Fibrous Tumors

Question 1

Factor XIIIa stain

Answer 1

DF, dermal dendritic cells, angiofibroma

Question 2

CD34 stain

Answer 2

dermal dendritic cells, DFSP, nephrogenic systemic fibrosis, vascular endothelium

Question 3

young girls with acrochordon midline anterior to anus

Answer 3

infantile perianal pyramidal protrusions

Question 4

genoderms with ‘skin tags’

Answer 4

basal cell nevus syndrome (PTCH)
birt hogg dube (folliculin)
cowden’s (PTEN)

Question 5

fibroma with plywood pattern

Answer 5

sclerotic fibroma

Question 6

fibrous tumor with triangular cells clinically mistaken for pyogenic granuloma on thighs

Answer 6

epitheloid cell histiocytoma

Question 7

syndromes with angiofibromas

Answer 7

tuberous sclerosis
birt-hogg-dube
MEN 1

Question 8

acral fibrokeratoma vs supernumerary digit

Answer 8

acral fibrokeratoma = perpendicular collagen

supernumerary digit = nerves, bone, cartilage

Question 9

painless nodule on digits of middle aged adults often slow growing around the nail
storiform with myxoid stroma

Answer 9

superficial acral fibromyxoma

CD34 and CD99 pos

Question 10

subungual exostosis

Answer 10

osteochondroma

Question 11

multiple DF associations

Answer 11

SLE, prednisone, immunosuppression, atopic derm, CML, lupus, HIV

Question 12

Ddx busy dermis

Answer 12

dermatofibroma
neurofibroma
granuloma annulare
scleromyxedema
dermal spitz nevus
blue nevus
kaposi's sarcoma
metastatic breast ca

Question 13

dupuytren contracture associations

Answer 13

alcoholic cirrhosis, DM, epilepsy, peyronie’s dz, knuckle pads (holoderma)

Question 14

genoderm with knuckle pads

Answer 14

bart-pumphrey

knuckle pads, PPK, hearing loss, leukonychia

Question 15

5 types of pachydermodactyly

Answer 15

1. classic
2. localized
3. transgrediens
4. familial
5. assoc w/ tuberous sclerosis

Question 16

MC fibrous tumor of infancy

prognosis?

Answer 16

infantile myofibromatosis
spontaneous resolution if limited to skin and bone
high mortality in first 4 months if visceral

Question 17

juvenile hyaline fibromatosis
mutation
4 findings

Answer 17

AR capillary morphogenesis protein 2
subcu nodules
pink papules of paranasal folds, preauricualr, perianal
gingival hypertrophy
joint contractures and osteolytic lesions