口腔颌面部腺泡状软组织肉瘤18例临床分析

Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis of 18 patients

目的 ：总结口腔颌面部腺泡状软组织肉瘤的临床表现、诊断、治疗及预后特点。方法 ：收集1995年1月—2013年12月就诊于上海交通大学医学院附属第九人民医院口腔颌面—头颈肿瘤科的18例腺泡状软组织肉瘤患者的临床资料,包括患者的性别、年龄、肿瘤大小、原发部位、有无远处转移、确诊方式、治疗及预后等,并对患者的资料进行回顾分析。结果：18例患者中,女10例、男8例;平均发病年龄20.2岁（3～61岁）;肿瘤平均直径4 cm（1.5～6.5 cm）;原发部位包括：舌（10例）、颊（5例）、口咽（1例）、上前牙龈（1例）、下前牙龈（1例）,其中1例发病初期即有肺转移,1例术后46个月发现肺转移。所有患者均经病理明确诊断,均以病灶扩大切除为主要治疗手段,术后放疗3例,辅助性化疗1例。患者平均随访33个月（6～75个月）,末次随访时,1例早期肺转移患者带瘤生存19个月,1例复发伴多处肺转移患者死亡,其余均无复发、转移。结论：口腔颌面部腺泡状软组织肉瘤的临床表现具有其自身特点;确诊依据免疫组织化学及PAS染色;治疗以病灶扩大切除为主,多数病例在病灶彻底切除后能够获得较好的预后。

PURPOSE： To summarize the clinical features, diagnosis, treatment strategies and prognosis of alveolar soft part sarcoma （ASPS） of oral and maxillofacial region. METHODS： We performed a retrospective study in a consecutive series of 18 patients with ASPS in the oral and maxillofacial region between 1995 and 2013. Demographics, tumor sizes, sites, metastasis and diagnosis of disease, treatments provided, and follow-up outcomes were documented and analyzed. RESULTS： Eighteen patients （10 females, 8 males; mean age of 20.2 years, range, 3-61 years） who were diagnosed pathologically had their primary tumor in the tongue （10）, cheek （5）, pharynx （1）, gingival （2）, with an average size of 4 cm （range, 1.5-6.5 cm）. Two patients exhibited pulmonary metastases. Extensive resections were performed in all patients. Postoperative radiotherapy was delivered in 3 cases, and adjuvant systemic chemotherapy was given to 1 case. Mean follow-up periods were 33 months （range, 6-75 months）. At the latest follow-up, one patient with lung metastases survived for 19 months, and one died 3 months after confirmation of local recurrence and multiple pulmonary metastases, the other patients remained in good health and were disease-free. CONCLUSIONS： ASPS of the oral and maxillofacial region has unique clinical characteristics. The immunohistochemical findings and PAS-positive results help to make the final diagnosis. The most commonly advocated and effective treatment for ASPS of oral and maxillofacial region remains to be radical surgical excision of the lesions.