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01.12.2017 | Case report | Ausgabe 1/2017 Open Access

Clinical Sarcoma Research 1/2017

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

Clinical Sarcoma Research > Ausgabe 1/2017
Salvatore Provenzano, Alessandra Raimondi, Rossella M. Bertulli, Vittoria Colia, Salvatore L. Renne, Paola Collini, Gianpaolo Dagrada, Dario Callegaro, Marco Fiore, Francesca G. Greco, Paolo G. Casali
Wichtige Hinweise
Paolo G. Casali is Editor-in-Chief of Clinical Sarcoma Research



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.


To our knowledge, this is the first report of activity of soft tissue sarcoma-oriented chemotherapy in advanced OFMT.
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