中国实用儿科杂志

中国实用儿科杂志

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儿童卵巢未成熟畸胎瘤合并腹膜胶质瘤病1例并文献复习

  

  1. 中山大学孙逸仙纪念医院  a.儿科血液/儿童肿瘤专科，b.小儿外科，c.病理科，广东  广州  510120
  • 出版日期:2016-05-06 发布日期:2016-05-10

Ovarian immature teratoma（OIT） with gliomatosis peritonei（GP） in children：A report of one case and review of the literature

  1. Department of Pediatric Hematology/Oncology，SUN Yat-sen Memorial Hospital，SUN Yat-sen University，Guangzhou  510120，China
  • Online:2016-05-06 Published:2016-05-10

摘要：

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）295.98 μg/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 mL，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%（+），CgA（-）。术后复查AFP 179.50 μg/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 1.58 μg/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。

关键词: 儿童, 畸胎瘤, 卵巢, 腹膜胶质瘤病

Abstract:

Objective    To explore the diagnosis and therapeutic procedure of a child case of ovarian immature teratoma （OIT） associated with gliomatosis peritonei（GP）. Methods    The clinical features and pathological characteristics of a child case of OIT associated with GP admitted in 2014 in SUN Yat-sen Memorial Hospital，SUN Yat-sen University were analyzed retrospectively；the treatment method and follow-up data were analyzed as well，and related literatures were reviewed. Results    A nearly 2-year-old girl presented with a 10-day history of painless increasing abdominal girth was hospitalized. A computed tomography（CT） examination of the abdomen revealed a large and complex mass containing tissue of fat，calcified nodules and liquid density，accompanied by a large amount of ascetic fluid，consistent with a malignant teratoma. The patient’s alpha-fetoprotein（AFP） level was elevated to 295.98 μg/L （reference range＜25 μg/L）. Exploratory laparotomy showed a large right ovarian mass with an irregular surface measuring 20 cm×15 cm×15 cm. A total of 1000 mL of clear ascite fluid with several cystic masses floating in it was drained. In addition，there were multiple nodules，1 cm to 2 cm in diameter，noted on the surface of the pelvis，abdominal wall，peritoneum，and both sides of undersurface of the diaphragm and hepatic hilar region. The right ovarian tumor，omentum majus and the mass on the undersurface of the right diaphragm were resected. The pathologic examination revealed an OIT（WHO grade III）， and the abdominal implants revealed mature glial tissue. Immunohistochemically， the tumor was positive for NSE， Syn， S-100 protein and vimentin，as well as part of bcl-2 and about 20% Ki67，but negative for CgA. The serum AFP after the surgery was 179.50 μg/L （reference range＜25 μg/L）. After the surgery，the patient was given 6 cycles of cisplatinum，etoposide （VP16） and bleomycin（PEB） therapy and had an uneventful recovery with a normal AFP level after 2 cycles of chemical therapy. A computed tomography（CT） scan of the abdomen after 4 cycles of therapy showed the peritoneum，omentum and the mesentery were thickened，especially the peritoneum around the liver and on the sub-phrenic surface；in addition，the right ovary was not well seen. A PET/CT after 5 cycles of chemical therapy showed low density and calcification in right costophrenic angle，liver capsule and hepatic hilar region. Serum AFP was 1.58 μg/L. The patient was doing well with no evidence of recurrence till now. Conclusion    OIT with GP is a rare condition in children. Combination therapy including surgery and chemotherapy should depend on the classification of OIT. Treatment for GP still requires clinical research.

Key words: children, teratoma, ovary, gliomatosis peritonei