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Clinical Sarcoma Research
Published in: Clinical Sarcoma Research 1/2017

Open Access 01-12-2017 | Case report

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

Authors: 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

Published in: Clinical Sarcoma Research | Issue 1/2017

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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.
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Metadata
Title
Response to isolated limb perfusion and chemotherapy with epirubicin plus ifosfamide in a metastatic malignant ossifying fibromyxoid tumor
Authors
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
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Clinical Sarcoma Research / Issue 1/2017
Electronic ISSN: 2045-3329
DOI
https://doi.org/10.1186/s13569-017-0086-2

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