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Neoplasms Flashcards

1
Q

Seborrheic Keratosis- Mutation

A

FGFR3, PIK3CA

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2
Q
A

Parakeratosis Ptychotropica

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3
Q

Epidermal Nevi- Mutation

Bilateral-Name

Syndrome

Other Syndromes with Epidermal Nevi

A

FGFR, PIK3CA, HRAS>NRAS>KRAS

Ichythosis Hysterix

Epidermal Nevus Syndrome (Neuro and MSK abnormal)

CLOVES, Proteus, PTEN hamartoma syndrome

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4
Q

Name that Path Finding? Syndrome? Mutation?

A

Path Finding: epidermal hyperplasia with prominent compact hyperkeratosis associated with granular and vacuolar degeneration of keratinocytes in the spinous and granular layers (epidermolytic hyperkeratosis).

Diagnosis: Epidermolytic Hyperkeratosis within Epidermal Nevi

Mutation Keratin 1 and 10 (mosaic)

When germline mutation: epidermolytic ichtyosis

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5
Q

Diagnosis?

A

Flegel disease (hyperkeratosis lenticularis perstans)

AD

Characteristic absent lamellar granules

Has been associated with endocrine disorders

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6
Q
A

Warty Dyskeratoma

M>F

Usually on the head and neck!!

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7
Q

AK pathogenesis

A

UVB

thymidine dimers (C-> T, CC-> TT)

p53 mutations

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8
Q
A

Erythroplasia of Querat

SCCis

High Risk HPV associated

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9
Q

SCC Met Risk Factors

A

> 2 cm

Breslow > 2mm

Poorly differentiated

Lip, Ear

Immunosuppresion (CLL), Marjolins

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10
Q

Medications associated with SCC

A

vemuRAFenib (BRAF)

voriconazole

MTX, etanercept

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11
Q

KA syndromes?

A

Ferguson–Smith syndrome is an autosomal dominant condition (25-30 yo at onset) due to mutations in TGFBR1 which encodes TGF-β receptor type 1.

Recurrent crops with spontaneous resolution

Grzybowski type (older 40-60) present as thousands of papules resembling milia or early eruptive xanthomas.

develop rapidly and may slowly resolve

ectropion, prominent oral involvement, mask-like facies.

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12
Q

Syndromes with BCCs

A

Gorlin

Bezex-Dupre-Christol

Rombo

Brooke-Spiegler

XP

Schoph-Schultz-Passarge

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13
Q

Multiple Epidermoid Cyst association

A

Gardner Syndrome (Familial Adenomatous Polyposis)- can have pilomatrical differentiation

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14
Q
A

Dermoid Cyst

Also has shark tooth lining (, but +granular layer)

Normal Adnexal Structures in cyst lining

Infants; Lateral Eyebrow

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15
Q

Blue Nevus Mutations

A

GNAQ, GNA11

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16
Q

Epithelioid Blue Nevus

A

Carney Complex

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17
Q
A

Baloon Nevus

Hint: Look for the conventional nevus cells within the lesion

Caused by melanosome degeneration

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18
Q

Spitz Mutation

Stains

A

HRAS

S100A6, S100, Melan-A, p16+ (vs atypical spitz where lost)

FISH with loss of 9p21 (loci for p16, CDKN2a)

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19
Q

Atypical Spitzoid Nevus Mutation

A

BAP-1 (BAPoma)

Also have BRAF mutations (in contrast to spitz nevi)

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20
Q

Deep Penetrating Nevus vs Cellular Blue

A

DPN

More likely to have junctional component

superficial ordinary nevus nests

pigmented within the melanocytes

nuclei: small smudged hyperchromatic, no nucleoli

(No GNAQ/GNA11 mutation)

Cellular Blue Nevus

epithelioid

pigment prominent in melanophages

vesicular nucleoli with prominent nucleolis

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21
Q

Congential Nevus

Mutation

Size Cut Offs

Risk of MM with Large

A

NRAS

< 1.5 cm, 1.5-19.9, >20

2-3%

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22
Q

Common Nevus: Mutation

A

BRAF

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23
Q

Desmoplastic melanoma stains

A

Negative Melan-A, HMB-45

Positive S-100, SOX-10

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24
Q

Diagnosis?

Stains?

A

Poroma

CEA, EMA, and PAS

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25
Diagnosis? Charateristic Features
Nodular Hidradenoma Cell Types: Poroid Cells, Squamoid Cells, Clear Cells Large Areas of Cystic Degeneration Dermal Sclerosis and Keloidal Collagen
26
Eruptive Syringomas
Down Syndrome
27
Clear Cell Syringoma
DM
28
Pleomorphic Adenoma
Oral variant of mixed tumor
29
chondroid syringoma
mixed tumor
30
Spiradenoma Syndromes
Brooke-Spiegler (CYLD) Spiradenocarcinoma more common (arising withing spiradenoma)
31
Diagnosis? Common Location?
Adenoid Cystic Carcinoma Primary Cutaneous ACC: Scalp Or Met OR primar salivary gland ACC (bad!)
32
Calcifying Epithelioma of Malherbe
Pilomatricoma
33
Pilomatricoma, Mutation
CTNNB1 (encodes B-catenin)
34
Conditions with Multiple Pilomatricomas
Myotonic Dystrophy Turner Syndrome Garder Syndrome (Familial Adenomatous Polyposis) Rubinstein-Taybi
35
Stains for Trichilemmoma
CD34 (Outer Root Sheath Differentiation) Pan-keratin
36
Trichilemmal Cyst
Pilar Cyst Outer Root Sheath Differentiation Isthmic Differentiation No granular layer
37
Schwannoma Stains
S-100, EMA+ Negative for Neurofilaments
38
Neurofibroma Stains
S-100+ Neurofilaments+
39
Bilateral Acoustic Neuromas
NF-2
40
Neurothekeoma (cellular)
S100- S-100A6+, NKI/C3+, PGP9.5+
41
Pustulo-ovoid bodies on milan
aggregated lysosomes granular cell tumor
42
Neuroblastoma
cutaneous mets are presenting sign in 30% with metastatic NSE+ and neurofilaments+
43
Reed Syndrome
Pilar Leiomyomas AD Fumarate Hydratase Uterin leiomyomas and RCC
44
Large Cell Transformation in MF
\>25% large cells (\> 4 times the normal size)
45
Hypopigmented MF
CD4-, DC8 +
46
Woringer-Kolopp
Pagetoid Reticulosis
47
Definition of Sezary Syndrome
CD4+ cells \> 1000 cells/uL
48
Granulomatous Slack Skin Association
30% develop Hodgkin's lymphoma
49
Another name for CD30
Ki 1
50
Foliculotropic MF (staining)
CD3+CD4+ CD8-
51
Adult t-cell leukemia/lymphoma staining
CD3+CD4+ CD8- CD25+
52
Types of LyP
A (75%) classic with Reed-Sternberg-like cells (CD-30+), eosinophils, neutrophils B (10-15%) Resembles patch/plaque MF C (10%) Pan-dermal infiltrate CD30+ large cells; same as ALCL and transformed MF histologically D (\<50%) Epidermotropic CD8+/CD30+ E
53
Primary Cutaneous Anaplastic Large Cell Lymphoma
Sheets of CD30+ cells (\>75% of cells) CD4+ (Negative EMA and negative ALK translocations (positive in systemic ALCL)
54
Extranodal NK/T-cell lymphoma, nasal type Association Stains
EBV+ CD2+/CD56+, CD3+
55
Subcutaneous panniculitis-like T-cell lymphoma
CD4-, CD56- CD8+ TIA1+ Granzyme B+ a/B phenotype Good prognosis
56
Primary Cutaneous y/d T-cell lymphoma
CD4-, CD8- CD56+, TIA-1+ B-F1 -
57
Agressive epidermotropic cytotoxic (CD8+) T-cell lymphoma
Eruptive Ulcerative Tumors Epidermotropic and angiodestructive Histological DDX for CD8+ MF, pagetoid reticulosis, type D Lyp
58
CD117
c-kit
59
Primary Cutaneous CD4+ small/medium pleomorphic T-cell lymphoma
solitary plaque or nodule CD4+, CD8-, CD30- Histologically same as MF
60
Primary Cutaneous Follicle Center Lymphoma
CD20+, CD79a+, bcl-6+, bcl-2-
61
Primary Cutaneous Marginal Zone Lymphoma
CD20+ bcl-2+, bcl-6 -
62
Primary Cutaneous diffuse large B-cell lymphoma
MUM-1+, bcl-2+, bcl-6+ 5 year survival 50%
63
Multiple Facial Angiofibromas
Tuberous Sclerosis MEN1 Birt-Hogg-Dube
64
Diagnosis? Association?
Sclerotic Fibroma Cowden's
65
Pleomorphic Fibroma-Stain
CD-34+
66
Path with giant cells and multiple dilated vessels in the dermis Diagnosis?
Multinucleate Cell Angiohistiocytoma
67
Dermatomyofibroma Staining?
CD-34 negative
68
Inclusion body fibromatosis Inclusions made of ???
Actine filaments (SMA+, red w/ trichrome)
69
Myofibroma Stain
SMA+
70
Syndromes associated with connective tissue nevi
tuberous sclerosis (shagreen patch) Buske-Olendorf (Elastomas and Collagenomas) +Osteopoikilosis Proteus (Collagenomas) MEN-1: collagenomas
71
Port Wine Stain Associations
Mafucci sydrome K-T Sturge- Webber Blue Rubber Bleb Proteus
72
Increased risk of infantile hemangiomas
premature females placental abnormalities
73
Epithelioid hemangioma
Same as ALHE
74
Tufted Hemangioma
Kasabach-Merrit Phenomenon
75
Glomeruloid Hemangioma
Polyneuropathy Organomegaly Endocrinopathy Monoclonalgammopathy Skin syndrome
76
Kaposiform Hemangioendothelioma Staining
CD-34 + (restricted to luminal vs tufted where they are diffuse) CD-31 +
77
Kaposi's Sarcoma Types
Classic: elderly mediterranean African Endemic: young males; lymph nodes involved and rapidly progressive Iatrogenic AIDS-associated
78
Kaposi's Sarcoma Stains
LANA-1 (latency associated antigen 1)- nuclear positivity
79
Angiosarcoma Stains
CD31+ CD34+ ERG+ FLI-1+ Ulex europeus lectin c-MYC in post-radiation (negative in atypical vascular lesions)
80
Conditions with Multiple Lipomas
Familial Multiple Lipomatosis Madelung's disease Gardner Bannayan-Riley-Ruvalcaba Proteus CLOVES PTEN hamartoma
81
Spindle Cell Lipoma Staining
CD-34+
82
Angiomyolipoma
No assication with tuberous sclerosis Considered Angioleiomyoma with mature fat
83
Liposarcoma marker
MDM2 (\>99%)
84
Pustulo-ovoid bodies of Milan
granules in granular cell tumor (lysosome and golgi material)
85
CD34 Tumors
DFSP Kaposi's sarcoma (endothelial cells) NF (Cellular NF is a DFSP mimick) Spindle cell lipoma fibrofolliculoma trichilemmoma sclerotic fibroma pleomorphic fibroma superficial angiomyxoma superficial acral fibromyxoma
86
Endothelial stains
ERG FLI-a Ulex europaeus agglutinin 1 vimentin
87
Histiocyte Stains
CD 68, CD163, lysozyme
88
Myofibroblast Stains
SMA (do not stain with desmin)
89
Natural Killer Cells
CD 56
90
Plasma Cells
CD138
91
Sebaceous Gland Stains
EMA adipophilin androgen receptor
92
CAM 5.2
Pagets and extramammary pagets
93
Stromelysin 3+
positive in DF, negative in DFSP
94
Modifier 25
Significant separate E/M on same day as procedure
95
Modifier 50
bilateral procedure
96
Modifier 58
Staged procedure within the postoperative global period
97
Modifier 59
distinct service, ie two separate procedures
98
Modifier 79
New procedure in the global period of a prior procedure
99
Destruction, excision, and linear repair global period
10 days
100
Flap, graft Global Period
90 days

Dermatology Boards (7 decks)

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