Background: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain lineage and intermediate biological potential. It is more common in middle-aged men, usually arising from the deep tissues of the extremities. It is now established that it is a translocation related tumor, most often marked by translocation of PHF1 gene. Surgery is the mainstay of treatment and proves usually curative, although, in rarer cases the disease shows malignant features and tendency to recur both locally and at distant sites. In such cases, no standard treatment exists. Case presentation: We report on a case of malignant advanced OFMT of the hand with lung metastases responding to isolated limb perfusion with human recombinant tumor necrosis factor and melphalan and chemotherapy with epirubicin and ifosfamide. Conclusions: To our knowledge, this is the first report of activity of soft tissue sarcoma-oriented chemotherapy in advanced OFMT.

Response to isolated limb perfusion and chemotherapy with epirubicin plus ifosfamide in a metastatic malignant ossifying fibromyxoid tumor / S. Provenzano, A. Raimondi, R.M. Bertulli, V. Colia, S.L. Renne, P. Collini, G. Dagrada, D. Callegaro, M. Fiore, F.G. Greco, P.G. Casali. - In: CLINICAL SARCOMA RESEARCH. - ISSN 2045-3329. - 7:1(2017), pp. 20.1-20.6. [10.1186/s13569-017-0086-2]

Response to isolated limb perfusion and chemotherapy with epirubicin plus ifosfamide in a metastatic malignant ossifying fibromyxoid tumor

S. Provenzano;A. Raimondi;V. Colia;S. L. Renne;D. Callegaro;M. Fiore;P. G. Casali
2017

Abstract

Background: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain lineage and intermediate biological potential. It is more common in middle-aged men, usually arising from the deep tissues of the extremities. It is now established that it is a translocation related tumor, most often marked by translocation of PHF1 gene. Surgery is the mainstay of treatment and proves usually curative, although, in rarer cases the disease shows malignant features and tendency to recur both locally and at distant sites. In such cases, no standard treatment exists. Case presentation: We report on a case of malignant advanced OFMT of the hand with lung metastases responding to isolated limb perfusion with human recombinant tumor necrosis factor and melphalan and chemotherapy with epirubicin and ifosfamide. Conclusions: To our knowledge, this is the first report of activity of soft tissue sarcoma-oriented chemotherapy in advanced OFMT.
Chemotherapy; Epirubicin; Ifosfamide; Isolated limb perfusion; Ossifying fibromyxoid tumor; Soft tissue sarcoma
Settore MED/06 - Oncologia Medica
CLINICAL SARCOMA RESEARCH
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/555587
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