Tumors Flashcards by mirko niro

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

Leser-Trelat sign
Gastrointestinal adenocarcinoma

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Syndromes associated with lentigenes

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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Epidermoid cyst:

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

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

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Pilar cyst is derived from?

Isthmus of the hair follicle (outer root sheath)

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Steatocystoma:
a. derived from?
b. associated diseases

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

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Clear cell acanthoma:

a. deficient enzyme?
b. accumulation of?

a. Phosphorylase
b. Glycogen

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Basal cell carcinoma:

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

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

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Paget’s disease:

a. stain
b. associated malignancy

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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Transformation rate of actinic keratosis to SCC?

1-20%

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Bowen’s disease:

a. stain
b. transformation rate to SCC?

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

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Squamous cell carcinoma:

a. grading?
b. stain

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

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Associated tumors at the base of a cutaneous horn

VKATSS

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

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Keratoacanthoma:

a. associated syndrome
b. first line of treatment

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

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Pilomatricoma:

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

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

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Trichoblastoma is derived from?

Follicular germ of the hair follicle

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Trichoepithelioma:

a. derived from?
b. associated syndromes

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

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Associated syndrome in Fibrofolliculoma?

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

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Trichoadenoma is derived from?

Infundibulum

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Trichilemmoma:

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

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

2 most common tumors associated with Nevus sebaceous

Trichoblastoma
Syringocystadenoma papilliferum (SCAP)

Most common associated neoplasm in sebaceous adenoma

Muir-Torre syndrome

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

Cylindroma:

a. derived from?
b. associated syndrome

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

Spiradenoma:

a. derived from?
b. associated syndrome

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

Syringoma:

a. derived from?
b. associated diseases/syndromes

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

Hidrocystoma is derived from?

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

Poroma is derived from?

Acrosyringium

Benign melanocytic nevus: a. types b. mutation c. stain d. melanoma risk

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

Congenital melanocytic Nevus: a. mutation b. stain c. size

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

Spitz nevus: a. mutation b. stain c. treatment

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

Blue nevus: a. mutation b. associated syndrome c. stain

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

Dysplastic nevus: a. melanoma risk b. stain

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

Melanoma: a. mutation b. stain

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

Stain for dermatofibroma

(+) Factor XIIIa, (-) CD34

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

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

Angiofibroma/Fibrous papule: a. associated syndromes b. stain c. variants

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

Lipoma: a. stain b. associated syndromes

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

Associated syndrome in Nevus lipomatosis superficialis

Michelin tire baby syndrome

Associated syndromes in acrochordon

Birt-Hogg-Dube syndrome (Folliculin), Gorlin syndrome (PTCH) *Birt-Hogg-Dube FAT (Fibrofolliculoma, Acrochordon, Trichodiscoma)

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

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)

Neurofibroma: a. associated syndrome b. stain

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

Associated syndrome and mutation in schwannoma

Neurofibtomatosis type 2 (Merlin gene)

Variants of angiokeratoma

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

Etiology of pyogenic granuloma

Trauma, pregnancy, OCP, retinoids, Indinavir

Infantile vs Congenital Hemangioma

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

Stain for angiosarcoma

CD34 (not specific) CD31 (very specific)

Stain for Kaposi’s sarcoma

HHV8