Boards Facts Flashcards
1
Q
Genetic syndromes with increased risk of rhabdomyosarcoma
A
li-fraumeni syn
costello syn
NF1
beckwith-wiedemann syn
2
Q
Two most useful stains for rhabdomyosarcoma
A
desmin +
myogenin +
3
Q
positive stains in leiomyosarcoma
A
desmin, h-caldesmon, SMA, MSA
need positivity of at least 2
4
Q
angiosarcoma risk factors
A
radiation, vinyl chloride, coal dust
5
Q
pathognomonic feature histologically for angiosarcoma
A
intracytoplasmic lumina
6
Q
What % of malignant peripheral nerve sheath tumors occur in NF1 patients
A
25-30%
7
Q
Most common nerves affected by MPNST
A
vagal and vestibular cranial nerves
8
Q
malignant triton tumor components
A
MPNST and rhabdomyosarcoma
9
Q
recurrent PAX3-MAML3 gene fusion is seen in
A
Biphenotypic sinonasal sarcoma
t(2;4)
10
Q
PAX3-FOX01 and PAX3-NCOA1 can be found in what two neoplasms
A
alveolar rhabdomyosarcoma
biphenotypic sinonasal sarcoma
11
Q
most common non-rhabdomyosarcoma soft tissue sarcoma in children, adolescents and young adults is?
A
synovial sarcoma
12
Q
greatest risk factor for synovial sarcoma
A
prior radiotherapy
13
Q
What mutation is associated with sporadic cases of desmoid-type fibromatosis
A
CTNNB1 (85%) of sporadic cases
14
Q
severe oncogenic osteomalacia has been reported in association with what lesion?
A
sinonasal glomangiopericytoma
15
Q
What region of the beta-catenin gene is specifically implicated in glomangiopericytoma
A
GSK3beta region
16
Q
3 risk factors (or associated factors) for development of hemangiomas
A
injury
hormonal factors
drug (vemurafenib)
17
Q
Hemangiomas have a greater association with what genetic syndromes
A
sturge-weber syndrome
von hippel lindau disease
18
Q
T/F angiosarcomas may arise within hemangiomas
A
false- angiosarcomas arise de novo
19
Q
What is Horner syndrome and what lesion may cause this presentation
A
oculosympathetic palsy- decreased pupil size, a drooping eyelid and decreased sweating on the affected side of your face
“miosis, ptosis, anhidrosis”
-Caused by many things, one being tumors in the area and particularly a sinonasal schwannoma
20
Q
What tumor of the sinonasal tract has a left sided predominance
A
meningioma
21
Q
chondromesenchymal hamartoma may be the presenting sign for what genetic syndrome
A
pleuropulmonary blastoma-associated DICER1 familial tumor susceptibility syndrome
22
Q
third most common sinonasal malignancy
A
sinonasal lymphoma
1. SCC 2. adneocarcinoma
23
Q
What populations are more greatly affected by sinonasal extra nodal NK/Tcell lymphoma
A
east asian
indigenous populations of mexico, central and south america
24
Q
two most common hematolymphoid tumors of the sinonasal region
A
extranodal NK/T cell lymphoma
DLBCL
25
what gene and polymorphism indicates increased susceptibility to NK-T Cell lymphoma
lymphotoxin alpha (LTA) +252 (AG) polymorphism
26
CIC-DUX4 fusion has been identified in what tumor?
ewing
**this may end up being a distinct tumor entity in the future as these tumors are also not diffusely positive with CD99 (they show membranous CD99 positivity)
27
what heritable tumor may predispose patients to ewing sarcoma
heritable retinoblastoma
28
What staging system is used for ewing sarcoma?
intergroup rhabdomyosarcoma study group staging system (IRSG)
29
What structure is nearly always involved in olfactory neuroblastoma
cribriform plate
30
Classic radiographic features of olfactory neuroblastoma
- dumbbell shaped mass extending across the cribriform plate
- peripheral tumor cysts
- speckled calcifications
31
What staging system is most appropriate for olfactory neuroblastoma
OLD: Kadish system and/or with Morita modification
CURRENT: Hyams et al (grade 1- most differentiated to grade 4= least differentiated)
32
What mutation is most common in mucosal type melanomas
KIT mut/amp
33
Populations at highest risk for nasopharyngeal carcinoma
chinese from southeast asia
inuit
northern africans
34
risk factors for non-keratinizing nasopharyngeal carcinoma
-high consumption of salted and fermented foods with high nitosamine content (non-keratinzing, endemic subtype)
"salted fish"
-EBV
35
Risk factors for keratinizing subtype of nasopharyngeal carcinoma
similar to other areas of keratinizing SCC- tobacco and alcohol
36
most common site of origin for nasopharyngeal carcinoma
pharyngeal recess (fossa of rosenmuller)
37
most common palpable node at presentation with nasopharyngeal carcinoma
jugulodigastric
*generally involves the posterior chain more freq than other head and neck carcinomas
38
What HLA types are associated with high risk of NPC
In chinese populations: HLA-A*02 and HLA-B*46
| HLA-A*0207
39
Diagnostic serology tests used to aid in the diagnosis of nasopharyngeal carcinoma
IgA to EBV viral capsid
IgG/IgA to early EBV antigens
-Can use circulating plasma levels of EBV to monitor disease control
40
most common salivary gland malignancy affecting the nasopharynx
adenoid cystic carcinoma
| -comprise 1/4 of all adenocarcinomas at this site
41
Population affected by salivary gland anlage tumor
INFANTS
| male predilection
42
What is the presenting sign for SGAT
respiratory distress due to nasal airway obstruction (in an infant)
43
You suspect a pituitary adenoma, what stains would be positive
synapto, some combo of hormones (GH, LH, prolactin, ACTH, TSH, FSH)
44
anterior pituitary tumors/pituitary adenomas are a component of what diseases/syndromes
- gigantism (producing GH)
- acromegaly (producing GH)
- can be a cause of hyperthyroidism if producing TSH
- cushings (producing ACTH)
- MEN1
45
Sheehan syndrome
postpartum necrosis of the anterior pituitary
| -during pregnancy ant pituitary enlarges for prolactin production
46
craniopharyngioma originates from what structure
remnants of rathke cleft
47
What nasopharyngeal tumor contains brown "machine-oil" fluid
craniopharyngioma
48
Paragangliomas should be distinguished from what malignant tumors and by what stains
DDX depends on the location of the tumor: middle ear, carotid body(neck), laryngeal, metastatic (usually bone, liver, lung)
Generally nothing else stains positive for neuroendocrine markers with the specific pattern of S100+ sustentacular cells in the typical zellballen morphology
Can additionally R/o
- metastatic renal cell carcinoma: PAX8+
- medullary thyroid carcinoma: calcitonin +
- Alveolar soft part sarcoma: neuroendocrine marker negative, TFE3+
49
syndromes associated with paragangliomas
```
Hereditary paragangliomatosis
NF1
MEN2A
MEN2B
von hippel lindau
carney stratakis syndrome
```
50
What ancillary test (after imaging) could be helpful in the diagnosis of nasopharyngeal angiofibroma
angiography- considered diagnostic
| shows the feeding vessel: typically internal maxillary artery
51
The stromal cells of nasopharyngeal angiofibroma are positive for what two stains (typically)
AR
| beta-catenin
52
Few reports of nasopharyngeal angiofibroma arising in association with what syndrome
familial adenomatous polyposis
53
Stains and expected pattern of staining for nasopharyngeal angiofibroma
AR+, nuclear b-catenin + stromal cells
| CD31, CD34 + vessels
54
What 4 features are evaluated at the invasive front of a SCC as part of the total malignancy score
degree of keratinization
nuclear polymorphism
pattern of growth
inflammatory response
55
church spire keratosis has been used to describe the surface features of what neoplasm
verrucous carcinoma
56
What neoplasm does a chordoma histologically resemble? How can you differentiate these two entities
myoepithelioma
- brachyury + chordoma
- chordoma classic locations: clivus, sacrum
- chordoma foamy/bubbly cells: physaliferous cells
57
neoplasms of pericytes
myopericytoma
| glomus tumor
58
pleomorphism in general like in undifferentiated pleomorphic sarcoma are a sign of what genetic aberration
anueploidy
59
Most neuroendocrine carcinomas of the larynx are _____ differentiated and have a _____ predilection
```
moderately differentiated (second most common poorly-diff)
male
```
60
Other than medullary thyroid carcinoma, what malignancy may express calcitonin and thus create a diagnostic pitfall (especially in cases of lymph node involvement)
well-mod neuroendocrine carcinoma
61
greatest risk factor for neuroendocrine carcinoma of the larynx
smoking
62
DNA coating of vessel walls in a poorly differentiated neuroendocrine carcinoma (or other malignancy) is termed?
azzopardi phenomenon
63
Adenoid cystic carcinoma developing in the trachea may mimic the symptoms of?
asthma
64
most common salivary gland malignancy of the larynx, hypopharynx, trachea
adenoid cystic carcinoma
65
cell of origin for oncocytic papillary cystadenoma
minor salivary duct cell
66
soft tissue granular cell tumors show ______ cell differentiation
schwann
67
What population is most affected by tracheal granular cell tumors
black
female
* other sites do not show this predilection
68
What two markers are positive in >90% of well-diff(atypical lipomatous tumor) and dedifferentiated liposarcoma
MDM2
| CDK4
69
SMA+ spindle cell tumor with plump spindle cells with open chromatin, can have prominent nucleoli numerous lymphocytes, plasma cells, +/- eosinophils and neutrophils, is characteristic of what tumor?
-Helpful molecular testing?
inflammatory myofibroblastic tumor
ALK- IHC ctyoplasmic staining
70
What population and location is most common for IMT
children and young adults in the abdomen or pelvis
71
ALK positivity can be seen in what neoplasms
- IMT
- Lung adeno
- Anaplastic large cell lymphoma
- Epitheliod histocytoma
72
```
WEll circumscribed
lesion smaller than 3 cm
pockets of myxoid mucinous degeneration
areas of thick fibrous tissue
spindle cells in short fascicles
scattered extravasated RBCs
common mitoses
keloidal collagen
```
Nodular fascitis
*random bizarre appearing cells that are hyperchromatic are actually atrophic muscle cells with multiple nuclei
73
tram-track pattern of staining with SMA in a spindle cell neoplasm is classic of what entity?
nodular fasciitis
| **though immunohistochemistry typically not needed
74
most common site fir laryngeal chondromas
cricoid cartilage
75
What population is most affected by chondromas and chondrosarcoma
white>>black 7:1
76
Predisposing factors for chondrosarcoma
radiotherapy, polytetrafluoroethylene injection (teflon), repeated trauma
77
What is the typical size cut off for chondroma
typically <2cm
| chondrosarcomas tend to be larger (3.5-12cm)
78
What do chondroid cells stain positive for
D240, s100
79
Oral cancer rates are higher in males in every country except
India
| THailand
80
Which types of SCC have a worse prognosis when compared to conventional SCC
spindle cell, adenosquamous carcinoma
81
Syndromes that increase the risk of oral SCC
li fraumeni syndrome
fanconi anemia
dyskeratosis congenita
82
Chromosomal losses typical of OSCC
| Chromosomal gains typical of OSCC
losses: 3p, 9p, 8p, 17p
gains: 3q, 11q
83
Genes reported to have a role in OSCC
TP53, CDKN2A, PTEN, HRAS, PIK3CA
84
Prevalence of leukoplakia in western countries, prevalence globally?
1-4%
| 2-3%
85
Prevalence of oral erythroleukoplakia
0.02-.83%
86
Erythroplakia is most common at what sites
soft palate, FOM, buccal mucosa
87
Mean transformation rate for leukoplakia
1-2%
88
Mean transformation rate of cases with diagnosis epithelial dysplasia
12%
89
Prognostic markers for malignant transformation of oral potentially malignant disorders
Additional factors associated with increased risk for malignant transformation
○ Female gender
○ Older age
○ Nonsmoking status
○ Lesion persistence for several years
○ Extensive lesion size
○ Involvement of ventrolateral tongue or FOM16 - 39% rate of malignant transformation, 47% in females
○ Loss of heterozygosity (LOH) of chromosomes arms 3p and 9p
○ Additional LOH at 4q, 8p, 11q, 13q, and 17p
○
90
15 year transformation rates for mild, moderate, and severe dysplasia
mild: 6%
mod: 18%
severe: 39%
91
Homozygous inactivation of what gene is implicated in PVL
CDKN2A (aka P16INK4a, P14ARF)
| gene codes for tumor suppressor proteins p16 and p14ARF
92
Mortality rate of PVL
30-40%
93
most common hpv lesion of the skin
verruca vulgaris
| types 2,4,40,57
94
neumann tumor is another name for
congenital granular cell tumor
95
rod shaped cytoplasmic crystals also called jack straws are characteristic of what neoplasm
adult rhabdomyoma
96
Strap cells are seen in what neoplasm
adult rhabdomyoma
| -eosinophilic cytoplasm, with occasional striations
97
Aberrations in what gene have been reported in fetal rhabdomyoma
PTCH1
98
Most common site for lymphangiomas intraorally
dorsum of the tongue
99
Malformations of lymphatic vessels and vascular anomalies are common in what syndrome? What somatic activating mutation is responsible for this syndrome?
Proteus syndrome
| AKT1
100
Genetic disorders associated with lymphangiomas
Proteus syndrome
Turner syndrome (45,X syndrome)
Trisomy 21
101
Most common benign tumor of the oral cavity in peds population
hemangioma
102
Hemangiomas are frequently associated with what genetic abnormality
full or partial polysomy 13
103
"shredded carrots" appearance of the collagen bundles is characteristic of what lesion
Neurofibroma
104
What % of HIV infected individuals develop oral kaposi sarcoma
20%
105
Checkerboard-like infiltration of adjacent voluntary muscle by a cellular spindle cell population with atypical hyperchromatic nuclei, variable SMA+, desmin+, calponin+, CD34+ describes what neoplasm?
Myofibroblastic sarcoma
106
Most common head and neck site for involvement by myeloid sarcoma
oral cavity
107
Most immunodeficiency associated lymphomas are _____+
EBV+
108
What cell is characteristic of anaplastic large cell lymphoma
Cells with horseshoe / kidney / embryo shaped nuclei
109
What stains typically mark the T cells of CD30-positive t cell lymphoproliferative disorder
CD30+ diffuse
| -Cytotoxic t cell markers: TIA1, granzyme B, perforin
110
What may also be on your differential on a biopsy specimen of suspected CD30 positive T cell lymphoproliferative disorder
TUGSE (though there should not be the population of clear tumor cells)
- EBV+ mucocutaneous ulcer (EBER positive)
- Anaplastic large cell lymphoma (due to some of the cells appearing like hallmark cells) but this is almost always ALK positive
111
EBV infection is none to cause a surge in what type of cell population
plasmablasts
112
MYC aberrations occur in half of the cases of what type of lymphoma
plasmablastic lymphoma
| burkitt lymphoma
113
Genetics associated with extramedullary myeloid sarcoma
monosomy 7
trisomy 8
t(8;21) peds patients
114
Oropharyngeal SCC arises from what type of epithelium?
crypt
115
what somatic mutation is unique to OPSCC-HPV related
TRAF3
| PIK3CA is more common in OPSCC-HPV+ than OPSCC-HPV-
116
Most common extranodal head and neck site for lymphoma
waldeyers ring
117
Elevation of what product in the serum is associated with poorer prognosis in patients with lymphoma
lactate dehydrogenase
118
Stains for Hodgkin lymphoma
positive: PAX5, CD30, CD15
negative: CD20, CD79a
119
Stains for DLBCL
CD20, CD10, BCL6, MYC
120
Mantle cell lymphoma is characterized by what translocation? resulting in over expression of what?
CCND1 translocation
| cyclin D1 overexpression
121
Stains for mantle cell lymphoma
positive: CD20, CD5, SOX11, cyclin D1
negative: Cd10, CD23, BCL6
122
Most common anatomical site for follicular dendritic cell sarcoma
head and neck region, specifically cervical lymph nodes > waldeyers ring
123
Features of unicentric castleman disease
more common than multicentric, benign, limited to one lymph node
124
Features of Multicentric castleman disease
systemic process, often associated with infection (HIV, HHV-8), malignancy (lymphomas and Kaposi sarcoma) and plasma cell disorders (POEMS syndrome and amyloidosis)
125
15% of follicular dendritic cell sarcomas arise in the setting of what (typically benign) disorder
Castleman disease
126
Positive markers in follicular dendritic cells (such as in follicular dendritic cell sarcoma)
CD21, CD23, CD35, clusterin, CXCL13, podoplanin
127
Merkel cell carcinoma arises in association with what other malignancies in as many as 36% of cases
CLL/SLL
128
positive staining by CD30, CD15, and PAX5 is helpful in identifiying
hodgkin lymphoma cells
129
The international prognostic index for NHL consists of what criteria
```
clinical stage
serum lactate dehydrogenase
patient age
performance status
involvement of extranodal sites
```
130
chicken wire calcifications are a classic feature of
chondroblastoma
131
storiform pattern is typical of
fibrous histiocytoma
| DFSP
132
Hemangiopericytic vessels are common in
```
SFT
Monophasic synovial sarcoma
biphenotypic sinonasal sarcoma
mesenchymal chondrosarcoma
nasopharyngeal angiofibroma
DFSP
```
133
Invasive spindle cell neoplasm with entrapped epithelial proliferations. Arising in the nasal cavity. Stains positive: actin, S100. Diagnosis?
Biphenotypic sinonasal sarcoma
134
A reported case of ghost cell odontogenic carcinoma demonstrated a novel mutation in _____ gene, suggesting a possible link between this neoplasm and what syndrome
APC gene
| Gardner syndrome
135
Most common site affected by osteosarcoma
metaphysis of long bones of children and adolescents (femur, tibia, humerus)
136
What stains may help distinguish low grade osteosarc from fibro-osseous mimics
MDM2
CDK4
amplification
137
syndromes increasing the risk for osteosarcoma
li-fraumeni
werner syndrome (adult progeria)
retinoblastoma
rothmund-thompson(poikiloderma atrophicans with cataract)
138
Molecular findings in DFSP
COL1A1 PDGFB fusion
| t(17;22)
139
Classic location for chondroblastoma
epiphysis of long bones
140
multiple CGCGs should raise suspicion for
Noonan syndrome
LEOPARD syndrome
NF1
**they separate out cherubism from this condition in WHO
141
CGCGs arising in the setting of NF1, Noonan, or LEOPARD are more likely to demonstrate germline mutation in what pathway
RAS/MAPK
142
cupping or shallow indentation of the bone is a characteristic finding of what gingival lesion
PGCG
143
One third of simple bone cysts have been found in association with what condition
florid osseous dysplasia
144
Minimal bone marrow involvement in a case of solitary plasmacytoma of bone is defined as
10%
| > or = 10% clonal is BM qualifies as multiple myeloma
145
Predominant etiology of pinna associated carcinoma
frost bite
| actinic overexposure
146
Suggested etiologies for carcinomas of the external auditory canal and middle ear
radiation exposure and chronic inflammation
147
ceruminous glands are located?
external auditory canal
| wax producing glands of the ear
148
Which malignant salivary gland neoplasms have an analogous counterpart that develops in the ceruminous glands of the external auditory canal
adenoid cystic carcinoma
mucoepidermoid carcinoma
-if you see adenocarcinoma of the ear that does not fit either of these definitions it is diagnosed as just adenocarcinoma or ceruminous adenocarcinoma
149
Distinguishing feature between: Ceruminous adenocarcinoma vs ceruminous adenoma
ceroid pigment- present in adenoma absent in adenocarcinoma
150
predominant location and population for middle ear SCC
left ear of elderly men
151
Patients with sporadic endolymphatic sac tumor in the temporal bone should be evaluated for what genetic finding
VHL gene mutation
| von-hippel lindau
152
acquired cholesteatoma is thought to be caused by
recurrent infections, blast injury to tympanic membrane
153
vestibular schwannomas arise in what part of the ear
internal auditory canal or labyrinth
154
most common tumor of the temporal bone
vestibular schwannoma
155
meningiomas are well described in what genetic disorder
NF2
156
tumor of the middle ear with duct-like structures that stains positive for neuroendocrine markers including ISL1(aberrant neuroendocrine marker)
middle ear adenoma
157
what lesion has the highest degree of heritability among human neoplasms
paragangliomas
158
risk factors for carotid body paraganglioma
```
high altitude (chronic hypoxia)
female gender (8:1 at high altitudes)
```
159
two cell types of paragangliomas and the corresponding stains to highlight them
chief cells: chromo, synapto, CD56, somatostatin
| sustentactular cells: S100
160
3 genes associated with inheritance of paraganglioma (but only when inherited from the father)
SDHD, SDHAF2, MAX
161
SDHC mutation is associated with
single head and neck paragangliomas
162
SDHD mutation is associated with
multiple paragangliomas, 85% have carotid body paraganglioma
163
What is the 3 P association of SDH gene defects
paraganglioma, pituitary adenoma, pheochromocytoma
164
Carney-Stratakis syndrome
SDH(succinate dehydrogenase) mutations
| paraganglioma and gastrointestinal stromal tumor
165
Carney triad
paraganglioma
GIST
pulmonary chondroma
166
Syndromic associations to paragangliomas
MEN2
NF1
von hippel lindau
paraganglioma syndromes 1-5
167
What paraganglioma mutation yields highest rate of recurrence and highest mortality
SDHB
168
Accessory tragus is associated with what syndrome
Goldenhar
| oculoauriculovertebral dysplasia
169
second most common benign salivary gland tumor
warthin tumor
170
CYLD cutaneous syndrome is a new name for what? What are the characteristic features
brooke spiegler syndrome
| -trichoepitheliomas, dermal cylindromas, spiradenomas, AND membranous basal cell adenomas
171
Mitral valve prolapse is commonly seen in what conditions and syndromes
```
Down,
Marfan,
Ehler-‐Danlos
syndromes
and
Rheumatic
heart
disease
```
172
most common salivary gland tumor in the pediatric population
hemangioma
173
What autoantibody is implicated in hashimotos thyroiditis
Auto-‐antibody is anti-‐thyroidal anti-‐microsomal antibody
(ATAMAB)
174
Cholesteatomas are predominantly found in what part of the ear
middle ear
175
Mitral valve prolapse is commonly seen in what conditions and syndromes
```
Down,
Marfan,
Ehler-‐Danlos
syndromes
and
Rheumatic
heart
disease
```
176
what cells secrete calcitonin
parafollicular aka C cells of the thyroid
177
What autoantibody is implicated in hashimotos thyroiditis
Auto-‐antibody
is anti-‐thyroidal anti-‐microsomal antibody
(ATAMAB)
178
What serum tests would be helpful in monitoring treatment effects for medullary thyroid carcinoma
calcitonin
| CEA- carcinoembryonic antigen
179
Cause of graves disease
autoantibody against TSH receptor
180
What syndromes result in an increased risk of papillary thyroid carcinoma
Cowden
| Gardners
181
medullary thyroid carcinoma is a component of what syndromes
MEN2A, 2B, familial MTC syndrome
182
What serum tests would be helpful in monitoring treatment effects for medullary thyroid carcinoma
calcitonin
| CEA- carcinoembryonic antigen
183
Characteristic background element present in medullary thyroid carcinoma
amyloid- 80% of cases
184
staining panel for medullary thyroid carcinoma
Positive: TTF1, CK7, PAX8, Calcitonin, Chromo, synapto
Negative: thyroglobulin
185
most common tumor of the appendix
carcinoid- usually incidental
186
Volvulus definition
bowel obstruction with twisting of a loop of bowel
187
HLAs most strongly associated with celiac disease
HLA-DQ2 and DQ8.
188
Mallory-Denk body
Mallory-Denk body, and Mallory's hyaline, is an inclusion found in the cytoplasm of liver cells. Mallory bodies are damaged intermediate filaments within the liver cells.
Associated with multiple causes of chronic liver disease
189
most common benign tumor of the female breast
fibroadenoma
190
malakoplakia is a rare inflammatory condition thought to be caused by ______?
bactericidal defect in macrophages
191
What organ undergoes liquefactive necrosis rather than coagulative necrosis when faced with ischemia/infarct
brain
192
most important risk factor for renal cell carcinoma
smoking
193
case of a child with abdominal pain and a fever-- what is the most likely diagnosis they are going for
wilms tumor
194
malakoplakia is a rare inflammatory condition thought to be caused by ______?
bactericidal defect in macrophages
195
conditions with increased risk of leukemia
```
Down syndrome
bloom syndrome
NF1
Klinefelter
fanconi anemia
schwachman(diamond) syndrome
ataxia-telangiectasia
wiskott-aldrich syndrome
```
196
What is the rule of 10s pertaining to pheochromocytoma
10% bilateral
10% extra-adrenal
10% malignant (defined by metastasis)
10% not associated with HTN
197
MAGIC syndrome features
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome refers to a condition in which features of Behcet's disease (BD) and relapsing polychondritis (RP) occur in the same individual.
198
erythema nodosum causes
Lofgren syndrome
valley fever
behcets
199
port wine stain is a features of what syndromes
sturge weber
beckwith-wiedemann
hemihyperplasia
200
Causes of leonine facies
```
leprosy
pagets
amyloiodosis
mycosis fungoides
lipoid proteinosis
```
201
benign nodules of the skin that cause pain
```
angiolipoma
neuroma
glomus tumor
eccrine spiradenoma
leiomyoma
```
202
Pheochromocytomas are a feature of what syndromes
```
men 2a
men 2b
NF1
Sturge-weber
von hippel lindau
```
203
port wine stain is a features of what syndromes
sturge weber
beckwith-wiedemann
hemihyperplasia
204
syndromes with seizures
sturge weber
tuberous sclerosis
incontinetia pigmenti
mucopolysaccharidosis
205
what cell surface molecule on epithelial cells can bind to EBV
CD21
206
HPV strains in butcher warts
7, 32
207
Most common bone affected by adamantinoma
tibia
- presents as a painful mass
- low grade malignancy
- highlight epithelial type cells in the bony stromal background with CKs (fibrous dysplasia or osseous dysplasia should not have CK positive background cells)
* osteofibrous dysplasia-like stromal component with small, often inconspicuous epithelial nests.
208
Supernumerary teeth are a feature of what syndromes
```
cleidocranial dysplasia
gardners
sturge weber
apert
crouzan
```
209
sunburst radiographic change can be seen in:
osteosarc
chondrosarc
hemangioma
MNTI
210
clear cell neoplasm of the jaw DDX
clear cell odontogenic carcinoma
ameloblastoma (must find some peripheral palisading)
mucoep (mucicarmine stain)
CEOT- amyloid stain
met- renal (PAX8), breast(GATA3), melanoma(HMB45)
211
Most common bone affected by adamantinoma
tibia
- presents as a painful mass
- low grade malignancy
212
most common salivay gland tumor in the pediatric population
PA (benign)
Mucoep (malignant)
213
Syndromes associated with pituitary adenoma include:
multiple endocrine neoplasia (MEN) syndromes MEN1 and MEN4, Carney complex(rare genetic disorder characterized by multiple benign tumors (multiple neoplasia) most often affecting the heart, skin and endocrine system), McCune-Albright syndrome, X linked acrogigantism (XLAG), hereditary pheochromocytoma and paraganglioma syndrome related to succinate dehydrogenase (SDH) genes
