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Synovial Sarcoma of Peripheral Nerves: Analysis of 15 Cases.

发生于外周神经的滑膜肉瘤:15例病例的分析

Chrisinger JSA,Salem UI,Kindblom LG,Amini B,Hansson M,Meis JM

Abstract

Synovial sarcoma of peripheral nerve (SSPN) is rare with only 26 cases reported in English. SSPN is often mistaken for a benign or malignant peripheral nerve sheath tumor (PNST) by clinicians and pathologists. Fifteen cases of SSPN were retrieved from the pathology files of 3 institutions. All tumors arose in a nerve based on imaging and/or operative findings and the diagnoses were histologically confirmed. Neuropathic symptoms predominated in 11 women and 4 men, 19- to 62-year-old (median, 39 years) with tumors involving the ulnar (5), median (3), peroneal (3) or sciatic (2) nerve, or the L4 or T1 nerve root (2). Tumor sizes ranged from 2 to 13 cm (median, 3.8 cm). The leading clinical diagnosis was PNST (9). Treatment was surgical (14) supplemented with radiation therapy (8) and chemotherapy (6). Fourteen tumors were monophasic and 1 was biphasic; 4 had poorly differentiated (PD) foci (1 rhabdoid). Diagnoses in 12 cases were verified by fluorescence in situ hybridization, reverse transcription polymerase chain reaction or both methods. Follow-up in 14 patients (median, 32 mo) revealed that 2/4 patients with PD tumors died with pulmonary metastases; another was alive with no current evidence of disease (NED) following 2 local recurrences, while the fourth had NED. In contrast, 9/10 patients without PD tumors were alive (7 NED) and 1 died at 12 months with pulmonary infiltrates. SSPN is under-recognized clinically and histologically as it mimics benign and malignant PNST. Molecular analysis is recommended to confirm the diagnosis. PD foci, including rhabdoid areas, may portend a worse outcome, similar to non-neural-based tumors.

摘要

发生于外周神经的滑膜肉瘤(SSPN)非常罕见,且英文文献中仅报道过26例。SSPN经常被临床医生和病理医生误诊为良性或是恶性外周神经鞘膜瘤(PNST)。从三个病理机构收集15例SSPN病例。所有肿瘤基于影像学检查或/和术中发现均可证实都起源于神经,并且组织学诊断明确。11名女性和4名男性患者均具有明显的神经系统症状,年龄范围为19-62岁(中位年龄为39岁),肿瘤分别发生于尺神经(5例)、正中神经(3例)、腓神经(3例)和坐骨神经(2例),以及L4或T1的神经根(2例)。肿瘤大小2-13cm不等(中位值3.8cm)。9例病例的临床首先考虑诊断为PNST。进行过手术治疗的病例有14例,且有8例后续辅以放射性治疗,另外6例辅以化疗。14例肿瘤为单相型滑膜肉瘤,另1例为双相型滑膜肉瘤;4例具有低分化(PD)病灶(1例为横纹肌)。12例病例的诊断经荧光原位杂交或逆转录聚合酶链反应或是两种方法均操作而得以证实。14例患者的预后(中位生存期为32个月)显示4例具有PD的肿瘤患者中有2例死于肺转移,另外1例患者在两处局部复发后、目前无疾病证据(NED)仍存活,但是第4例有NED;相反,另外没有PD的10例患者中有9例仍存活(7例NED),1例在12个月后肺受累死亡。SSPN在临床和组织学上经常认识不足,因为它与良性和恶性的PNST具有相似性。分子分析有助于明确诊断。PD病灶、包括横纹肌区域,可能与非神经系统来源肿瘤一样,意味着预后更差。












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