头颈部腺泡状软组织肉瘤3例并文献复习

Alveolar soft part sarcoma of head and neck:three cases report and literature review

目的探讨头颈部腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床特点,诊断和治疗方案。方法收集2005年8月~2016年8月就诊于我院的3例ASPS患者的临床资料,对患者的性别、年龄、肿瘤原发部位、有无远处转移、治疗方案等资料作回顾性分析,并进行随访。结合国内外文献对腺泡状软组织肉瘤的临床特点进行讨论。结果头颈部ASPS常见于青年女性,多数位于深部软组织中。组织学可见腺泡样或巢团样结构,周围由薄壁血管形成血窦环绕。3例术后给予放化疗,随访1-11年,1例因术中未广泛切除肿瘤,2年后远处转移而死亡,2例无瘤生存。结论 ASPS是高度恶性软组织肿瘤,早期易转移,术前应结合CT及MRI详细评估病情,手术广泛切除及放疗是主要治疗方式。

Objective To investigate the clinical features, diagnosis and treatment of alveolar soft part sarcoma （ASPS） of the head and neck. Methods From August 2005 to August 2016,there were 3 patients with ASPS who received treatment and were followed up in the our Hospital. We retrospectively analyzed the clinical factors of the disease related to prognosis,gender,age,primary tumor site,metastatic and therapy. The clinical features of ASPS were discussed in combination with literatures at home and abroad. Results ASPS of the head and neck was common in young females, most commonly located in deep soft tissue. Histologically, the tumor cells were arrange in alveolar or solid patterns and separated by sinusoidal vessels. Immunohistochemical study showed that the tumor cells were positive for TFE3.All of the patients took the postoperative radiotherapy and chemotherapy. At a follow-up from 1 to 11 years,2 patients achieved disease free survival, 1 patient died of pulmonary metastases. Conclusion Alveolar soft part sarcoma is a high grade malignant soft tissue neoplasm with early metastases to lung, bone or brain.The features of ASPS on MR and CT are very important for the surgery.Surgery and radiotherapy are the main treatment to the survival in our analysis.Further prospective studies are needed to clarify the best strategy for the treatment of ASPS.