis
280
Patient with red face bumps that go away with tetracycline?
rosacea
281
STAT6 for which soft tissue tumor
solitary fibrous tumor
282
Flexner-Wintersteiner rosettes
olfactory neuroblastoma
283
chromosome for nevoid basal cell carcinoma syndrome? mutation?
9...patch PTCH1
284
Pierre-Robin sequence seen with which syndrome?
velocardial facial syndrome
285
Most common lesion in a person with Beckwith-Wiedemann syndreome?
Wilms tumor
286
Best site for an FNA
Soft palate swelling (other answers were epithelial)
287
Which amelogenesis imperfecta is scattered in primary
Hypoplastic AI often affects both primary and permanent, but can affect primary only, so maybe they are referring to this?
288
Which symptom associated with DSPP mutation?
hearing loss
289
Old dude with renal failure, lumbar pain, IgG spike
multiple myeloma
290
True about thyroglossal duct cyst:
midline presentation below the hyoid
291
Perinuclear eosinophilic condensation
white sponge nevus
292
Genodermatosis with autosomal recessive and problems with DNA repair
xeroderma pigmentosum
293
most common salivary neoplasm on lower lip
mucoepidermoid carcinoma (pg443 neville)
294
Most common thyroid carcinoma in ectopic thyroid sites
PTC
295
5 Most common dental maifestation in oculo-facio-cardio-dental syndrome
1. radiculomegaly 2. oligodontia 3. root dilacerations 4 malocclusion 5 delayed eruption
296
OKCs in NBCCS have up regulation of which gene
p53
297
Most common location for lateral periodontal cyst
mandibular canine / premolar
298
most common presentation of orthokeratinizing odontogenic cyst
unerupted molar
299
Most common odontogenic cyst in the anterior mandible
glandular odontogenic cyst
300
Most likely variant of ameloblastoma to present as mixed RL/RO
desmoplastic ameloblastoma
301
Best stain for a peripheral ameloblastoma or intraoral BCC
BerEP4
302
Second hit of double hit colorectal cancer
According to Robbins: Methylation abnormalities, inactivation of normal alleles: APC and B-catinin
303
Which is most likely to be trisomy?
Patau (trisomy 13)
304
Most common thing with Chtovek sign
hypocalcemia
305
Facial paralysis, fever, serum/urine calcium; which chronic granulomatous condition is most likely associated with these?
Sarcoid
306
Best test for lingual thyroid
99-technitium scan
307
What is NOT a required feature of sjogren?
scarring alopecia
308
Parabasal/basal and speckled DIF
CUS chronic ulcerative stomatitis
309
Which antibody is used for chronic ulcerative stomatitis?
delta-NP63-alpha
310
CHichen-wire pattern DIF
pemphigus
311
Shaggy deposition of fibrinogen at basement membrane with cytoid bodies
lichen planus
312
lichen planus can sometimes be seen in association with
superfical mucoceles
313
GFAP stainin peripheral of which salivary gland neoplasm?
PA
314
Which is associated with HLA-DR4
prurigo (Hecks and Giant cell arteritis not options
315
Laryngeal papillomatosis is associated with which HPV strains
6,11
316
Osteogenic osteosarcoma is most often seen with which type of tumor?
phosphturic mesenchymal tumor
317
which proteins are associated with HPV
E7 (E6 not an option)
318
Which is true regarding AOT:
well-circumscribed RL
319
Lyre is associated with
Carotid body paraganglioma )lyre sign = splaying of the internal and external carotid arteries
320
Most likely site for a saddle embolism to originate
leg
321
Odontohypophosphatasia is most often associated with
stress fractures and premature loss of adult dentition
322
USP6 can be used to identify which spindle cell tumor?
nodular fasciitis
323
USP6 is rearranged in which bone tumor?
Aneurysmal bone cyst
324
Example of transient neoplastic disease
nodular fasciitis
325
Most common soft tissue location for metastatic tumor
gingiva
326
Which is associated with NKX3.1
Prostate
327
Most specific marker for differentiating adrenal cortical carcinoma from a met?
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
Which site is most likely to have dysplasia or early invasive carcinoma on biopsy
floor of mouth
329
Most common soft tissue tumor in children
rhabdomyosarcoma
330
Leser-Trelat sign is associated with onset of multiple what?
seborrheic keratoses
331
Most common head and neck site for an osteoma
frontal sinus
332
loose bodies are seen in
synovial chondromatosis
333
rhomboid bodies are seen in:
gout
334
Patient with trisumus, no radiographic changes, acellular dense fibrous tissue
oral submucous fibrosis (but scleroderma also option)
335
Bartonella causes cat scratch and
bacilliary angiomatosis
336
Lyme disease is caused by
borrella burgdorferi
337
Most common mutation in melanoma
BRAF
338
Which is true regarding PGCG?
Occurs on the ridge
339
Fibrous dysplasia may mimic which type of osteosarcoma?
fibroblastic?
340
Cemento-osseous dysplasia is often seen with
simple bone cyst
341
Which of the following has an autosomal dominant pattern of inheritance with genetic imprinting?
Paraganglioma
342
Which of the following defines epigenetic?
methylation of DNA
343
Which of the following stains calcium
von Kossa
344
Cochineal beetles are related to oral pathology because
eosin dye
345
Which stains oncocytomas?
PTAH
346
False positives in TB testing
vaccination with BCG
347
Who will most likely get parotid lymphoepithelial cysts?
HIV
348
Which is true about giant cell arteritis?
sudden blindness (or seen in women or men - women are affected 2x more)
349
Which odontogenic neoplasms can have clear cell changes?
CEOT
350
Spheroid bodies with Maltese cross
High urinary protein nephrotic syndrome /Fabry disease vs babesiosis aka babesia microti (tick borne illness)
351
Ferruginous bodies seen in
asbestos fibers
352
Electric cautery of acinar cells in parotid glands can make them seem like
spindle cells
353
Increased risk of oral cancer can be seen in
dyskeratosis congenita
354
Wernicke-Korsakoff in alcoholics caused by deficiency in
thiamine (B1)
355
Vitamin A deficiency associated with
blindness
356
Which is true about a blue nevus
tyndall effect
357
Which is true about a halo nevus
hyperpigmented lesion surrounded by hypopigmentations
358
McCune-Albright genetic mutation
GNAS1
359
BRAF inhibitors may be used in the treatment of which odontogenic lesion
ameloblastoma
360
mutations in SH3BP2 seen in
cherubism
361
Which syndrome presents with cherubism-like CGCG-like leisons
Noonan syndrome
362
Which is the most common complication of a middle ear infection?
Cholesteatoma or acute mastoiditis (I'm going to favor mastoiditis thanks to google)
363
What is used in reverse transcriptase PCR?
cDNA
364
HHV8 associated with
KS
365
Hyaline globules associated with
KS
366
Kikuchi-Fujimoto disease AKA
necrotizing histiocytic lymphadenitits
367
Emperipolesis is seen in
massive sinus histiocytosis with lymphadenopathy (Rosai Dorfman)
368
Sarcomatoid differentiation can be seen in which salivary neoplasm
salivary duct carcinoma
369
Most common salivary duct carcinoma cell type
apocrine
370
What part of the body controls sleep and appetitie?
hypothalamus
371
Which is associated with myasthenia gravis
thymoma
372
Stage III and IV oropharyngeal carcinoma which have prognostic implications
both HPV and tobacco use
373
If a child has gingival bleeding and arthralia, consider:
vitamin c deficiency
374
MDM2 therapy and radiation therapy is novel treatment for
rhabdomyosarcoma
375
MUC4 is a specific marker for
low grade fibromyxoid sarcoma
376
what type of lung cancer is a mid age female non-smoker
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
Extravasated RBCs and fibrin in biopsy seen in
plasminogen deficiency
378
Biphasic salivary gland tumor
PA, epi-myoepi carcinoma, cribriform (?), Adenoid cystic ca
379
Progressive multifocal leukoencephalopathy is seen in
AIDS
380
Hypophosphatasia is associated with reduced levels of
alkaline phosphatase
381
Segmental odontomaxillary dysplasia may present with
hypertrichosis!
382
Which 2 markers are best for diagnosing a desmoplastic melanoma
HMB45 and S100
383
Which stain uses the same target as MART-1
Melan-A
384
Which marker is positive in PEComa
HMB45
385
Which is true regarding amyloidosis in dialysis
beta-2-microglobulin
386
Adult patient presenting for extraction with severe bleeding, which may be an initial sign of:
von Willebrand disease
387
Cytoplasmic antineutrophil antibody seen in
Wegener (GPA)
388
Nuclear staining with beta catenin seen in
desmoid fibromatosis
389
Which of the following is inherited in X-linked fashion
Hemophilia A
390
WHich of the following is a manifestation of Crohn disease
linear ulceration
391
Which of the following presents with skip lesions of the colon
Crohn disease
392
Eosinophilic anucleated squamous like cells on all slides during a day may be from
dandruff LOL is this a real question??
393
Formication is meth addicts with
thinking there are bugs on them
394
Which has fruiting bodies
aspergillosis
395
Demitaceous fungal organisms seen in
allergic fungal sinusitis
396
Which of the following has increased risk for mucormycosis
hemochromatosis
397
Mycosis fungoides is a disease of which cells
Helper T lymphocytes
398
Pautrier abscesses are seen in
cutaneous T cell lymphoma (mycosis fungoidies)
399
Facial paralysis in the setting of VZV is called
Ramsay Hunt Syndrome
400
Anhydrosis, miosis, enopthalmos in a patient with an apical lung carcinoma is called
Horner syndrome
401
Most common CNS lymphoma
diffuse large B cell lymphoma
402
Lesion in spleen positive with podoplanin
lymphangioma
403
Cardiac myxomas seen in
carney complex
404
Bilateral acoustic schwannomas
NF2
405
Most common non-odontogenic oral/facial cyst
nasopalatine duct cyst?
406
Most common cause of granulomatous gingivitis
implantation of dental abrasives
407
Which is true regarding Graves
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
Cell of origin for a granular cell tumor
schwann cells
409
Electron micrographs of a granular cell odontogenic tumor show that the granules are
lysozymes
410
Reed-Sternberf cell in Hodgkin is what origin?
B cell
411
Michaelis-Guttman bodies are seen in
malakoplakia
412
Pink teeth during autopsy may be caused by
The post mortem pink tooth is a common finding related to cases of asphyxia, such as strangulation, drowning or suffocation
413
Odontogenic tumor most likely at multiple sites
squamous odontogenic tumor
414
1st president of the ABOMP
so mean: Henry Goldman, DMD?
415
Concept of a globular maxillary cyst is no longer in favor because
most have proven to be OKCs
416
SCC occuring in a branchial cleft cyst is most likely
met SCC to a cervical LN
417
Paramedian lip pits
clefting: kabuki, popliteal-pterygium, and van der woude
418
commisure lip pits also associated with
auricular pits
419
tests ordered for suspecting Wegeners (GPA)
cANCA (PR3-anca) and pANCA (mPo-anca)
420
Hyaline membrane disease cause
deficiency of pulmonary surfactant in an immature lung
421
Swiss cheese holes processing artifact
Freezing artifact
422
End point decalcification - determining methods
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
Drug for pemphigus, pemphigoid, and b cell lymphoma
Rituximab, veltuzumab? CHOP therapy?
423
Rituximab mechanism of action
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
COVID most common oral manifestation
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
Similarity between covid and Kawasaki
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
Calceneurin mechanism
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
Mantle zone lymphoma
** Cyclin D1 ** Classical immunophenotype of MCL is CD20-positive/ CD5-positive/ cyclin D1-positive/ CD23-negative/ CD10-negative.
428
Imatinib used to treat
Certain types of cancer (such as acute lymphoblastic leukemia, chronic myeloid leukemia, gastrointestinal stromal tumors, and myelodysplastic/myeloproliferative diseases)
429
Best pain killer after oral surgery
Acetaminophen and Ibuprofen????
430
Oral amyloidosis type of amyloid
Light chain
431
Water fixation artifact
432
Denosumab (Xgeva)
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
most frequent hematologic manifestations
anemia, leukopenia, lymphopenia, thrombocytopenia, lymphadenopathy, and splenomegaly
434
Mucopolysaccharidosis most common oral manifestation
* 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
Glucocerebrosidase deficiency
Gaucher disease
436
Erlenmeyer flask and bone pain which disease
Gaucher disease
437
Gleason score what the numbers mean
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
Fibrotic bands buccal mucosa but no submucous fibrosis as option
Submucosal Fibrotic Bands in Oral Lichen Planus?
439
DSPP which conditions
Dentinogesis imperfecta
440
Complication of using immune checkpoint inhibitors
Widespread inflammation Fatigue, headache, arthralgia, rash, pruritus, pneumonitis, diarrhoea and/or colitis, hepatitis and endocrinopathies
441
monotypic salivary gland neoplasm
Monophasic? Polymorphous adenocarcinoma vs Canalicular adenoma
442
Cetuximab mechanism of action
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
Premalignant oral condition with highest rate of transformation (oral submucous vs PVL?)
444
Which genetics technique includes microwaving
microwave irradiation
445
If tumor stage is like rT3N2bM0 what do the lower case letters mean
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
Most common head and neck location for carcinoid tumor
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
How to ddx Merkel cell from other small round blue cell with IHC
CK20 "Perinuclear dot" pattern
448
Non-smoking young woman what kind of lung cancer
Lung cancer in non-smokers is almost exclusively non-small cell lung cancer, with adenocarcinoma being the most common type
449
Which salivary gland tumor can be ciliated or warthin like
warthin-like mucoep or ciliated mucoep
450
Follicular adenoma vs Follicular adenocarcinoma
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
Eosinophilic esophagitis findings on endoscopy
GERD?
452
Necrotic hepatocytes
Hepatic necrosis is defined as death of hepatocytes, which maybe single cell, multiple cells in piecemeal, focal, multifocal, submassive or massive
453
Intrahepatic cause of cirrhosis
Intrahepatic causes of portal hypertension include cirrhosis and hepatic fibrosis or scarring Alcohol abuse, Hepatitis B and C infections,
454
Most common bladder cancer
Urothelial carcinoma, also known as transitional cell carcinoma (TCC), is by far the most common type of bladder cancer.
455
What kind of epithelium is used for dx of paraneoplastic pemphigus
rat urinary bladder mucosa (rich expression of plakins)
456
Hodgkin associated with which virus
EBV
457
Ddx adenoid cystic from basaloid squamous ca
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
GIST
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
Nasopharyngeal ca which virus
EBV
460
Spitz
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
HRPT which protein is defective
Parathyroid hormone-related protein? MEN1?
462
Schwannoma histo description
463
GNAS1
fibrous dysplasia
464
GNAS stands for
GNAS stands for Guanine Nucleotide binding protein, Alpha Stimulating activity polypeptide
465
Eosinophilic angiocentric sclerosis
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
Drugs for hypophosphatasia
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
IHC for Gorham Stout
D2-40 stain to highlight lymphatic endothelium
468
Histo description on Paget's
* 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
SOX10 which salivary tumor
secretory carcinoma canalicular adenoma
470
ki-67 increased in which layer of OKC
Greater expression of proliferating cell nuclear antigen (PCNA) and Ki-67 in suprabasilar layer
471
Syndromic OKC what's upregulated
OKCs show overexpression of p53 and cyclin D1 (bcl-1) oncoprotein
472
Esophageal varices most common cause
Scarring (cirrhosis) of the liver is the most common cause of esophageal varices
473
Multiple trichoepitheliomas what syndrome
Brooke-Spiegler syndrome (CYLD cutaneous syndrome)
474
Saponification
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
Tongue necrosis can be a manifestation of
giant cell arteritis
476
Stains for Rosai dorfman
S100 (+), CD1a (-), langerin (-)
477
Stains for LCH
(+) CD-1a or CD-207 (langerin) or S100
478
VEGF inhibitors
479
Dermatitis herpetiformis
Dermatitis herpetiformis (DH) is a chronic, intensely itchy, blistering skin manifestation of gluten-sensitive enteropathy, commonly known as celiac disease
480
Oral findings in tuberous sclerosis
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
VHL and endolymphatic sac tumor
Associated with Von hippel lindau Most arise within intraosseous portion of the endolymphatic duct / sac, with precursor lesions present in VHL patients
482
Endolymphatic sac tumor characteristics and location
Embryologically, the sac derives from the neuroectoderm and is located on the posteromedial surface of the temporal bone
483
Homer Wright rosettes but no olfactory neuroblastoma or retinoblastoma as options
medulloblastoma, supratentorial PNETs and pineoblastomas, Ewing
484
Mdm2 and cdk4 which tumors
Osteosarc Liposarc
485
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
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).