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第100例硬化性上皮样纤维肉瘤(SEF)。

The hundredth case of sclerosing epithelioid fibrosarcoma (SEF).

作者信息

Monarca Cristiano, Fino Pasquale, Rizzo Maria Ida, Palmieri Annapina, Tarallo Mauro, Scuderi Nicolò

出版信息

Ann Ital Chir. 2013 May-Jun;84(3):315-8.

Abstract

Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare and distinct variant of low grade fibrosarcoma, found mainly in deep soft tissue of adult extremities. We report a case of Sclerosing Epithelioid Fibrosarcoma of soft tissue, which developed in a 69-year-old woman who presented a tumor involving the fourth finger of her right foot and which has not progressed much in size during the three months prior the surgical excision. Our patient is the hundredth case of Sclerosing Epithelioid Fibrosarcoma reported in literature since 1995, thus confirming the rarity of the tumor. Our experience showed that it is important to make an early diagnosis, in consideration of the clinical aggressiveness of this cancer. Another important aspect concerns the postoperative follow-up. The monitoring of PET-CT technique , although not standardized, could become part of proceedings of therapy and follow-up of tumor, thus allowing oncological radicality and avoid large amputations. To date, 24 months after diagnosis of SEF, our patient feels well, attends our outpatient clinic regularly and shows no evidence of relapse and/or metastasis.

摘要

硬化性上皮样纤维肉瘤(SEF)是一种罕见且独特的低级别纤维肉瘤变体,主要见于成人四肢的深部软组织。我们报告一例软组织硬化性上皮样纤维肉瘤病例,该病例发生在一名69岁女性身上,她的右脚第四指出现肿瘤,在手术切除前三个月内肿瘤大小没有明显进展。我们的患者是自1995年以来文献报道的第100例硬化性上皮样纤维肉瘤病例,从而证实了该肿瘤的罕见性。我们的经验表明,鉴于这种癌症的临床侵袭性,早期诊断很重要。另一个重要方面涉及术后随访。PET-CT技术的监测虽然尚未标准化,但可成为肿瘤治疗和随访程序的一部分,从而实现肿瘤根治并避免大截肢。迄今为止,在诊断SEF 24个月后,我们的患者感觉良好,定期到我们的门诊就诊,没有复发和/或转移的迹象。

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