Michal Michal, Fanburg-Smith Julie C, Lasota Jerzy, Fetsch John F, Lichy Jack, Miettinen Markku
Sikl's Department of Pathology, Faculty Hospital, Pilsen, Czech Republic, and the Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 2006 Jun;30(6):721-6. doi: 10.1097/00000478-200606000-00007.
Synovial sarcoma, one of the most common types of soft tissue sarcomas, usually presents in the proximal or middle portions of the extremities, often as a large mass with an aggressive clinical behavior. Gland-forming biphasic and spindle cell fibrous monophasic tumors are the most common subtypes. In this study, we evaluated 21 minute synovial sarcomas, <1 cm in diameter, from the hands and feet. These tumors occurred in 14 females and 7 males with a median age of 29 years (range, 8-60 years). Clinically, all tumors were thought to be benign processes such as a ganglion cyst or glomus tumor, and on microscopic examination, they were also often initially misinterpreted as benign lesions such as nerve sheath or (myo) fibroblastic tumors. Histologically, 7 tumors were biphasic and 14 were monophasic spindle cell variants. Microscopic calcifications were present in 8 cases and were prominent in 3 tumors. All monophasic tumors tested had elements positive for EMA, and all but one had reactivity for a keratin cocktail. S-100 protein-positive neuroma-like neural proliferations were commonly present in the monophasic but not in biphasic tumors. SYT-SSX fusion transcripts were demonstrated in 5 cases studied by polymerase chain reaction assay. All tumors were enucleated, followed by local reexcision of the site, and often combined with postoperative radiation. Three patients had amputation of the involved digit or metatarsal. Four patients had local recurrences, 2 of which were successfully treated; 2 of these patients were lost to follow-up. Despite some variation in treatment, all 12 patients with complete follow-up were alive and well, 2 to 32.2 years after surgery (median, 14.7 years), including 2 patients who received neither amputation nor postoperative radiation. Minute synovial sarcomas of hands and feet are clinically favorable tumors if completely excised; there is some evidence to suggest that they may be managed more conservatively than larger tumors. These tumors should be recognized as part of the spectrum of synovial sarcomas.
滑膜肉瘤是最常见的软组织肉瘤类型之一,通常出现在四肢的近端或中部,常表现为具有侵袭性临床行为的大肿块。腺形成双相性和梭形细胞纤维单相性肿瘤是最常见的亚型。在本研究中,我们评估了21例直径小于1 cm的来自手足的微小滑膜肉瘤。这些肿瘤发生在14名女性和7名男性中,中位年龄为29岁(范围8 - 60岁)。临床上,所有肿瘤最初都被认为是良性病变,如腱鞘囊肿或血管球瘤,在显微镜检查时,它们也常常最初被误诊为良性病变,如神经鞘瘤或(肌)纤维母细胞瘤。组织学上,7例为双相性,14例为单相性梭形细胞变体。8例出现显微镜下钙化,3例钙化明显。所有检测的单相性肿瘤EMA均呈阳性,除1例之外所有肿瘤对角蛋白混合物均有反应。S-100蛋白阳性的神经瘤样神经增生常见于单相性肿瘤而非双相性肿瘤。通过聚合酶链反应检测,在5例研究病例中证实了SYT-SSX融合转录本。所有肿瘤均行摘除术,随后对手术部位进行局部再次切除,且常联合术后放疗。3例患者接受了受累手指或跖骨截肢术。4例患者出现局部复发,其中2例得到成功治疗;这些患者中有2例失访。尽管治疗方式存在一些差异,但所有12例获得完整随访的患者在术后2至32.2年(中位时间14.7年)均存活且情况良好,其中包括2例既未接受截肢术也未接受术后放疗的患者。手足部微小滑膜肉瘤如果能完全切除,临床预后良好;有证据表明,与较大肿瘤相比,它们的治疗可能更保守。这些肿瘤应被视为滑膜肉瘤谱系的一部分。