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中枢神经系统孤立性纤维瘤:附5例报告并文献综合复习

Solitary fibrous tumor of the central nervous system: report of an additional 5 cases with comprehensive literature review.

作者信息

Bisceglia Michele, Dimitri Lucia, Giannatempo Giuseppe, Carotenuto Vincenzo, Bianco Mario, Monte Vincenzo, D'Angelo Vincenzo, Magro Gaetano

机构信息

IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.

出版信息

Int J Surg Pathol. 2011 Aug;19(4):476-86. doi: 10.1177/1066896911405655.

DOI:10.1177/1066896911405655
PMID:21878477
Abstract

Solitary fibrous tumor (SFT) of the central nervous system was first described in 1996. A number of cases have been reported since. The authors present 5 new cases: 4 intracranial and 1 intraspinal. All patients were adults (age range, 47 to 75 years); 4 were male and 1 female; 4 cases were primary tumors; and 1 was a second tumor recurrence. All patients were surgically treated with gross total removal. All cases were histologically examined with immunohistochemical confirmation; 2 tumors exhibited diffuse classic histology, 1 tumor was a cellular variant, 1 tumor was myxoid, and 1 was predominantly classic with focal myxoid features and focally pleomorphic. The postoperative course was uneventful in all. The patient with the cellular variant experienced 2 local recurrences and eventually died of disease 10 years after the initial diagnosis. The patient with the myxoid variant--the tumor studied--which was the second recurrence of a previously misdiagnosed fibrous meningioma surgically treated 15 years earlier, had a recurrence after 2 years for the third time and eventually died of disease. Three patients are alive and well 11.6, 6, and 4 years after surgery. SFT is a rare tumor that needs to be differentiated from some mimickers, mainly fibrous meningioma, hemangiopericytoma, and with regard to the myxoid variant, also adult-onset myxochordoid meningioma and myxoid peripheral nerve sheath tumor. Immunohistochemistry is crucial for the correct diagnosis of SFT. The authors also performed a review of the literature and found a little more than 200 cases on record.

摘要

中枢神经系统孤立性纤维瘤(SFT)于1996年首次被描述。此后已有多例病例报告。作者介绍了5例新病例:4例颅内肿瘤和1例脊髓内肿瘤。所有患者均为成年人(年龄范围47至75岁);4例为男性,1例为女性;4例为原发性肿瘤;1例为肿瘤复发。所有患者均接受了手术全切治疗。所有病例均进行了组织学检查及免疫组化确诊;2例肿瘤表现为弥漫性经典组织学,1例为细胞型变体,1例为黏液型,1例主要为经典型但有局灶性黏液样特征且局灶性具有多形性。所有患者术后病程均平稳。细胞型变体患者经历了2次局部复发,最终在初次诊断后10年死于该疾病。黏液型变体患者(即所研究的肿瘤)是15年前曾被误诊为纤维性脑膜瘤并接受手术治疗的肿瘤的第二次复发,术后2年第三次复发,最终死于该疾病。3例患者术后11.6年、6年和4年仍存活且状况良好。SFT是一种罕见肿瘤,需要与一些相似疾病相鉴别,主要包括纤维性脑膜瘤、血管外皮细胞瘤,对于黏液型变体,还需与成人型黏液性脊索样脑膜瘤和黏液型周围神经鞘瘤相鉴别。免疫组化对于SFT的正确诊断至关重要。作者还对文献进行了回顾,发现记录在案的病例略多于200例。

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