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Dermatology > Dermal And Subcutaneous Tumors > Flashcards

Dermal And Subcutaneous Tumors Flashcards

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Dermatology Board Prep flashcards
1
Q

Hamartoma

A

Abn arrangement of tissues normally present ata given site

Nevus - nl arrangement

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2
Q

Revised phakomoatosis pigmentovascularis

A

Phakomatosis cesioflammeus - blue spots and PWS
Phako spilorosa - Nevus spilus and a pale pink spot
Pkako cesiomarmarota - blue spots and cmtc

A - only cutaneous findings
B - dz associated

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3
Q

Phakomatosis pigmentovascularis associated findings

A

Intracranial and visceral vascular anomalies
Ocular
Choroidal melanoma
Hemihypertrophy

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4
Q

MC phako pigmentovascularis

A

Type II

Half have KTW or parkes Webber
BL deafness and malignant HTN described

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5
Q

Type III phako association

A

Multiple GCT

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6
Q

Eccrine angiomatous hamartoma

A

Acral, particularly palms and soles
Painful
Hyperhidrosis
Associated with spindle cell hemangioma, AVM, verrucous hemangioma

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7
Q

Nevus anemicus

A

Pale macules that cannot be made red
Associated with TS, NF and phako pigmentovascularis
Increased sensitivity to catecholamines

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8
Q

Nevus oligemicus

A

Livid skin patch cooler than normal

Due to vasoconstriction

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9
Q

CMTC common anomalies

A

Varicosities, PWS, ulceration, macrocephaly, hypo/hyper of bone

Unusual associations - generalized congenital fibromatosis, premature ovarian failure, chairi I, rectal and genital anomalies

High copper and incr elastolysis have been described

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10
Q

Bockenheimer syndrome

A

Progressive venous ecstasias of one limb

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11
Q

Reports of this occurring in PWS

A

BCC

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12
Q

SWS

A

Fibronectin gene expression incr in lesional fibroblasts

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13
Q

Roberts syndrome

A

Facial PWS, hypomelia, hypotrichosis, growth retardation and cleft lip

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14
Q

Wyburn mason syndrome

A

UL retinal AVM with IL PWS near ear

TAR - also has PWS

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15
Q

Coats disease

A

Retinal telangectasias with IL PWS

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16
Q

PWS

A

Increased VEGF and its receptor VEGFR2

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17
Q

Gorhams disease

A

Cutaneous and osseous venous and lymphatic malf with osteolysis

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18
Q

Novel sclero agent for VM

A

Ethanolamine

Soft tissue injury and neuropathy found

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19
Q

Earliest and MC presentation of KT

A

PWS of extremity

VG5Q mutation associated with KTS

Ulcers may respond to sunitinib

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20
Q

Cirsoid aneurysms

A

Congenital AV fistulas of scalp and face

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21
Q

MC in lymphangiomatosis

A

Chylothorax 49%, pulmonary infiltrates 45%, bone lesions, splenic lesions, cervical inv, DIC

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22
Q

Gorham stout

A

Lymphangiomatosis, chylous effusions, esteolytic bones

Response to peg-IFN reported

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23
Q

Lupus vs scleroderma nailfold changes

A

Tortuous glomeruloid loops in lupus

Dilated loops and avascular in scleroderma

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24
Q

GET vs telangectasias of systemic dz

A

GET vessels do not have alk phos activity

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25
Q

Unilateral nevoid telangectasias

A

MC involve trigeminal, C3 and C4, R>L

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26
Q

Acral pseudolymphomatous angiokeratomas in children (apache)

A

UL and sporadic
No cold sensitivity
Variant of pseudolymphoma

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27
Q

Kimuras disease

A

Massive periauricular and submandibular swelling in young Asian men
Prominent germinal centers with eos
Associated with allergic dz

Coexistence of alhe with T cell lymphoma reported

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28
Q

IPEH

A

Head, neck or upper ext

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29
Q

Angioma serpiginosum

A

No alk phos vs normal capillaries

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30
Q

MC vascular anomaly

A

Cherry angiomas

Consider amyloidosis if lesion surrounded by a purpuric halo
Eruptive lesions described with nitrogen mustard

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31
Q

Targetoid hemosiderotic hemangioma

A

Post traumatic
Biphasic pattern with central superficial dilated vessels lined by hobnail endothelium and deeper collagen dissecting vessels
Stain CD31 but not 34

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32
Q

Glomeruloid hemangioma

A

Poems, castlemans, ITP, sjogrens

Lesions associated with poems show incre VEGF and flt-1 receptor

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33
Q

Microvenular hemangioma

A

Forearms of young to middle aged adults
Monomorphous elongated bld vessels in reticular dermis
May be seen in POEMS

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34
Q

TA vs KHE vs IH

A

TA - CD34 pos endothelial cells and few actin positive cells
KHE - CD34 pos ONLY in luminal endothelial cells
IH - actin positive cells > CD34 cells

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35
Q

Angioblastoma

A

Rare pediatric tumor associated with destruction of regional structures

Elevated basic fibroblast growth factor

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36
Q

KHE

A

Common,y occurs retroperitoneal
Distant mets NOT reported
Associated with nonne Milroy disease
Transcription factor PROX-1 shown to induce proliferation and deep extension

Prognosis depends on depth and location

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37
Q

MLT

A

Endothelial cells show high proliferation index with ki-67 stain
LYVE-1 positive

38
Q

Avg age of kassabach Merritt

A

7 weeks

Most deaths due to bleeding complication, 30% mortality

39
Q

Glomus cells

A

Modified smooth muscle

Stain with vimentin (not desmin), SMA often positive

40
Q

Adult hemangiopericytoma

A 
Staghorn ectatic spaces
wLE is tx of choice
20% mets rate
MC COD is pulmonary mets
Infantile lesions do not metastasize
41
Q

Proliferating angioendotheliomatosis

A

2 types, reactive involuting and malignant
Reactive occurs in endocarditis, chagas, TB, severe atherosclerosis, cryoproteinemia, periodontal dz and antiphospholipid ab

Diffuse dermal angiomatosis associated with CAD

Occurs on thigh most often

May show amyloid deposits or HHV8

Malignant type considered intavascular lymphoma, LCA positive

42
Q

Hemangioendothelioma

A

Blue firm nodules on a child or young adult distal ext
Histo resembles hemmorrhagic lung
Phleboliths common
Benign and due to reactive

43
Q

MC location for classic KS

A

Toes or soles

AIDS associated predilection for head, neck, trunk and MM

44
Q

MC site of internal involvement for KS

A

GI, especially SI

Bone inv indicates widespread dz

African KS frequently has bone inv and lymphedema

45
Q

MC location for AIDS KS

A

GI, LN and lungs

25% have only cutaneous inv

46
Q

Increased risk of malignancies in KS

A

HL, T cell lymphoma, NHL

20 x incr risk

47
Q

First line for advanced KS

A

Liposomal anthracyclines

Lesions are radiosensitive

48
Q

Epitheliod hemangioendothelioma

A

Slow growing on distal ext
Male predominate
Dilated vascular channels and spindle cells
Wide excision with LN evaluation (usual site of mets)

49
Q

Retiform hemangioendothelioma

A

Low grade angiosarcoma
Upper or LE of young adults
hHV8 reported

50
Q

Endovascular papillary angioendothelioma

A

Dabska tumor

Low grade angiosarcoma on head neck of infants/children

51
Q

Angiosarcoma

A

M>F
Sudden thrombocytopenia may herald mets or enlarging pulmonary tumor
Virtually all malignant tumors positive for podoplanin
Excision followed by radiotherapy
MC COD are lung mets
23 yrs btw radiation and development of angiosarcoma if preceding condition was benign, if preceding illness was malignant then 12 yrs

52
Q

MC location for keloids

A

Sternum

Paucity of elastic tissue like a scar
Incr mucopolysacharides and mast cells
Anti TGF beta and NFkb inhibition promising therapy
Also green tea polyphenol and epigallocatechin-3-gallate
5FU, CCBs show some improvement
Verapamil decr IL6 and VEGF
Silicone sheets reduce MC and decrease itching
ILTAC at 2 week intervals

53
Q

Dupuytrens

A

Associated with alcoholic cirrhosis, diabetes and epilepsy
Nodules composed of myofibroblasts that express androgen R
Lack beta catenin and APC mutations unlike deep fibromatosis

54
Q

Knuckle pads (heloderma)

A

PIP joints
Freely movable
AD form associated with hearing loss and leukonychia
K9 mutation with PPK and knuckle pads

55
Q

Pachydermodactyly

A

Fullness of the medial and lateral digit proximal to PIP
Swelling from incr collagen and mucin
Form associated with TS

56
Q

Desmond tumor

A

Arises from muscular aponeurosis
MC on abdomen, especially pregnant F
Dangerous if at root of neck or intraabdominal

57
Q

Collagenous fibroma (desmoblastic fibroma)

A

Slow growing deep benign
Characterized by hypocellularity and hyalinized collagen that may infiltrate skeletal m
(2;11)(q31;q12) reported
Positive for vimentin, may stain with actin
Negative cd34, s100, keratin

58
Q

Infantile myofibromatosis

A

MC fibrous tumor of infancy
Head and neck
60% congenital or soon after birth
Skeletal lesions (metaphysial bone) in 50%
Females more likely to get generalized dz
If widespread 80% die of obstruction or compression of an organ

59
Q

Pseudo ainhum

A

Vohinkel, mal de meleda, PC
ED
EPP

Use pseudo when associated with hereditary conditions

60
Q

Familial collagenomas

A

Associated with ASD

Eruptive collagenomas in syphylis

61
Q

Cellular angiofibroma

A

Genitals of older F
Express E and progesterone R
CD34

62
Q

Superficial acral fibromyxoma

A

Cd34, cd99, EMA

63
Q

Oral submucous fibrosis

A

West pacific and south Asia who eat chili and chew betel
7% risk of SCC
Andr 28-28

64
Q

Cutaneous pseudosarcomatous polyp and umbilical polyp

A

Benign skin tag like proliferations
Dramatic atypia
Pos vimentin

65
Q

Tag like BCC

A

Should suggest gorlins

66
Q

Dermal dendrocyte hamartoma

A

Round medallion like lesion on upper trunk
CD34, factor XIIIa
Fig 28-30

67
Q

Nodular fasciitis

A

MC arms

68
Q

Solitary fibrous tumor

A

Mediastinum

CD34 and progesterone R

69
Q

Plexiform fibrohistiocytic tumor

A 
F>M
Slowly growing painless
Histiocyte like and osteoclast like GC
GC label vimentin and CD68
Spindle cells for sma
70
Q

DFSP

A

CD34, stromeolysin-3 and nesting positive vs DF

71
Q

MC type of mastocytosis

A

UP

72
Q

H/o of Hymenoptera induced anaphylaxis and elevated tryptase should be tested for

A

Systemic masticytosis

MC express CD25 and CD2

73
Q

Mast cell stains

A

Metachromatic - toluidine blue and giemsa
Leder - red

Subepi bulla

Solitary lesions improve in 3 years usually

74
Q

Neurofibroma stains

A

Cholinesterase activity positive

S100, vimentin, MBP positive

75
Q

GCT stains

A

Vimentin, NSE, S100, myelin protein, p75 nerve growth factor, calretinin, NKI/C3, PGP9.5

Mutant p53 find in half of malignant GCTs

76
Q

Cellular neurothekeoma

A

S100 neg

S100A6, PGP9.5, MITF, NK1C3

77
Q

Nasal glioma

A

Incompressible
Does not trans illuminate
Glial giant cells, bld v., fibrous tissue

78
Q

Psammoma bodies

A

Meningioma, IDN, schwannoma in carney, JXG

79
Q

Meningioma vs cephalocele

A

If brain remnants present then called a cephalocele

Hard, fibrous, calcified nodules along the spine, scalp, forehead or rarely over scalp

80
Q

Encephalocele and meningocele

A

Defects in neural tube
Midline face, scalp, neck, back
Soft compressible, enlarge with crying, transilluminate
10% have occult spinal dysraphism

81
Q

Chordomas

A

Slow growing locally invasive neoplasm
Firm smooth nodules in the sacrococcygeal region or base of skull in middle aged pts
Mets may occur
WLE

82
Q

Benign lipomas

A

May have 12q13-15 or 13q12-22 trans

83
Q

Frohlich syndrome

A

Multiple lipomas, obesity, sexual infantilism

84
Q

Neural fibrolipoma

A

Fibrofatty tissue along n. Trunk
Tender enlarging subq mass
Median n MC

85
Q

Chondroid lipoma

A

Deep seated firm yellow tumor on legs of F

S100 and vimentin positive

86
Q

Phosphatidylcholine injection

A

May be used for lipomas and Nevus lipomatosus

87
Q

Lipoblastoma

A

90% before age 3
Soft tissues of the upper and lower ext
8q11-q13 translocation or may see PLAG1-HAS fusion gene by Fish

88
Q

Eruptive leiomyomas described with

A

CLL

89
Q

Genital leiomyomas

A

May be seen on scrotum, labia majora, nipples

Associated with alports

90
Q

Angioleiomyoma

A

Arise from veins
Painful
Leg of middle aged women
If associated with AIDS cells may be EBV positive

91
Q

Smooth m. Hamartoma

A

Associated with adult myofibromas

Dermatology (28 decks)

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