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

Clinical Sarcoma Research Pub Date : 2017-12-28 eCollection Date: 2017-01-01 DOI:10.1186/s13569-017-0086-2

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

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引用次数: 6

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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转移性恶性骨化纤维黏液样瘤对孤立肢体灌注和表柔比星加异环磷酰胺化疗的反应。

背景:骨化性纤维黏液样瘤(OFMT)是一种罕见的软组织肿瘤，谱系不确定，生物学潜力中等。它在中年男性中更为常见，通常起源于四肢的深层组织。现在已经确定它是一种易位相关的肿瘤，最常见的标志是PHF1基因易位。手术是治疗的主要方法，通常可以治愈，但在少数病例中，该疾病表现出恶性特征，并有局部和远处复发的倾向。在这种情况下，没有标准的治疗方法。病例报告:我们报告了一例恶性晚期手部OFMT伴肺转移的病例，对分离肢体灌注人重组肿瘤坏死因子和美法兰以及表柔比星和异环磷酰胺化疗有反应。结论:据我们所知，这是第一次报道软组织肉瘤导向化疗在晚期OFMT中的活性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Clinical Sarcoma Research

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期刊介绍： Clinical Sarcoma Research considers for publication articles related to research on sarcomas, including both soft tissue and bone. The journal publishes original articles and review articles on the diagnosis and treatment of sarcomas along with new insights in sarcoma research, which may be of immediate or future interest for diagnosis and treatment. The journal also considers negative results, especially those from studies on new agents, as it is vital for the medical community to learn whether new agents have been proven effective or ineffective within subtypes of sarcomas. The journal also aims to offer a forum for active discussion on topics of major interest for the sarcoma community, which may be related to both research results and methodological topics.