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Dermal And Subcutaneous Tumors 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
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

Dermatology (28 decks)

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