Term
| Soft-Tissue Tumor Syndromes |
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Definition
| Kaposi, neurofibromatosis I, neurofibroma, malignant peripheral nerve sheath tumor, gardner syndrome, Li-Fraumeni, Osler-Weber-Rendu |
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Term
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Definition
| common. (conventional:12q14-q15, 6p, 13q), (spindle cell/pleomorphic: 16q, 13q) Angiolipomas are painful. |
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Term
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Definition
| 40-60s, deep tissues and retroperitoneum. (Well-differentiated, myxoid/round cell, and pleomorphic) 12q14-15 amplification of MDM2 which inhibits p53. Lipoblasts mimic fetal fat cells. Pleomorphic variant is worst. |
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Term
| Reactive Pseudosarcomatous Proliferations |
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Definition
| Nodular Fasciitis, Myositis Ossificans |
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Term
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Definition
| also infiltrative or pseudosacromatous faciitis. Volar aspect of the forearm. Immature fibroblasts |
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Term
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Definition
| Metaplastic bone in proximal extremity muscles often following trauma. Ill-defined trabeculae, peripherally well-formed. Patchy flocculent radiodensities. Zonation is vital: Intermediate with osteoblasts |
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Term
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Definition
| (Palmar is Dupuytren's contracture, Plantar, Penile is Peyronie disease) |
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Term
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Definition
| puckering and dimpling. Slowly progressive flexion of the fourth and fifth fingers |
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Term
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Definition
| Also deep-seated fibromatosis. (Extra-abdominal in musculature, Abdominal after pregnancy, Intra-abdominal in mesentery with Gardner) Genes: APC or beta-catenin. Rubbery, tough, banal fibroblasts. Treatment with tamoxifen, chemotherapy, radiation. |
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Term
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Definition
| Unencapsulated, infiltrative, soft, fish-flesh with hemorrhage and necrosis. |
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Term
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Definition
| before 20, head/neck/genitourinary tract. (Embryonal, Alveolar, Peomorphic) Botryiod has best prognosis, anaplastic embryonal/pleomorphic/alveolar are often fatal. |
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Term
| Embryonal Rhabdomyosarcoma |
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Definition
| Most common. (Sarcoma botryoides, spindle cells, anaplastic) <10yr. isodisomy of chromosome 11p15.5 with IFGII gene. Camdium later. Cross-striations. |
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Term
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Definition
| Submucosal zone of hypercellularity in Embryonal rhabdomyosarcoma |
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Term
| Alveolar rhabdomyosarcoma |
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Definition
| middle adolescence, deep extremity musculature. Centers of aggregates are dyscohesive. Fusion: FOXO1a on 13q14 with PAX3 on 2q35 or PAX7 on 1q36. PAX3 is worse |
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Term
| Pleomorphic Rhabdomyosarcoma |
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Definition
| large multinucleated bizarre eosinophilic cells. deep soft tissue of adults. |
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Term
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Definition
| Uterus or arrector pili muscles (pilar leiomyomas). Genes: fumarate hydratase on 1q42.3. Blunt ended, elongated nuclei |
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Term
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Definition
| adult women in extremities and retroperitoneum. Bundles of thin filaments. Stain for actin and desmin |
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Term
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Definition
| dual lines of differentiation (epithelial-like or mesenchymal-like). Keratin and epithelial membrane antigen. t(x;10)(p11;q11) producing SS10-SSX1/2/4 |
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