Advanced malignant solitary fibrous tumor in pelvis responding to radiation therapy
Advanced malignant solitary fibrous tumor in pelvis responding to radiation therapy
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DOI：
10.1111/j.1440-1827.2007.02083.x
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发表时间：
2007-04-01
影响因子：
2.2
通讯作者：
Fukuoka, Junya
中科院分区：
文献类型：
--
作者：
Kawamura, Shinobu;Nakamura, Takafumi;Fukuoka, Junya
孤立性纤维瘤是一种罕见的梭形细胞肿瘤，大多数情况下是良性的。虽然SFT最初被认为只发生在胸膜，但最近的报告表明，SFT可以涉及广泛的解剖部位。迄今为止，已报告了17例骨盆SFT。本文报告一位74岁女性，在骨盆部有一个巨大的恶性SFT。沿着放射学检查发现的对邻近器官的大量侵犯和多发性肺转移，肿瘤经阴道壁的活检显示恶性梭形细胞肿瘤，细胞结构增加，坏死区域和高有丝分裂活性（5/10高倍视野）。免疫组化显示，肿瘤细胞呈弥漫性强阳性表达CD 34、CD 99和bcl-2。结合病理及临床表现，诊断为恶性SFT。患者接受全身和动脉内化疗，随后进行全盆腔放射治疗（50戈伊）。最初的化疗未能控制肿瘤。在放射治疗后的12个月随访中，放射学和病理学观察到改善。这是第一个报告有关治疗意见的晚期恶性SFT。
Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that is benign in most cases. Although SFT was first recognized to arise only in the pleura, recent reports indicate that SFT can involve a wide range of anatomical sites. To date, 17 cases of pelvic SFT have been reported. Herein is reported a case of a 74-year-old woman with a giant malignant SFT in the pelvis. Along with massive invasion to adjacent organs and multiple lung metastases detected on radiography, biopsy from the tumor through the vaginal wall showed malignant looking spindle-cell neoplasm with increased cellularity, areas of necrosis, and high mitotic activity (5/10 high-power fields). Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34, CD99, and bcl-2. Based on pathological features and clinical presentation, diagnosis of malignant SFT was made. The patient received systemic and the intra-arterial chemotherapy followed by whole pelvic radiation therapy (50 Gy). Initial chemotherapies failed to control the tumor. Afterwards, improvement was observed radiologically and pathologically in the 12 months' follow up after the radiation therapy. This is the first report related to therapeutic remarks on advanced malignant SFT.