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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.
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]
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 ]
A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal (connective tissue) origin. [1] [2] Connective tissue is a broad term that includes bone, cartilage, muscle, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues.
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.
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.
Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, [1] high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, [2] is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm (i.e., an abnormal growth of cells that have an unclear identity and/or cell of origin). [3]
A soft-tissue sarcoma (STS) is a malignant tumor, a type of cancer, that develops in soft tissue. [1] A soft-tissue sarcoma is often a painless mass that grows slowly over months or years. They may be superficial or deep-seated. Any such unexplained mass must be diagnosed by biopsy. [2]