Fibrohistiocytic neoplasm

Question 1

write short notes on dermatofibroma

Answer 1

Question 2

write short notes on dermatofibrosarcoma protuberans

Answer 2

Question 3

Write short notes on atypical fibroxanthoma (AFX)

Answer 3

Laaste punt: UPS has similar ugly cell types as AFX, but is a deep sarcoma that arises jn deep soft tissues (thigh #1) of middle-aged adults . 5 year mortality = 50%

Question 4

Write notes on angiofibroma

Answer 4

Question 5

write short notes on sclerotic fibroma

Answer 5

Question 6

pleomorphic fibroma

Answer 6

Question 7

multinucleate cell angiohistiocytoma

Answer 7

1.Clinical features
- multiple, grouped red papules on dorsal hands or legs of women in their 40s
2. Histologic features - looks like a ‘cell poor DF’ with characteristic multinucleated giant cells and prominent proliferation of dilated vessels in dermis, stain like DF (factor13a+ and S100 negative)

Question 8

Epithelioid cell histiocytoma (epithelial fibrous histiocytoma)

Answer 8

Question 9

Acral fibrokeratoma

Answer 9

Question 10

dermatomyofibroma

Answer 10

Question 11

Inclusion body fibromatosis (infantile digital fibroma)

Answer 11

Question 12

Fibromatosis

Answer 12

Question 13

nodular fasciitis

Answer 13

Question 14

Fibrous hamartoma of infancy

Answer 14

Question 15

myofibroma (infantile myofibromatosis)

Answer 15

Question 16

Giant cell tumour of tendon sheats (tenosynovial giant cell tumor)

Answer 16

Question 17

connective tissue nevus (collagenoma and elastoma)

Answer 17

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Question 18

Write notes on skin tags

Answer 18

Question 19

Write short notes on cutaneous angiofibroma

Answer 19

1)benign vascular neoplasm composed of dermal fibrous tissue and blood vessels
2) classified acc to associated genetic conditions or body site
3) A/w genetic disorders:
Tuberous sclerosis
Birt-Hogg-Dubé syndrome
Multiple endocrine neoplasia type 1 (MEN-1).
4) Angiofibromas can also be acquired and unrelated to a genetic syndrome, commonly in the form of Fibrous papule of the nose/face
Pearly penile papules.
fibrous papule is a solitary lesion usually on the nose, often clinically mistaken for a basal cell carcinoma
5) An angiofibroma is a firm, flesh-coloured dome-shaped papule less than 5 mm in diameter. Small capillaries may be visible on the surface of the lesion.
6) Facial angiomas associated with a genetic syndrome are commonly found in clusters in the butterfly region of the face.
7) The histopathology of angiofibroma shows an ‘onion skin’ pattern around vessels and follicles, hyperkeratosis, and vascular proliferation

Question 20

Pathology of skin tags

Answer 20

Question 21

Superficial acral fibromyxoma

Answer 21

1) benign fibromyxoid lesions.
2) small isolated lesions with a predilection for the fingers, toes, and nails.
3) In superficial acral fibromyxoma, sections show a paucicellular fibromyxoid lesion arising in the dermis of acral skin Subcutaneous involvement is common. The cells are bland, spindled or stellate and set in a fibromyxoid stroma
4) Immunohistochemical studies positivity with CD34, EMA. S100, cytokeratins and smooth muscle markers are negative.

Question 22

Cutaneous adult myofibroma

Answer 22

rare, benign skin tumor that is the adult (can be seen in older kids too) counterpart of infantile myofibromatosis.
It typically presents as a solitary, painless, slow-growing nodule, usually on the head, neck, trunk, or extremities.
Key characteristics of cutaneous adult myofibroma:
Appearance: Firm, flesh-colored to purplish nodules, sometimes with a bluish hue.
Location: Most commonly found on the head and neck, but can also occur on the trunk or extremities.
Growth: Slow-growing, typically asymptomatic.
Histology: Characterized by spindle-shaped cells arranged around small blood vessels, a biphasic pattern with both spindle and rounded cells, and primitive vascular formations.

Question 23

Pictures of
-skin tags
-fibrous papule of the nose
-pearly penile papules

Answer 23

Question 24

picture of deematofibroma

Answer 24

Question 25

name the clinical and histological variants of dermatofibroma

Answer 25

Question 26

Tabulate and compare histological variants of dermatofibroma

Answer 26

Question 27

picture of DFSP

Answer 27

Question 28

collagenoma picture

Answer 28

Question 29

picture of acral fibrokeratoma

Answer 29

Question 30

picture if multinucleate cell angiohistiocytoma

Answer 30

Question 31

picture of giant cell tumor of tendon sheath

Answer 31

Question 32

picture of comnective tissue nevus

Answer 32

Question 33

picture of infantile digital fibroma and osteopoikilosis

Answer 33

Question 34

Picture of infantile myofibromatosis

Answer 34

Question 35

picture of knuckle pads

Answer 35

Question 36

picture of pachydermadactaly

Answer 36

Question 37

picture of atypical fibroxanthoma

Answer 37

Question 38

picture of DFSP

Answer 38

Question 39

Classification of fibrosarcoma

Answer 39

Question 40

Differentiating dermatofibroma from dermatofibrosarcoma protruberans

Answer 40

Question 41

Plexiform fibrohistiocytic tumor

Answer 41

1. slow growing, firm, painless mass in a child/young adult 2. Usually <3cm and favor wrists and hands 3. Regarded as being intermediate malignancy, with a tendency to recur but only rarely metastazising 4. in dermis and subcut, muktinodular mass is composed of nodules of histiocytes and fasicles of spindled cells intersecting the stroma in a plexiform pattern. 5. Multinucleated giant cells are frequently present. Mitotic features and mild nuclear pleomorphism can be seen. - growing evidence to suggest cellular neurothekeoma on spectrum 6. Locally aggressive Excise with wide margins and long term evaluation

Question 42

Giant cell fibroblastoma

Answer 42

1. Rare, locally aggressive, histologic variant of DFSP 2. Solitary, slow growing, asymptomatic skin coloured nodule involving the neck, trunk or groin 3. 50% have protuberant/polypoid shape 4. Occur in early childhood with strong predisposition for boys 5. Pathology - diffuse infiltrative sheets of spindle shaped fibroblast and multi-nucleated cells with hyperchromatic nuclei within myxoid matrix Irregular ‘angiectoid’ or pseudovascular spaces lined by multinucleated giant cells. These giant cells dont react with endothelial markers BUT are positive for CD34 antibodies. Shares sam chromosomal translocation as DFSP t(17;22) Low grade sarcoma - can occasionally recur/metastasize

Question 43

Fribrosarcoma

Answer 43

1. Fibrosarcoma are a heterogenous group of high-grade and low-grade soft tissue tumors 2. Classic form - affect young to middle aged adults 3. Most common location is lower extremities then upper extremities, trunk and head and neck region 4. Usually deep soft tissue with secondary involvement of subcutis and skin 5. Slow growing from 1-10cm 6. Palpable painful nodule 7. can appear in old burn scars ir prior radiation site 8. Pathology - prolif of atypical spind-shaped cells arranged as intersecting fascles in ‘herringbone’ pattern. There are thin collagen bundles between the cells that have scanty cytoplasm and elongated hyperchromatic nuclei. Mitotic figured. High-grade fibrosarcoma are characterized bt increased cellularity with more atypical nuclei, higher mitotic rate, foci of necrosis and less distinct herringbone’ pattern. Rx = wide local excision, if unable to het clear margins adjuvant RT/amputation

Question 44

Epithelioid Sarcoma

Answer 44

1) 2 clinicopathologic subtypes - the conventional/classic (‘distal form’) characterized by proclivity for acral sites and pseudogranulomatous growth pattern -the proximal-type (‘large cell’) variant that arises more proximally on trunk ans composed of nests/sheets of large epithelioid cells 2) classic epithelioid sarcomas present as slow growing, firm, subcutaneous nodule of distal extremities, usually the hands/fingers 3) may be ulceration if involved dermis 4) advanced disease - linear arrangement of widely spaced, ulcerated nodules extend from distal to proximal sites on affected extremities with intervening tumor-free skin. If large nerve involved - a/w pain/parasthesia 5)pathology: nodular proliferation of spindle shaped to round/polygonal cells with abundant eosinophilic cytoplasm and small uniform nulcei without prominent nucleoli frequently a/w fascia, periosteum, tendons and nerves. Larger tumor nodules -> zones of central necrosis surrounded by palisaded tumor cells and a mixed chronic inflamm infiltrate. IHC: Tumor cells positive for both keratins (‘Epithelioid’) and vimentin (‘sarcoma’) They are also positive for epithelial membrane antigen and CD34z. negative for S100, HMB 45, CD31 and INI1