39例头颈部滑膜肉瘤患者的回顾研究

Synovial sarcoma of the head and neck-a retrospective study of 39cases

目的：本研究目的在于探索头颈部滑膜肉瘤（SS）的病理诊断、肿瘤部位和临床预后之间的关系。方法：对1966—2011年间华西医院收治的39例头颈部SS患者病历资料进行回顾性研究，复习术中标本的病理切片。术后随访1～192个月，平均43．2个月。临床预后指标主要观察术后首次复发时间和患者随访时间，对结果进行统计学处理。结果：患者中男27例，女12例；年龄8～66岁，中位年龄35岁；病理诊断18例为双相性SS，17例为单相性SS，3例为低分化SS。其中4例患者标本通过荧光原位杂交或逆转录聚合酶链反应等细胞遗传学方法确诊。采用单一手术治疗的23个病例术后出现复发，其中最多的进行了4次手术。所有患者中，仅有1例发生了可疑淋巴系统转移。16例在术后行辅助放疗或化疗。4例死亡，但仅有1例死亡与肿瘤复发直接相关。肿瘤的病理分型与复发无显著相关性（Fisher确切概率法，P〉0．05），肿瘤的原发部位与复发亦无显著相关（Fisher确切概率法，P〉0．05）。结论：头颈部sS是一类特殊的实体肿瘤，具有容易复发的特点，但是预后良好。手术仍应作为头颈部SS的首选治疗方式。细胞遗传学方法对本病的确诊以及制定治疗方案具有重要意义。

Objective：Synovial sarcoma （SS） of the head and neck is rare in comparison with those took place in the extremities. This study was planned to investigate the relationship between pathological diagnosis, tumor location and clinical outcome of SS of the head and neck. Method：Thirty-nine cases of SS in head and neck hospitalized in West China Hospital from 1966 to 2011 was retrospectively studied by reviewing the medical record data, the pathological slices of the operative specimen and followed-up from 1 to 192 months with the mean time of 43.2 months postoperatively. The parameters of clinical outcome were focused on the time to first recurrence after primary surgery and follow-up time. The reviewed results were statistically processed. Result：The age of the patients ranged from 8 to 66 years old with the median age of 35, among them 27 are males. Pathologically, 18 cases are biphasic, 17 cases are monophasic and 3 cases are low-differentiated SS. 4 cases were proved by cytogenetic methods of either fluorescence in situ hybridization（FISH） or RT-PCR. 23 cases experienced repeated recurrence with the most up to 4 times operations after sole surgical approach. Only one lymphatic metastasis was suspected in all. 16 patients got adjuvant radiotherapy or chemotherapy. 4 patients died but only one death was associated directly with SS recurrence. There was no significant relationship between pathological subtype and recurrence（Fisherrs Exact Test P-value〉0.05）, no significant relationship between tumor location and recurrence（Fisher＇s Exact Test P-value〉0.05）. Conclusion：SS of head and neck is a special entity that has potential of easy recurrence but good prognosis. Surgery should still be the primary treatment approach. Cytogenetic methods are recommended to ascertain the diagnosis in order to choose reasonable treatment protocols.