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Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcomas mainly arise in people between the ages of 25 and 79. [1]
Malignant FMTs Solitary fibrous tumor, malignant type , a malignant form of the solitary fibrous tumors [ 37 ] Fibrosarcoma NOS , i.e. fibrosarcoma, not otherwise specified, or, alternatively, adult fibrosarcoma to distinguish it from rarely metastasizing infantile fibrosarcoma.
Currently, however, inflammatory pseudotumor designates a large and heterogeneous group of soft tissue tumors that includes inflammatory myofibroblastic tumor, plasma cell granuloma, xanthomatous pseudotumor, solitary mast cell granuloma, inflammatory fibrosarcoma, [4] pseudosarcomatous myofibroblastic proliferation, myofibroblastoma ...
Infantile myofibromatosis (IMF) is a rare tumor found in 1 in 150,000 to 1 in 400,000 live births. It is nonetheless the most common tumor derived from fibrous connective tissue that occurs primarily in infants and young children.
They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive and invasive, with spindle-like growths. The tumors can lead to pain, life-threatening problems, or, rarely, death when they invade other soft tissue or compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves.
A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma . About half the cases are diagnosed in people with neurofibromatosis ; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. [ 2 ]
Treatment options for soft-tissue sarcomas include surgery, radiotherapy, chemotherapy, and targeted drug therapy. [3] Surgery is the most common treatment for soft-tissue sarcomas, and usually the only way to achieve a cure. The tumor is removed leaving a safe margin of surrounding healthy tissue to decrease the chances of its recurrence.
Dermatofibrosarcoma protuberans (DFSP) [2] is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis). [3] Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year.