成人右侧侧脑室原发性上皮样肉瘤1例

A primary epithelioid sarcoma of the right lateral ventricle in an adult

上皮样肉瘤(ES)是一种少见且具有高度侵袭性的软组织恶性肿瘤,其特征为生长缓慢的无痛性肿块,通常发生在四肢远端,常常误诊为良性疾病;但局部复发倾向高,易发生淋巴和血液转移扩散,预后较差。中枢神经系统原发性ES极为罕见,临床表现及影像学表现无特异性,术前诊断困难,容易造成误诊而延误病情。本文报道1例60岁男性,因记忆力减退1周入院,入院体格检查未见阳性体征;颅脑MRI发现右侧侧脑室三角区大小约1.8 cm×1.6 cm结节状长T1、稍长T2信号影,增强后呈明显强化。完善术前准备,经颞叶皮层入路手术全切除右侧侧脑室三角区病灶,术后病理诊断为ES。术后未接受放化疗,术后1.5年随访,肿瘤无复发及远处转移,无神经系统阳性体征。

Epithelioid sarcoma(ES)is a rare and highly aggressive malignant soft tissue neoplasm characterized by indolent,painless growths typically manifesting in the distal extremities of the limbs.These lesions are frequently misdiagnosed as benign entities.However,ES exhibits a pronounced predilection for local recurrence and propensity for lymphatic and hematogenous dissemination,resulting in an unfavorable prognosis.Primary ES originating within the central nervous system(CNS)is exceedingly uncommon,with clinical and radiological features lacking specificity,thereby posing diagnostic challenges that may lead to misidentification and delayed intervention.This report details a 60-year-old male presenting with a one-week history of declining memory function.Physical examination revealed no discernible abnormalities.Brain MRI showed a nodular lesion measuring approximately 1.8 cm×1.6 cm in the trigone of the right lateral ventricle,displaying prolonged T1 and slightly protracted T2 signal characteristics with conspicuous enhancement following contrast administration.Following thorough preoperative preparations,a complete resection of the lesion in the right lateral ventricle was achieved via the temporal lobe cortex approach.The postoperative pathological diagnosis confirmed ES.The patient did not undergo adjuvant radiotherapy or chemotherapy postoperatively.A follow-up was conducted 1.5 years after the surgery,and no tumor recurrence or distant metastasis was observed.Additionally,there were no positive neurological signs.