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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。
Acinar soft-tissue sarcoma (ASPS) is a rare soft-tissue tumor whose origins have not been elucidated, often with an unbalanced chromosomal translocation t (X; 17) (p11.2; q25) and resulting in the production of the fusion gene ASPL- TFE3. Occurred mainly in the limbs, especially the lower limb deep soft tissue. Histology showed tumor cells were characterized by acinus or nest-like arrangement, the cytoplasm can be seen eosinophilic granules and crystals. Occur in young women, although the slow clinical growth, but easy blood metastasis, the prognosis is poor, the main transfer sites for the lungs, bones, liver and brain. Broad excision is the key to treatment, auxiliary radiotherapy and chemotherapy are poor. Tumor Hospital of Tianjin Medical University reported a 37-year-old female patient with left calf acinar soft tissue sarcoma with lung and brain metastases, the course of 18 years, has given surgical treatment, radiation therapy and interventional treatment, due to the progress of lung metastases, given Immunotherapy, molecular targeted therapy and Chinese medicine treatment. This article aims to explore whether modern treatment can prolong the survival of patients.