论文部分内容阅读
目的探讨儿童卵巢未成熟畸胎瘤(OIT)合并腹膜神经胶质瘤病(GP)的诊治经验。方法回顾性分析2014年中山大学孙逸仙纪念医院收治的1例儿童OIT合并GP的临床资料、病理特征、治疗及预后并复习国内外文献。结果患儿女,1岁11个月,腹胀10 d。CT示腹部巨大混合密度肿块,内见脂肪、结节状钙化及液性密度影,伴大量腹腔积液,考虑恶性畸胎瘤可能。血清甲胎蛋白(AFP)295.98μg/L。术中见肿物位于右侧卵巢,大小约20 cm×15 cm×15 cm,表面不规则;腹腔积液量约1000 m L,其中可见囊性小肿物漂浮;盆腔、腹壁、网膜、双侧膈下及肝门区散布大量鹅卵石样大小不等肿物。行右侧卵巢巨大肿瘤切除、大网膜切除、右侧膈下肿物切除术。病理显示肿物符合未成熟畸胎瘤(WHO 3期)特征;鹅卵石样肿物镜下显示为成熟神经胶质组织。免疫组织化学染色结果:NSE(+),Syn(+)、S-100(+);Vimentin(+),bcl-2部分(+),Ki67约20%(+),Cg A(-)。术后复查AFP179.50μg/L。采用PEB方案(顺铂、依托泊苷、博来霉素)化疗,至今6个疗程,化疗2个疗程后血清AFP转阴性。4个疗程后腹部CT示腹膜、网膜及肠系膜弥漫性增厚,以肝周及膈下腹膜为著。5个疗程后PET/CT示右侧肋膈角、肝包膜及肝门区多发低密度灶伴高密度钙化,局部代谢增高,血清AFP 1.58μg/L。目前患儿病情稳定。结论OIT合并GP是一种儿童罕见病症,治疗应根据OIT的分期及分级,采取相应的手术或手术+化疗治疗,是否需要针对GP行相应化疗,其临床意义有待进一步研究。
Objective To investigate the diagnosis and treatment of immature ovarian teratoma (OIT) combined with peritoneal gliomatosis (GP). Methods The clinical data, pathological features, treatment and prognosis of 1 OIT combined with GP in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were analyzed retrospectively in 2014 and reviewed the literature both at home and abroad. Results Children, 1 year old and 11 months, bloating 10 d. CT showed abdomen huge mixed density mass, see the fat, nodular calcification and liquid density shadow, with a large number of ascites, malignant teratoma may be considered. Serum alpha-fetoprotein (AFP) 295.98 μg / L. Surgery, see the tumor located in the right ovary, size of about 20 cm × 15 cm × 15 cm, surface irregularities; peritoneal effusion volume of about 1000 m L, which showed cystic small floating objects; pelvic, abdominal wall, omentum, Both sides of the diaphragm and the hilar area scattered a large number of pebble-like size of the tumor. Line right ovary huge tumor resection, omental resection, right subphrenic tumor resection. The pathology showed that the tumor was characteristic of immature teratoma (WHO stage 3); the pebble-like appearance was microscopically microscopic. Immunohistochemical staining results showed that NSE (+), Syn (+), S-100 (+); Vimentin (+), bcl-2 part (+), Ki67 about 20% (+), Cg A (-). Postoperative review AFP179.50μg / L. PEB program (cisplatin, etoposide, bleomycin) chemotherapy, so far 6 courses, 2 courses of chemotherapy after chemotherapy AFP negative. After 4 courses of abdominal CT showed peritoneum, omentum and mesentery diffuse thickening of the liver and peritoneal diaphragm. After 5 courses of PET / CT, the right costophrenic angle, hepatic capsule and portal lesion with high density calcification and local metabolism increased, serum AFP 1.58 μg / L. Children with stable condition. Conclusion OIT combined with GP is a rare disease in children. The treatment should be based on the staging and grading of OIT, and the corresponding surgery or surgery plus chemotherapy should be taken. Whether the need for GP chemotherapy is needed or not, the clinical significance remains to be further studied.