左小腿肿物伴肺脑转移

A Patient with Neoplasm in the Left Leg with Lung and Brain Metastases

腺泡状软组织肉瘤(ASPS)是一种罕见的软组织肿瘤,其组织起源尚未定论,往往伴有不平衡染色体易位t(X;17)(p11.2;q25)并导致产生融合基因ASPL-TFE3。发生部位主要位于四肢尤其是下肢的深部软组织。组织学可见瘤细胞呈特征性的腺泡或巢团样排列,胞质内可见嗜伊红颗粒及结晶体。好发于年轻女性,虽然临床生长缓慢,但易血行转移,预后差,主要转移部位为肺、骨、肝、脑。广泛切除是治疗的关键,辅助放、化疗效果差。天津医科大学附属肿瘤医院报道1例37岁女性患者,左小腿腺泡状软组织肉瘤伴肺、脑转移,病程18年,先后给予手术治疗、放射治疗及介入治疗,由于肺转移灶再次进展,给予免疫治疗、分子靶向治疗及中医药治疗。本文旨在探讨现代治疗方法能否延长患者生存时间。

Alveolar soft part sareoma （ASPS） is a tare soft tissue tumor of uncertain histogenesis with a non-reiprocal transloeation der （17） t （X;17） （p1 1.2; q25） leading to the fusion of the ASPL gene to the gene encoding transcription faetor TEF3. ASPS is mainly found in the extremities, especially the deep soft parts of lower limbs. Histologically, the tumor shows typical alveolar and nest arrangcmcnt with eosinophilic granules or crystals in the cytoplasm of tumor eells. ASPS often occurs in young women. Ahhough it displays a slow elinieal course, it often melastasizes to the lung, bone, liver and brain, worsening the prognosis. Complete resection is critieat tor treatment of localized alveolar soft part sarcolna. Adjuvant chemotherapy and radiotherapy have demonstrated few benefits. Here. we present a case of primary ASPS of the left leg with lung and brain metastases in a 37-year-old woman. Site underwent surgery, radiotherapy and interventional therapy. Because of the progression of metastases in her lungs site was also given immunotherapy, molecular target therapy and traditional Chinese medicine. We investigate whether the modern treatment can prolong the survival of ASPS patients.