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Flashcards in Dermal And Subcutaneous Tumors Deck (91):
1

Hamartoma

Abn arrangement of tissues normally present ata given site

Nevus - nl arrangement

2

Revised phakomoatosis pigmentovascularis

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

3

Phakomatosis pigmentovascularis associated findings

Intracranial and visceral vascular anomalies
Ocular
Choroidal melanoma
Hemihypertrophy

4

MC phako pigmentovascularis

Type II

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

5

Type III phako association

Multiple GCT

6

Eccrine angiomatous hamartoma

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

7

Nevus anemicus

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

8

Nevus oligemicus

Livid skin patch cooler than normal
Due to vasoconstriction

9

CMTC common anomalies

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

10

Bockenheimer syndrome

Progressive venous ecstasias of one limb

11

Reports of this occurring in PWS

BCC

12

SWS

Fibronectin gene expression incr in lesional fibroblasts

13

Roberts syndrome

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

14

Wyburn mason syndrome

UL retinal AVM with IL PWS near ear

TAR - also has PWS

15

Coats disease

Retinal telangectasias with IL PWS

16

PWS

Increased VEGF and its receptor VEGFR2

17

Gorhams disease

Cutaneous and osseous venous and lymphatic malf with osteolysis

18

Novel sclero agent for VM

Ethanolamine
Soft tissue injury and neuropathy found

19

Earliest and MC presentation of KT

PWS of extremity

VG5Q mutation associated with KTS

Ulcers may respond to sunitinib

20

Cirsoid aneurysms

Congenital AV fistulas of scalp and face

21

MC in lymphangiomatosis

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

22

Gorham stout

Lymphangiomatosis, chylous effusions, esteolytic bones

Response to peg-IFN reported

23

Lupus vs scleroderma nailfold changes

Tortuous glomeruloid loops in lupus
Dilated loops and avascular in scleroderma

24

GET vs telangectasias of systemic dz

GET vessels do not have alk phos activity

25

Unilateral nevoid telangectasias

MC involve trigeminal, C3 and C4, R>L

26

Acral pseudolymphomatous angiokeratomas in children (apache)

UL and sporadic
No cold sensitivity
Variant of pseudolymphoma

27

Kimuras disease

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

28

IPEH

Head, neck or upper ext

29

Angioma serpiginosum

No alk phos vs normal capillaries

30

MC vascular anomaly

Cherry angiomas

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

31

Targetoid hemosiderotic hemangioma

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

32

Glomeruloid hemangioma

Poems, castlemans, ITP, sjogrens

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

33

Microvenular hemangioma

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

34

TA vs KHE vs IH

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

35

Angioblastoma

Rare pediatric tumor associated with destruction of regional structures

Elevated basic fibroblast growth factor

36

KHE

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

37

MLT

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

38

Avg age of kassabach Merritt

7 weeks
Most deaths due to bleeding complication, 30% mortality

39

Glomus cells

Modified smooth muscle
Stain with vimentin (not desmin), SMA often positive

40

Adult hemangiopericytoma

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

41

Proliferating angioendotheliomatosis

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

Hemangioendothelioma

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

43

MC location for classic KS

Toes or soles

AIDS associated predilection for head, neck, trunk and MM

44

MC site of internal involvement for KS

GI, especially SI

Bone inv indicates widespread dz

African KS frequently has bone inv and lymphedema

45

MC location for AIDS KS

GI, LN and lungs
25% have only cutaneous inv

46

Increased risk of malignancies in KS

HL, T cell lymphoma, NHL
20 x incr risk

47

First line for advanced KS

Liposomal anthracyclines

Lesions are radiosensitive

48

Epitheliod hemangioendothelioma

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

49

Retiform hemangioendothelioma

Low grade angiosarcoma
Upper or LE of young adults
hHV8 reported

50

Endovascular papillary angioendothelioma

Dabska tumor
Low grade angiosarcoma on head neck of infants/children

51

Angiosarcoma

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

MC location for keloids

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

Dupuytrens

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

Knuckle pads (heloderma)

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

55

Pachydermodactyly

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

56

Desmond tumor

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

57

Collagenous fibroma (desmoblastic fibroma)

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

Infantile myofibromatosis

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

Pseudo ainhum

Vohinkel, mal de meleda, PC
ED
EPP

Use pseudo when associated with hereditary conditions

60

Familial collagenomas

Associated with ASD

Eruptive collagenomas in syphylis

61

Cellular angiofibroma

Genitals of older F
Express E and progesterone R
CD34

62

Superficial acral fibromyxoma

Cd34, cd99, EMA

63

Oral submucous fibrosis

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

64

Cutaneous pseudosarcomatous polyp and umbilical polyp

Benign skin tag like proliferations
Dramatic atypia
Pos vimentin

65

Tag like BCC

Should suggest gorlins

66

Dermal dendrocyte hamartoma

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

67

Nodular fasciitis

MC arms

68

Solitary fibrous tumor

Mediastinum
CD34 and progesterone R

69

Plexiform fibrohistiocytic tumor

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

70

DFSP

CD34, stromeolysin-3 and nesting positive vs DF

71

MC type of mastocytosis

UP

72

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

Systemic masticytosis
MC express CD25 and CD2

73

Mast cell stains

Metachromatic - toluidine blue and giemsa
Leder - red

Subepi bulla

Solitary lesions improve in 3 years usually

74

Neurofibroma stains

Cholinesterase activity positive
S100, vimentin, MBP positive

75

GCT stains

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

Mutant p53 find in half of malignant GCTs

76

Cellular neurothekeoma

S100 neg
S100A6, PGP9.5, MITF, NK1C3

77

Nasal glioma

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

78

Psammoma bodies

Meningioma, IDN, schwannoma in carney, JXG

79

Meningioma vs cephalocele

If brain remnants present then called a cephalocele
Hard, fibrous, calcified nodules along the spine, scalp, forehead or rarely over scalp

80

Encephalocele and meningocele

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

81

Chordomas

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

Benign lipomas

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

83

Frohlich syndrome

Multiple lipomas, obesity, sexual infantilism

84

Neural fibrolipoma

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

85

Chondroid lipoma

Deep seated firm yellow tumor on legs of F
S100 and vimentin positive

86

Phosphatidylcholine injection

May be used for lipomas and Nevus lipomatosus

87

Lipoblastoma

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

Eruptive leiomyomas described with

CLL

89

Genital leiomyomas

May be seen on scrotum, labia majora, nipples
Associated with alports

90

Angioleiomyoma

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

91

Smooth m. Hamartoma

Associated with adult myofibromas