Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ossifying fibromyxoid tumour of soft parts (OFMT) is a recently described soft tissue tumour. Although rare it deserves recognition as a locally invasive but essentially benign neoplasm. Prior to its description in 1989 by Enzinger, ossifying fibromyxoid tumours of soft parts were classified as a wide variety of lesions, ranging from nodular fasciitis to osteosarcoma. We report a typical case of such a tumour and review current knowledge of this distinct neoplasm.
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PMID:Ossifying fibromyxoid tumour of soft parts. 930

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm which varies in the amount of bone tissue laid down. We report on a case of OFMT in a 59-year-old male which mimicked a large parosteal osteosarcoma on radiography, MRI, and CT. T1-weighted MR images showed high-intensity areas which reflected fatty marrow. The metaplastic bone was connected to the cortex of the femur, but the tumor did not involve the medullary cavity. The tumor was irradiated and the patient was administered chemotherapy. Wide excision of the tumor and hip rotation plasty was performed. The patient has been disease free for 18 months.
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PMID:Ossifying fibromyxoid tumor resembling parosteal osteosarcoma. 984 Mar 96

Ossifying fibromyxoid tumor (OFMT) is a soft tissue neoplasm of uncertain differentiation and intermediate (rarely metastasizing) biologic potential, with typical morphologic features, of an encapsulated, lobulated tumor comprising uniform polygonal cells within fibromyxoid stroma, which is surrounded by or contains metaplastic bone, classically as a peripheral rim of lamellar bone. Ossifying fibromyxoid tumor can arise at almost any site, although most frequently occurs within the extremities and trunk. Although most behave in a benign fashion, tumors can rarely show atypical or malignant features. It is now established that OFMTs represent translocation-associated tumors, with up to 85% associated with recurrent gene rearrangements, mostly involving the PHF1 gene (including in typical, atypical, and malignant neoplasms), with EP400-PHF1 in approximately 40% of tumors, and ZC3H7B-BCOR, MEAF6-PHF1, and EPC1-PHF1 fusions also described. Correct diagnosis is clinically important to ensure correct treatment and prognostication, both to avoid overdiagnosing OFMT as a malignant neoplasm such as osteosarcoma and also because of the propensity for aggressive behavior in a small number of OFMT. We review OFMT, with emphasis on the morphologic spectrum, recent molecular genetic findings, and the differential diagnosis.
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PMID:Ossifying fibromyxoid tumor: morphology, genetics, and differential diagnosis. 2673 2