mesna and Fibrosarcoma

Topics: Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Cyclophosphamide; Dactinomycin; Disease-Free Survival; Doxorubicin; Etoposide; Female; Fibrosarcoma; Humans; Ifosfamide; Infant; Infant, Newborn; Male; Mesna; Soft Tissue Neoplasms; Vincristine

The role of chemotherapy in the treatment of myxofibrosarcoma is unclear. There are no randomized clinical trials evaluating the therapeutic effect of chemotherapy on myxofibrosarcoma. We report, to the best of our knowledge, the first case of myxofibrosarcoma successfully treated with mesna, pirarubicin, ifosfamide and dacarbazine (modified MAID) regimen. The patient achieved complete remission evaluated according to Response Evaluation Criteria in Solid Tumours (RECIST).

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Dacarbazine; Doxorubicin; Fibrosarcoma; Humans; Ifosfamide; Lung Neoplasms; Male; Mesna

Myxoinflammatory fibroblastic sarcoma is a soft-tissue neoplasm most frequently found in the distal extremities of middle-aged adults. Most myxoinflammatory fibroblastic sarcoma are low-grade tumors with propensity for local recurrence after incomplete removal. We report a myxoinflammatory fibroblastic sarcoma which developed in the foot of a 41-year-old male and showed an exceptionally aggressive course with metastatic spread and fatal outcome within 16 months. We managed to establish a spontaneously transformed continuous cell line, called JU-PI, from a metastatic lesion. The JU-PI cells have a sub-tetraploid karyotype including the 1;10 chromosomal translocation and amplification of the proximal end of 3p; these features are considered genetic signatures of myxoinflammatory fibroblastic sarcoma. Both the primary tumor and the JU-PI cells showed nuclear expression of the TFE3 transcription factor but TFE3-activating chromosomal rearrangements were not found. To our knowledge, JU-PI is the first established myxoinflammatory fibroblastic sarcoma cell line. JU-PI cells offer a tool for investigating the molecular oncology of myxoinflammatory fibroblastic sarcoma.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Cell Line, Transformed; Cell Proliferation; Dacarbazine; Doxorubicin; Fibrosarcoma; Humans; Ifosfamide; Karyotype; Male; Mesna; Neoplasm Recurrence, Local; Primary Cell Culture; Soft Tissue Neoplasms; Translocation, Genetic; Vincristine

Topics: Adult; Angiogenesis Inhibitors; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Antineoplastic Combined Chemotherapy Protocols; Bevacizumab; Bowman Capsule; Dacarbazine; Deoxycytidine; Disease Progression; Docetaxel; Doxorubicin; Endothelium, Vascular; Fibrosarcoma; Gemcitabine; Glomerulonephritis, Membranoproliferative; Humans; Ifosfamide; Lung Neoplasms; Male; Mesna; Muscle Neoplasms; Podocytes; Proteinuria; Taxoids; Vascular Endothelial Growth Factor A