Tumors Flashcards

1
Q

Eruptive seborrheic keratosis - what do you call this sign and what is the associated malignancy?

A

Leser-Trelat sign
Gastrointestinal adenocarcinoma

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2
Q

Syndromes associated with lentigenes

A

LEOPARD (Lentigenes, ECG abnormalities, Ocular hypertelorism, Pulmonary stenosis, Abnormalities of genitalia, Retardation of growth, Deafness)

LAMB/Carney Complex (Lentigines, Atrial, Myxoma, Blue nevu)

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3
Q

Epidermoid cyst:

a. derived from?
b. associated syndrome and gene mutation

A

a. Infundibulum of the hair follicle
b. Gardner’s syndrome (APC gene)

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4
Q

Pilar cyst is derived from?

A

Isthmus of the hair follicle (outer root sheath)

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5
Q

Steatocystoma:
a. derived from?
b. associated diseases

A

a. Sebaceous duct
b. Steatocystoma multiplex (AD, Keratin 17 mutation), Pachyonychia congenita

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6
Q

Clear cell acanthoma:

a. deficient enzyme?
b. accumulation of?

A

a. Phosphorylase
b. Glycogen

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7
Q

Basal cell carcinoma:

a. derived from?
b. stain
c. associated syndromes
d. metastatic rate

A

a. follicular germ
b. BerEP4 (+), EMA (-)
c. Gorlin’s, Rombo, Bazex
d. 0.0028 to 0.55%

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8
Q

Paget’s disease:

a. stain
b. associated malignancy

A

a. (+) CK7, (+) CAM 5.2, (+) CEA, (+) EMA, (+) GCDFP15 in primary, (+) CK20 in secondary

(+) PAS
Diastase resistant (sialomucin)
(+) Alcian blue (high pH)
(+) Mucicarmine

b. Intraductal breast adenocarcinoma (MPD), GIT/GUT CA (EMPD)

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9
Q

Transformation rate of actinic keratosis to SCC?

A

1-20%

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10
Q

Bowen’s disease:

a. stain
b. transformation rate to SCC?

A

a. (+) PAS, Diastase sensitive (Glycogen)
b. 3-5%

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11
Q

Squamous cell carcinoma:

a. grading?
b. stain

A

a. Broder’s grading: well differentiated (1) to poorly differentiated (4)
b. AE1/AE3 (+), EMA (+), BerEP4 (-)

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12
Q

Associated tumors at the base of a cutaneous horn

A

VKATSS

Verruca vulgaris
Keratoacanthoma
Actinic keratosis (most common, 40%)
Trichilemmoma
Seborrheic keratosis
SCC

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13
Q

Keratoacanthoma:

a. associated syndrome
b. first line of treatment

A

a. Muir-Torre syndrome (MSH2 > MLH1 mutation): Keratoacanthoma + Sebaceous adenoma + Colorectal cancer
b. Excision

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14
Q

Pilomatricoma:

a. derived from?
b. associated with what electrolyte imbalance?
c. associated syndromes
d. mutation?

A

a. Follicular matrix
b. Hypercalcemia
c. Gardner, Turner, Rubenstein, Taybi, Myotonic dystrophy, Churg-Strauss
d. Beta catenin mutation

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15
Q

Trichoblastoma is derived from?

A

Follicular germ of the hair follicle

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16
Q

Trichoepithelioma:

a. derived from?
b. associated syndromes

A

a. Follicular germ of the hair follicle
b. Brooke-Spiegler (CYLD): AD; Cylindroma + Spiradenoma + Trichoepithelioma + Milia

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17
Q

Associated syndrome in Fibrofolliculoma?

A

Birt-Hogg-Dube (Fibrofolliculoma, Trichodiscoma, Acrochordon)

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18
Q

Trichoadenoma is derived from?

A

Infundibulum

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19
Q

Trichilemmoma:

a. derived from?
b. stain
c. associated syndrome and gene mutation

A

a. (+) PAS (glycogen), (+) CD34 - ORS marker
b. COWDEN syndrome (PTEN mutation)

20
Q

2 most common tumors associated with Nevus sebaceous

A

Trichoblastoma
Syringocystadenoma papilliferum (SCAP)

21
Q

Most common associated neoplasm in sebaceous adenoma

A

Muir-Torre syndrome

-Keratoacanthoma + Sebaceous adenoma + Colorectal cancer
-MSH2 > MLH1 mutation)
-Autosomal dominant

22
Q

Cylindroma:

a. derived from?
b. associated syndrome

A

a. Apocrine secretory duct
b. Brooke-Spiegler (CYLD): AD; Cylindroma + Spiradenoma + Trichoepithelioma + Milia

23
Q

Spiradenoma:

a. derived from?
b. associated syndrome

A

a. Eccrine secretory duct
b. Brooke-Spiegler (CYLD): AD; Cylindroma + Spiradenoma + Trichoepithelioma + Milia

24
Q

Syringoma:

a. derived from?
b. associated diseases/syndromes

A

a. Eccrine straight duct
b. Diabetes mellitus, Down syndrome, Marfan, Ehlers-Danlos

25
Q

Hidrocystoma is derived from?

A

Apocrine (with decapitation secretions) and Eccrine (no decapitation secretions)

26
Q

Poroma is derived from?

A

Acrosyringium

27
Q

Benign melanocytic nevus:

a. types
b. mutation
c. stain
d. melanoma risk

A

a. Junctional, Compound, Intradermal
b. BRAF mutation (acquired)
c. (+) S100, (+) HMB45, (+) Melan A/Mart1
d. 100 typical nevi 3.4 fold

28
Q

Congenital melanocytic Nevus:

a. mutation
b. stain
c. size

A

a. NRAF mutation
b. (+) S100, (+) HMB45, (+) Melan A/Mart1
c. Small (< 1.5cm), Large (20cm and above)

29
Q

Spitz nevus:

a. mutation
b. stain
c. treatment

A

a. HRAS mutation
b. (+) S100, (+) HMB45, (+) Melan A/Mart 1, Trichome (stain for Kamino bodies)
c. Excision with 3-5mm margin

30
Q

Blue nevus:

a. mutation
b. associated syndrome
c. stain

A

a. GNAQ mutation
b. LAMB/Carney Complex
c. (+) S100, (+) HMB45, (+) Melan A/Mart 1

31
Q

Dysplastic nevus:

a. melanoma risk
b. stain

A

a. >10 dysplastic: 12-fold increased risk for melanoma
b. (+) S100, (+) HMB45, (+) Melan A/Mart 1

32
Q

Melanoma:

a. mutation
b. stain

A

a. cKit (ALM, majority [70%] arise without a precursor nevus)
b. (+) S100, (+) HMB45, (+) Melan A/Mart 1, (+) Ki-67

33
Q

Stain for dermatofibroma

A

(+) Factor XIIIa, (-) CD34

34
Q

Dermatofibrosarcoma protuberans:

a. stain
b. mutation
c. recurrence rate
d. most common site of metastasis

A

a. (-) Factor XIIIa, (+) CD34
b. t(17;22) - chromosomal translocation
c. 30-50%
d. Lungs

35
Q

Angiofibroma/Fibrous papule:

a. associated syndromes
b. stain
c. variants

A

a. Tuberous sclerosis, Multiple Endocrine Neoplasia (MEN) 1
b. (+) Factor XIIIa
c. Pearly penile papule, Adenoma sebaceum, Periungal fibroma, Fibrous papule of the face

36
Q

Lipoma:

a. stain
b. associated syndromes

A

a. Oil-Red O, Sudan black (fresh-frozen)
b. Bannayan-Riley-Ruvalcaba (PTEN), Cowden (PTEN), Gardner (APC), Madelung’s disease (benign symmetric familial lipomatosis)

37
Q

Associated syndrome in Nevus lipomatosis superficialis

A

Michelin tire baby syndrome

38
Q

Associated syndromes in acrochordon

A

Birt-Hogg-Dube syndrome (Folliculin), Gorlin syndrome (PTCH)

*Birt-Hogg-Dube FAT (Fibrofolliculoma, Acrochordon, Trichodiscoma)

39
Q

Leiomyoma:

a. associated syndrome
b. mutation
c. origin
d. stain

A

a. Reed’s syndrome: multiple lesions + renal cell carcinoma + uterine leiomyomas
b. Fumarate hydratase gene
c. arrector pili muscle
d. Masson’s trichrome (red), Desmin, Smooth Muscle Actin (SMA)

40
Q

Neurofibroma:

a. associated syndrome
b. stain

A

a. Neurofibromatosis type 1 - AD, Neurofibromin gene, Buttonholing sign
b. S100

41
Q

Associated syndrome and mutation in schwannoma

A

Neurofibtomatosis type 2 (Merlin gene)

42
Q

Variants of angiokeratoma

A

AK of Mibelli - fingers and toes
AK of Fordyce - scrotum and vulva
AK corporis diffusum - Fabry Disease

43
Q

Etiology of pyogenic granuloma

A

Trauma, pregnancy, OCP, retinoids, Indinavir

44
Q

Infantile vs Congenital Hemangioma

A

Infantile Hemangioma
-GLUT1 (+)
-more common
-develops 2-8 weeks of age
-F>M (5:1)
-grows rapidly for 6-12 months
-slow involution (5-9 years)

Congenital Hemangioma
-GLUT1 (-)
-less common
-present at birth
-F=M
-growth is complete at birth or grows proportionately with child’s growth
-rapid involution (within 12-18 months) or no involution

45
Q

Stain for angiosarcoma

A

CD34 (not specific)
CD31 (very specific)

46
Q

Stain for Kaposi’s sarcoma

A

HHV8