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AliKhan Derm > Neoplastic Dermatology chapter 6 > Flashcards

Neoplastic Dermatology chapter 6 Flashcards

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Dermatology Board Prep flashcards
1
Q

SK’s are associated with what mutations?

A

FGFR3 and PIK3CA activating mutations

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2
Q

Acanthosis, papillomatosis, hyperkeratosis with pseudohorn cysts, flat base (“string sign”), bland keratinocytes without atypia seen with?

A

Seborrheic keratosis

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3
Q

Seborrheic Keratosis Histologic Variants?

A

Acanthotic (most common), Hyperkeratotic (“church spire”), Reticulated, Irritated, Clonal, Melanoacanthoma

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4
Q

Porokeratosis onset in infancy or childhood, extremities; large (often >3 cm) circinate plaque with keratotic border?

A

Porokeratosis of Mibelli

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5
Q

Porokeratosis in middle age, F > M with numerous brownish-red macules w/ keratotic borders in sun-exposed areas; most common on legs?

A

Disseminated superficial actinic porokeratosis DSAP - most common

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6
Q

Porokeratosis onset in newborns with linear lesions on extremities, follows lines of Blaschko is?

A

Linear porokeratosis

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7
Q

Porokeratosis with onset in adolescence, 1- to 2-mm “seed-like” papules on palms/soles?

A

Punctate porokeratosis

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8
Q

Porokeratosis onset in childhood occurs on palms and soles initially is?

A

Porokeratosis palmaris, plantaris, et disseminata

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9
Q

Porokeratosis that clinically resembles a nevus comedonicus of palm or sole, but histology shows abundant cornoid lamellae arising from acrosyringium is?

A

Porokeratotic eccrine ostial and dermal duct nevus

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10
Q

Highest risk of SCC in porokeratosis in what subtype?

A

linear porokeratosis

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11
Q

White scaly variant of SK is called?

A

Stucco keratosis associated with HPV-23b

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12
Q

Endophytic variant of irritated SK is called?

A

Inverted follicular keratosis, appears on cheek and upper lip with histology w/ prominent squamous eddies

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13
Q

Tan-flesh colored warty papules on dorsal hands and feet, autosomal dominant disorder of keratinization often a/w Darier disease, ATP2A2 mutations with histology with “church spire” is called?

A

Acrokeratosis verruciformis of Hopf

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14
Q

Solitary erythematous papule on lower leg with “wafer-like scale” at the periphery is?

A

Clear cell acanthoma (Degos acanthoma)

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15
Q

Nevus comedonicus is associated with what mutation?

A

FGFR2

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16
Q

Erythroplasia of Queyrat?

A

Juicy red, erosive plaque on glans penis >30% progress to invasive SCC, HPV-16 is #1 cause.

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17
Q

Drugs that increase SCC risk?

A

Voriconazole
Vemurafenib
Vismodegib
AZA

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18
Q

Systemics for SCC?

A

Cemiplimab or pembrolizumab if surgery or advanced or metastatic disease

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19
Q

Factor related to SCC DEAth?

A

DIAmeter

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20
Q

Low-grade, locally destructive SCC a/w HPV-6 and HPV-11 is?

A

Verrucous carcinoma

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21
Q

Rapid onset of multiple KA’s with onset third decade, sun-exposed areas,
and resolves spontaneously is called?

A

Ferguson-Smith

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22
Q

1000s of milia-like KAs in later
adulthood may involve airway a/w scarring, ectropion, and mask-like facies is called?

A

Grzybowski - Mnemonic: “Old (later onset) Grizzlies Growl” -> airway affected

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23
Q

“shark-tooth” or “dragon scale” on histology is seen in?

A

Steatocystoma

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24
Q

Steatocystoma multiplex is due to mutations in?

A

KRT17 mutations

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25
Q

Midline anterior neck swelling vs. lateral neck are?

A

Thyroglossal duct cyst - midline anterior
Branchial cleft cyst - lateral neck

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26
Q

Freckles are called?

A

Ephelides

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27
Q

Multiple CALMs may be seen with?

A

■ NF1 > NF2
■ McCune-Albright syndrome
■ Russell-Silver
■ Noonan syndrome
■ Bloom syndrome
■ Tuberous sclerosis
■ MEN-I
■ Fanconi anemia
■ Ataxia-telangiectasias
Histology: ↑ melanin deposition in basilar keratinocytes

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28
Q

Conditions a/w multiple lentigines?

A

■ LEOPARD, most heavily “spotted” appearance*
■ Carney complex (LAMB/NAME)*
■ Peutz-Jeghers, perioral*
■ Bannayan-Riley-Ruvalcaba, penile*
■ Laugier-Hunziker
■ Cowden syndrome
■ Xeroderma pigmentosum
■ Cronkhite-Canada

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29
Q

Risks of nevus of Ota?

A

10% develop glaucoma and uveal melanoma (rare malignant degeneration)

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30
Q
A

Hori’s nevus, acquired nevus of Ota-like macules of the bilateral zygomatic region seen in East Asian females

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31
Q
A

Nevus of Ito, located on the shoulder, supraclavicular and scapular regions, no risk of progression to melanoma

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32
Q

Most common sites for blue nevus?

A

Scalp, sacral area, and distal extensor extremities

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33
Q

Multiple blue nevi and epithelioid blue nevi seen with?

A

Carney complex

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34
Q

Risk with blue nevi?

A

Uveal melanoma (GNAQ and GNA11) and metastasis and death if concomitant BAP-1 loss

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35
Q

Subtype of blue nevus seen in Carney complex?

A

Epithelioid blue nevus, PRKAR1A and PRKCA mutations

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36
Q
A

“Cellular blue nevus” Dense proliferation of plump/fusiform pale gray melanocytes containing little pigment characteristically bulges into subcutis “dumbbell configuration”

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37
Q

Spitz nevus mutations?

A

HRAS mutations/11p gain

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38
Q

On histology, pink clumps of basement membrane zone, material (collagen IV) within epidermis are called?

A

Kamino bodies seen in Spitz nevus

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39
Q

Immunostains in spitz nevus?

A

S100A61, S1001, Melan-A1, and p161

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40
Q

Deep penetrating nevus mutation?

A

Activating b-catenin mutations

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41
Q

Congenital melanocytic nevus mutation?

A

NRAS

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42
Q

Mutations associated with melanoma?

A

Sun-damaged areas, acral or mucosal -> C-KIT or CCND1/CDK4
Uveal melanoma, blue nevi, and nevus of Ota -> GNAQ/GNA11

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43
Q

A benign sweat gland neoplasm that presents as a solitary, vascular-appearing papule, +/- ulceration and bleeding; is called?

A

Poroma

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44
Q

Poromatosis?

A

widespread or acral eruption of poromas

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45
Q

Poroma stains?

A

CEA, EMA, and PAS highlight ducts and intracytoplasmic lumina

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46
Q

What are the 3 poroma variants?

A

1- Wholly intraepidermal poroma - mistaken for SK, on distal extremities
2- Juxtaepidermal poroma - classic
3- Wholly dermal poroma - “blue balls/nodules within dermis”

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47
Q

Most common sweat gland malignancy?

A

Porocarcinoma

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48
Q

A benign sweat gland neoplasm (apocrine>eccrine) that presents as a solitary nodule (often multilobulated) with a deep red-purplish hue and cystic quality is called a?

A

Hidradenoma

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49
Q

“Prominent dermal sclerosis with keloidal collagen” clues towards?

A

Hidradenoma

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50
Q

Hidradenoma is composed of 3 main cell types?

A

Squamoid cells, poroid cells, and clear cells

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51
Q

Most common location for Syringoma?

A

Periorbital region (eyelids #1), cheek > anterior trunk, genitals

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52
Q

Solitary, painful dermal or subcutaneous nodule with blue-purple hue; favors upper half of body is?

A

Spiradenoma

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53
Q

Cylindroma’s usually occur on the?

A

Scalp

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54
Q

A “turban tumor” seen in Brooke-Spiegler syndrome is basically a cluster of?

A

Cylindroma’s

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55
Q

Benign, painless 1- to 2-cm skin colored nodule; almost exclusively on vulva (labia majora #1) of young adult women?

A

Hidradenoma papilliferum
“maze-like” hits

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56
Q

Benign apocrine neoplasm; presents at birth or early childhood with a solitary, warty papule/plaque on scalp (. other sites on head/neck . trunk and extremities); usually a/w nevus sebaceus is a?

A

Syringocystadenoma papilliferum

57
Q

Papillary eccrine adenoma usually occur on?

A

Legs #1, favors Black women
Also Syringofibroadenoma on the legs

58
Q

Syringofibroadenoma are seen in?

A

Schöpf-Schulz-Passarge
Clouston syndrome
Chronic stasis dermatitis (reactive process)
Burns and scarring

59
Q

Thin, anastomosing strands of sweat duct-
containing epithelium projecting downward from epidermis into mid dermis seen in?

A

Syringofibroadenoma

60
Q

Firm, indurated plaque on lip >chin and cheek of middle-aged women that is locally aggressive is likely?

A

Microcystic adnexal carcinoma
Tx: Mohs

61
Q

Microcystic adnexal carcinoma lineage?

A

Locally aggressive adnexal carcinoma with divergent, bi-lineage, differentiation “follicular + sweat gland”

62
Q

Histo deeply-infiltrative sclerosing basaloid proliferation with divergent/bi-lineage differentiation (mixture of small sweat ducts 1 keratinizing “microcysts”); typically has PNI and prominent lymphoid aggregates?

A

microcystic adnexal carcinom

63
Q

Tx of digital papillary adenocarcinoma?

A

Its aggressive digital papillary adenocarcinoma, tx: amputation

64
Q

Cutaneous mucinous carcinoma on the face vs. on the trunk?

A

Mucinous carcinomas on the face are almost always primary, whereas lesions arising on trunk may represent metastasis of visceral malignancy, GI, breast, lung, or ovarian.

65
Q

Basaloid epithelial tumor nodules “floating in lakes of mucin,” sialomucin, intratumoral ducts give rise to cribriform appearance, CK7+, CK20- is?

A

Primary cutaneous mucinous carcinoma

66
Q

“blue balls within dermis”

A

Dermal duct tumor, wholly dermal poroma variant

67
Q

Well-circumscribed proliferation of multiple small-to-medium-sized blue tumor lobules encircled by thick hyaline BMZ material (type IV collagen mainly) → leads to “jigsaw puzzle” pattern; scattered small ducts; biphasic cell population?

A

Cylindroma

68
Q

Primary cutaneous mucinous carcinoma stains?

A

p63/p401, SMA1, calponin1, SOX101, and S1001

69
Q

Extensive nevus sebaceous think of?

A

Schimmelpenning syndrome or phakomatosis pigmentokeratotica

70
Q

Syndromes a/w multiple trichoepitheliomas?

A

○Brooke-Spiegler syndrome: CYLD mutation, multiple trichoepitheliomas, trichoblastomas, spiradenomas, and cylindromas
○Rombo syndrome: atrophoderma vermiculatum, hypotrichosis, acro-facial vasodilation and cyanosis, milia, and multiple BCCs

71
Q

well-circumscribed; complex cystic proliferation with internal “rolls and scrolls” appearance is?

A

Pilomatricoma

72
Q

Schwannoma (neurilemmoma) is?

A

Benign proliferation composed almost entirely of Schwann cells (S100+, stains Schwann cells) with perineurial capsule (EMA+, stains perineurial capsule)

73
Q

Plexiform neurofibromas, “bag of worms”, stain with?

A

S100+, neurofilaments+

74
Q

Cellular neurothekeoma stains?

A

Boards favorite: always S100 negative (vs. classic “neurothekeoma”); but S100A6+, NKI/C3+, and PGP 9.5+

75
Q

Aggressive malignant neoplasm (deadlier than melanoma); most common in elderly on head/neck; UV exposure is major risk factor; ↑ risk w/ immunosuppression is?

A

Merkel cell carcinoma

76
Q

densely cellular proliferation of atypical spindle cells often with large areas of necrosis (“geographic necrosis”) and a high mitotic rate is seen in?

A

Malignant peripheral nerve sheath tumor

77
Q

Histology: infiltrative or nodular dermal/subcutaneous mass composed of sheets of uniform basaloid cells with high N:C ratio and finely speckled “salt-and-pepper” chromatin (usually without prominent nucleoli); numerous mitoses (often > 30/mm2) and apoptotic cells

A

Merkel cell carcinoma

78
Q

Merkel cell carcinoma stains?

A

■ Positive stains: CK20 (paranuclear dot pattern, highly sensitive), neurofilaments (paranuclear dot pattern, highly sensitive), chromogranin/synaptophysin, NSE, EMA, and CD56; MCPyV+ (80%; polyomavirus)
■ Negative stains: TTF-1, CK7, S100, and CEA

79
Q

if mcc, CK20-, THINK OF?

A

Small cell lung carcinoma (main consideration!

80
Q

Recurrent locally advanced MCC tx?

A

pembrolizumab

81
Q

Primitive neuroectodermal tumor (neuroblastoma) px?

A

metastatic disease → p/w multiple blue nodules on trunk and extremities
“Raccoon eyes” (periorbital darkening/purpura) from orbital metastases
↑ Urinary catecholamines (>90%)

82
Q

Red-brown papules on trunk or extremities, Painful, especially with cold exposure, Pseudo Darier sign?

A

Pilar leiomyoma, Benign proliferation of smooth muscle arising from arrector pili

83
Q

Multiple Pilar leiomyoma can occur as part of?

A

Reed syndrome, AD inheritance, fumarate hydratase mutations, multiple cutaneous and uterine leiomyomas and RCC

84
Q

Pilar leiomyomas stains?

A

Desmin+, SMA+, and caldesmon+; smooth muscle fibers are pink-red with Masson trichrome stain

85
Q

Bright pink cytoplasm and “cigar- shaped” nuclei?

A

Pilar leiomyoma

86
Q

Painful Skin Lesions?

A
87
Q

Most commonly middle-aged females on lower extremity; frequently painful?

A

Angioleiomyoma, desmin+ (differentiates from myopericytoma), SMA+, calponin+ and h-caldesmon+

88
Q

MF immunophenotype?

A

■ Typical phenotype: CD3+/CD4+/CD8- mature T-lymphocytes
■ Variable loss of pan T-cell markers: CD7 loss (most common, least specific) > CD5 and CD2 loss (less common, more specific)

89
Q

Hypopigmented MF immunophenotype?

A

CD4- /CD8+

90
Q

Adult T-cell leukemia/lymphoma px with?

A

leukemia, lymphadenopathy, organomegaly, hypercalcemia, and skin lesions

91
Q

Sezary syndrome histology?

A

Histopathology resembles MF, but has characteristic “floret” or “clover-leaf” malignant T cells
■ Immunophenotype: CD4+/CD8-/CD25+, PD-1+

92
Q

Wedge-shaped infiltrate with clusters of large, atypical, Reed-Sternberg-like CD30+ (Ki-1) lymphocytes are seen in?

A

Type A Lymphomatoid papulosis

93
Q

Lymphomatoid papulosis ALCL-type?

A

Type C (ALCL-type, 10%)

94
Q

Angioinvasive LyP?

A

Type E, CD8+/CD30+

95
Q

What associated malignant lymphoma associated with LyP?

A

MF > Hodgkin disease, ALCL > others

96
Q

Extranodal NK/T-cell lymphoma, nasal type immunophenotype?

A

CD2+/CD56+ and CD3+

97
Q

Leukemia cutis is most commonly due to?

A

Acute myeloid leukemia

98
Q

Violaceous, usually solitary papule or nodule on the scalp/forehead or back (“Crosti lymphoma”) is what type of cutaneous B-cell lymphoma?

A

Primary cutaneous follicle center cell lymphoma

99
Q

Dermatofibroma positive stains?

A

Factor XIIIa
CD10 strong, diffuse
Stromelysin-3, distinguishes from DFSP

Negative stains: CD34, S100, and pan-keratin

100
Q

Translocation for Dermatofibrosarcoma protuberans?

A

t(17;22) COL1A1-PDGFB fusion

101
Q

Mnemonic: “DFSP affects people 17 to 22 years old Called Pat”…

A

Remember young age (17–22) and the order of the fused genes:
17 = COL1A1
22 = PDGFB

102
Q

Monotonous spindle cells (cells are more bland and uniform than DF!) with a storiform architecture in dermis and throughout SQ fat; characteristic “honeycomb” infiltration of fat; 5% with melanin (Bednar tumor)
■ CD34 strongly positive

A

Dermatofibrosarcoma protuberans

103
Q
A

Zycomycosis, Bull’s –eye cutaneous infarcts

104
Q

Cell types in atypical fibroxanthoma?

A

1) spindle cells
2) histiocyte-like cells
3) xanthomatous cells
4) bizarre, multinucleated giant cells

105
Q

Atypical fibroxanthoma differential?

A

“SLAM” DDx
■ SCC (sarcomatoid/spindle cell variant): stains positively with high-molecular-weight keratin (CK903 and CK5/6) or broad-spectrum keratin (MNF-116), p63 and p40 (newest and most specific marker)
■ Leiyomyosarcoma: desmin+, SMA+
■ AFX: negative for high-molecular-weight keratin, p63, p40, S100, SOX10, and desmin
■ Melanoma

106
Q

Histology of Sclerotic fibroma?

A

“plywood” or “starry night” pattern

107
Q

Pleomorphic fibroma clinically resemble?

A

Skin tags

108
Q

Multiple, grouped red papules on dorsal hands or legs of women in their 40s; benign?

A

Multinucleate cell angiohistiocytoma
w/ characteristic multinucleated giant cells and prominent proliferation of dilated vessels in dermis

109
Q

Solitary, pyogenic granuloma-appearing papules; most
common on thighs of 50-year-old women?

A

Epithelioid cell histiocytoma
Resembles spitz nevus histologically w/ epidermal collarette

110
Q

A protruding, skin tower from fingers or feet?

A

Acral fibrokeratoma

111
Q

Fibromatosis types?

A

Superficial variants:
○Palmar (Dupuytren’s)
○Plantar (Ledderhose)
○Penile (Peyronie’s)
○Knuckle pads

Deep desmoid tumors a/w Gardner syndrome, b-catenin mutations, and stain b-catenin+

112
Q

What histology looks like pseudo sarcoma?

A

Nodular fasciitis

113
Q

Biphasic proliferation, with hypo cellular and hyper cellular areas, staghorn (“hemangiopericytoma-like”) vessels, SMA+ tram-track seen in?

A

Myofibroma

114
Q

Numerous osteoclastic giant cells?

A

Giant cell tumor of tendon sheath

115
Q

Buschke-Ollendorf ?

A

Elastomas + osteopoikilosis

116
Q

Angiokeratoma’s in children?

A

Angiokeratoma of Mibelli: 10 to 15 years old; fingers and toes

117
Q

Angiokeratoma’s in vulva or scrotum?

A

Angiokeratoma of Fordyce: older men (scrotum) or females (vulva)

118
Q

Angiokeratoma’s in Fabry’s disease?

A

Angiokeratoma corporis diffusum: multiple lesions in childhood/adolescence; bathing suit distribution; seen in Fabry disease and other enzyme deficiencies

119
Q

Angiokeratoma’s that aggregate and form a plaque?

A

Angiokeratoma circumscriptum

120
Q

Epithelioid hemangioma is also known as?

A

Angiolymphoid hyperplasia with eosinophils

121
Q

Angiolymphoid hyperplasia with eosinophils is usually seen on?

A

Ear* Head/neck

122
Q

Red-brown papules with “target-like” appearance (dark centrally → pale area is first ring → bruise-like patch in outer ring) is called?

A

“hobnail hemangioma” or “targetoid hemosiderotic hemangioma”

123
Q

“cannonball” pattern; a characteristic empty-appearing “crescent” surrounds the periphery of each lobule?

A

Tufted angioma

124
Q

Histology (Boards favorite): well-circumscribed dermal proliferation comprised of dilated vessels that are filled centrally with a small ball of well-formed capillary loops → resultant architecture resembles renal glomeruli

A

Glomeruloid hemangioma

125
Q

Rare vascular tumor of childhood; a/w Kasabach-Merritt phenomenon; GLUT-1 negative?

A

Kaposiform hemangioendothelioma
- Positive for CD34 and CD31
- Negative for Factor VIII

126
Q

Stewart-Treves syndrome?

A

Angiosarcoma seen in chronic lymphedema associated, mostly following breast cancer treatment with axillary LN dissection

127
Q

Angiosarcoma immunostains?

A

ERG+ (most sensitive and specific), CD31+, CD34+, and FLI-1+
c-MYC amplications (detected by
immunostaining or FISH)

128
Q

Genetic lipoma?

A

↑ HMGA2 gene

129
Q

Conditions a/w multiple lipomas?

A

■ Familial multiple lipomatosis: AD; multiple painless
lipomas on trunk/extremities
■ Madelung disease: multiple large lipomas around neck/
shoulders; generally in middle-aged alcoholic males
■ Gardner syndrome
■ Bannayan-Riley-Ruvalcaba syndrome
■ Proteus syndrome
■ CLOVES syndrome (Congenital Lipomatous
asymmetric Overgrowth, Vascular malformations,
Epidermal nevi, Skeletal and spinal anomalies)
■ PTEN hamartoma tumor syndrome

130
Q

Benign tumors of brown fat?

A

Hibernoma

131
Q

Histo of Hibernoma?

A

Hibernoma cells (polygonal cells with
eosinophilic, multivacuolated cytoplasm) admixed with
normal appearing adipocytes

132
Q

“floret-like” cells seen in?

A

Pleomorphic lipoma
Has thick “ropey” collagen fibers

133
Q

Mutation detected in well-differentiated liposarcoma?

A

MDM2 amplification (12q13-15 ring/giant chr)

134
Q

Rare type of liposarcoma; most testable point is its characteristic histology (buzzword: “chicken-wire” vessels)?

A

Myxoid/round cell liposarcoma

135
Q

Kasach-Merritt syndrome seen with?

A

Tufted hemangioma
Kaposiform hemangioendothelioma

136
Q

Dermoscopy of SK?

A
  • Milia-like cysts
  • Comedo-like openings
  • Fissures and ridges
  • Moth-eaten borders
  • Sharp demarcation
  • Fingerprint-like pattern
137
Q

“Strawberry pattern” is seen on dermoscopy of what lesion?

A

Actinic keratosis

138
Q

Dermoscopy of BCC?

A
  • Leaf-like structures at periphery
  • Blue-gray ovoid nests and globules
  • Pigmented specks
  • Spoke-wheel structures/concentric structures
  • Arborizing(branch-like) telangiectasias (non-superficialBCCs)
  • Serpentine vessels (superficial BCCs)
  • Ulceration/erosion
139
Q

Vessels seen on dermoscopy of SCC in situ?

A

Clusters of glomerulus-like (coiled) vessels

AliKhan Derm (13 decks)

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