腹膜胶质瘤病临床病理分析

Clinicopathologic features of gliomatosis peritonei

目的:探讨腹膜胶质瘤病(gliomatosis peritonei,GP)的临床病理特征。方法:观察和分析15例GP患者的临床病理学特征,并复习相关文献。结果:15例GP患者,年龄6~28(中位22)岁;合并卵巢未成熟畸胎瘤12例,成熟畸胎瘤2例,混合性生殖细胞瘤1例;15例中8例与原发瘤同时发现,7例二次手术时发现。随访5~148个月,至随访终点,15例中有4例复发或进展;GP是否复发或进展与原发瘤破裂/粘连有显著相关性(χ~2=2.47,P=0.03),与患者年龄、化疗与否、是否妊娠、淋巴结有无转移、原发肿瘤大小、原发肿瘤是否成熟、原发瘤未成熟成分分级、GP自身大小均无统计学意义(P>0.05)。结论:GP非常罕见,主要发生于卵巢未成熟畸胎瘤,有着独特的临床病理学特征,当原发瘤破裂/粘连时,复发或进展概率增高,须长期随访。

Objective： To elucidate the pathological and clinical features of gliomatosis peritonei （GP）. Methods： Fifteen cases of GP were obtained from Peking Union Medical College Hospital （PUMCH）. The clinical and pathological findings were analyzed with a review of literature. Results： Fifteen patients aged 6–28 （median, 22） years. The primary ovarian tumors consisted of immature teratoma （n=12） and mature teratoma （n=2）. GP was discovered with the primary tumor in 8 patients and at a secondary surgery in 7 patients. Follow-up data were available for 15 patients with a follow-up time of 5–148 months. Recurrence or progression were found in 4 of 15 patients （26.7%）. We found that recurrence or progression was correlated with primary tumor rupture or adhesion （χ2=2.47, P=0.03）, but not correlated with age, chemotherapy, pregnancy, lymphatic metastasis, primary tumor size, primary tumor maturity, grade of primary tumor （P〉0.05）. Conclusion： GP is rare, and often associated with ovarian teratoma. Owing to the risk of recurrence and malignant transformation of GP, a long-term follow-up is necessary, especially for those with primary tumor rupture or adhesion.