Fibrosarcoma - Skin
Fibrosarcoma of soft tissues can go undetected for a long period of time, compared to fibrosarcoma of the bone, because of its painless character. Fibrosarcoma of soft tissues may occur in areas of prior burn scars or irradiation. Mutations in p53 have been identified in fibrosarcomas.
Fibrosarcoma of the bone may present with pain, swelling, and pathologic fractures. Fibrosarcoma of the bone may be related to bone trauma such as bone infarcts, bone dysplasia, Paget's disease, chronic osteomyelitis, and bone irradiation.
After excision, local recurrence rates approach 60% but are reduced to 25% when postoperative radiation is used. The 5-year survival rate for patients with soft tissue fibrosarcomas is 40% to 60% in adults and 80% for the infantile form.
Pediatric Patient Considerations:
Congenital infantile fibrosarcoma usually occurs in children less than 2 years of age and has a better prognosis than the adult form. The t(12;15)(p13;q25) chromosomal translocation helps distinguish this fibrosarcoma from other fibrous lesions of childhood.
C49.9 – Malignant neoplasm of connective and soft tissue, unspecified
254748009 – Cutaneous fibrosarcoma
- Nodular fasciitis – Rapidly growing, painful mass presenting especially on volar aspect of forearm.
- Myositis ossificans – Presence of metaplastic bone, many times following an episode of trauma.
- Malignant fibrous histiocytoma – Histology shows variable populations of tumor cells with spindling and epithelioid cells, tumor or benign giant cells, and inflammatory cells.
- Osteosarcoma – Produces tumor osteoid.
- Malignant neurosarcoma or malignant schwannoma – Malignancies of peripheral nerve sheaths.
- Desmoid tumors – Histology shows a relative increase in collagen and absence of cytologic atypia.
- Monophasic synovial sarcoma – Histology may show focal calcifications, gland or gland-like spaces.
- Neurofibrosarcoma – Arises from an identifiable nerve; apparent neurofibroma components may be present.
- Liposarcoma – Histology shows lipocytes in well-differentiated tumors, or lipoblasts if less well-differentiated.