Neoplasms Flashcards

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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
Q

Diagnosis?

Charateristic Features

A

Nodular Hidradenoma

Cell Types: Poroid Cells, Squamoid Cells, Clear Cells

Large Areas of Cystic Degeneration

Dermal Sclerosis and Keloidal Collagen

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26
Q

Eruptive Syringomas

A

Down Syndrome

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27
Q

Clear Cell Syringoma

A

DM

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28
Q

Pleomorphic Adenoma

A

Oral variant of mixed tumor

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29
Q

chondroid syringoma

A

mixed tumor

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30
Q

Spiradenoma Syndromes

A

Brooke-Spiegler (CYLD)

Spiradenocarcinoma more common (arising withing spiradenoma)

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31
Q

Diagnosis?

Common Location?

A

Adenoid Cystic Carcinoma

Primary Cutaneous ACC: Scalp

Or Met OR primar salivary gland ACC (bad!)

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32
Q

Calcifying Epithelioma of Malherbe

A

Pilomatricoma

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33
Q

Pilomatricoma, Mutation

A

CTNNB1 (encodes B-catenin)

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34
Q

Conditions with Multiple Pilomatricomas

A

Myotonic Dystrophy

Turner Syndrome

Garder Syndrome (Familial Adenomatous Polyposis)

Rubinstein-Taybi

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35
Q

Stains for Trichilemmoma

A

CD34 (Outer Root Sheath Differentiation)

Pan-keratin

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36
Q

Trichilemmal Cyst

A

Pilar Cyst

Outer Root Sheath Differentiation

Isthmic Differentiation

No granular layer

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37
Q

Schwannoma Stains

A

S-100, EMA+

Negative for Neurofilaments

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38
Q

Neurofibroma Stains

A

S-100+

Neurofilaments+

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39
Q

Bilateral Acoustic Neuromas

A

NF-2

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40
Q

Neurothekeoma (cellular)

A

S100-

S-100A6+, NKI/C3+, PGP9.5+

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41
Q

Pustulo-ovoid bodies on milan

A

aggregated lysosomes

granular cell tumor

42
Q

Neuroblastoma

A

cutaneous mets are presenting sign in 30% with metastatic

NSE+ and neurofilaments+

43
Q

Reed Syndrome

A

Pilar Leiomyomas

AD

Fumarate Hydratase

Uterin leiomyomas and RCC

44
Q

Large Cell Transformation in MF

A

>25% large cells (> 4 times the normal size)

45
Q

Hypopigmented MF

A

CD4-, DC8 +

46
Q

Woringer-Kolopp

A

Pagetoid Reticulosis

47
Q

Definition of Sezary Syndrome

A

CD4+ cells > 1000 cells/uL

48
Q

Granulomatous Slack Skin Association

A

30% develop Hodgkin’s lymphoma

49
Q

Another name for CD30

A

Ki 1

50
Q

Foliculotropic MF (staining)

A

CD3+CD4+ CD8-

51
Q

Adult t-cell leukemia/lymphoma staining

A

CD3+CD4+

CD8-

CD25+

52
Q

Types of LyP

A

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
Q

Primary Cutaneous Anaplastic Large Cell Lymphoma

A

Sheets of CD30+ cells (>75% of cells)

CD4+

(Negative EMA and negative ALK translocations (positive in systemic ALCL)

54
Q

Extranodal NK/T-cell lymphoma, nasal type

Association

Stains

A

EBV+

CD2+/CD56+, CD3+

55
Q

Subcutaneous panniculitis-like T-cell lymphoma

A

CD4-, CD56-

CD8+

TIA1+

Granzyme B+

a/B phenotype

Good prognosis

56
Q

Primary Cutaneous y/d T-cell lymphoma

A

CD4-, CD8-

CD56+, TIA-1+

B-F1 -

57
Q

Agressive epidermotropic cytotoxic (CD8+) T-cell lymphoma

A

Eruptive Ulcerative Tumors

Epidermotropic and angiodestructive

Histological DDX for CD8+

MF, pagetoid reticulosis, type D Lyp

58
Q

CD117

A

c-kit

59
Q

Primary Cutaneous CD4+ small/medium pleomorphic T-cell lymphoma

A

solitary plaque or nodule

CD4+, CD8-, CD30-

Histologically same as MF

60
Q

Primary Cutaneous Follicle Center Lymphoma

A

CD20+, CD79a+, bcl-6+, bcl-2-

61
Q

Primary Cutaneous Marginal Zone Lymphoma

A

CD20+

bcl-2+, bcl-6 -

62
Q

Primary Cutaneous diffuse large B-cell lymphoma

A

MUM-1+, bcl-2+, bcl-6+

5 year survival 50%

63
Q

Multiple Facial Angiofibromas

A

Tuberous Sclerosis

MEN1

Birt-Hogg-Dube

64
Q

Diagnosis?

Association?

A

Sclerotic Fibroma

Cowden’s

65
Q

Pleomorphic Fibroma-Stain

A

CD-34+

66
Q

Path with giant cells and multiple dilated vessels in the dermis

Diagnosis?

A

Multinucleate Cell Angiohistiocytoma

67
Q

Dermatomyofibroma

Staining?

A

CD-34 negative

68
Q

Inclusion body fibromatosis

Inclusions made of ???

A

Actine filaments (SMA+, red w/ trichrome)

69
Q

Myofibroma Stain

A

SMA+

70
Q

Syndromes associated with connective tissue nevi

A

tuberous sclerosis (shagreen patch)

Buske-Olendorf (Elastomas and Collagenomas) +Osteopoikilosis

Proteus (Collagenomas)

MEN-1: collagenomas

71
Q

Port Wine Stain Associations

A

Mafucci sydrome

K-T

Sturge- Webber

Blue Rubber Bleb

Proteus

72
Q

Increased risk of infantile hemangiomas

A

premature

females

placental abnormalities

73
Q

Epithelioid hemangioma

A

Same as ALHE

74
Q

Tufted Hemangioma

A

Kasabach-Merrit Phenomenon

75
Q

Glomeruloid Hemangioma

A

Polyneuropathy

Organomegaly

Endocrinopathy

Monoclonalgammopathy

Skin syndrome

76
Q

Kaposiform Hemangioendothelioma

Staining

A

CD-34 + (restricted to luminal vs tufted where they are diffuse)

CD-31 +

77
Q

Kaposi’s Sarcoma Types

A

Classic: elderly mediterranean

African Endemic: young males; lymph nodes involved and rapidly progressive

Iatrogenic

AIDS-associated

78
Q

Kaposi’s Sarcoma

Stains

A

LANA-1 (latency associated antigen 1)- nuclear positivity

79
Q

Angiosarcoma Stains

A

CD31+

CD34+

ERG+

FLI-1+

Ulex europeus lectin

c-MYC in post-radiation (negative in atypical vascular lesions)

80
Q

Conditions with Multiple Lipomas

A

Familial Multiple Lipomatosis

Madelung’s disease

Gardner

Bannayan-Riley-Ruvalcaba

Proteus

CLOVES

PTEN hamartoma

81
Q

Spindle Cell Lipoma Staining

A

CD-34+

82
Q

Angiomyolipoma

A

No assication with tuberous sclerosis

Considered Angioleiomyoma with mature fat

83
Q

Liposarcoma marker

A

MDM2 (>99%)

84
Q

Pustulo-ovoid bodies of Milan

A

granules in granular cell tumor (lysosome and golgi material)

85
Q

CD34 Tumors

A

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
Q

Endothelial stains

A

ERG

FLI-a

Ulex europaeus agglutinin 1

vimentin

87
Q

Histiocyte Stains

A

CD 68, CD163, lysozyme

88
Q

Myofibroblast Stains

A

SMA (do not stain with desmin)

89
Q

Natural Killer Cells

A

CD 56

90
Q

Plasma Cells

A

CD138

91
Q

Sebaceous Gland Stains

A

EMA

adipophilin

androgen receptor

92
Q

CAM 5.2

A

Pagets and extramammary pagets

93
Q

Stromelysin 3+

A

positive in DF, negative in DFSP

94
Q

Modifier 25

A

Significant separate E/M on same day as procedure

95
Q

Modifier 50

A

bilateral procedure

96
Q

Modifier 58

A

Staged procedure within the postoperative global period

97
Q

Modifier 59

A

distinct service, ie two separate procedures

98
Q

Modifier 79

A

New procedure in the global period of a prior procedure

99
Q

Destruction, excision, and linear repair global period

A

10 days

100
Q

Flap, graft Global Period

A

90 days