Hanna A, Edan C, Heresbach N, Guegan Y
Service de Neurochirurgie, CHRU Pontchaillou, 35033 Rennes Cedex 2.
Neurochirurgie. 2000 Dec;46(6):568-572.
We present a case of growing teratoma syndrome of the pineal region. To our knowledge, this is the fourth case reported in the literature. A 13-year-old boy was referred for intracranial hypertension and bilateral papillary edema. CT scan showed a pineal region tumor with obstructive hydrocephalus. After CSF (cerebrospinal fluid) shunting, MRI showed that the tumor had a heterogenous signal enhancement. The tumor marker HCG (human chorionic gonadotrophin) was elevated in CSF and serum. After three cycles of chemotherapy, MRI showed an important increase in tumor size with morphologic modifications. However, HCG in CSF and serum returned to normal. Surgical resection was performed and histological examination of the whole specimen showed mature teratoma. On postoperative MRI, there was a small area of signal enhancement of the left thalamus. Radiotherapy was given. The child was in complete remission 15 months after the diagnosis. Growing teratoma syndrome is a mixed germ cell tumor with a secreting portion that responds to chemotherapy and a non secreting portion of mature teratoma that continues to grow under chemotherapy. The treatment should include chemotherapy for the malignant secreting portion and surgery for the mature teratoma.
我们报告一例松果体区成熟性畸胎瘤综合征病例。据我们所知，这是文献报道的第四例。一名13岁男孩因颅内高压和双侧视乳头水肿前来就诊。CT扫描显示松果体区肿瘤伴梗阻性脑积水。脑脊液分流术后，MRI显示肿瘤有不均匀信号增强。脑脊液和血清中的肿瘤标志物人绒毛膜促性腺激素（HCG）升高。三个周期化疗后，MRI显示肿瘤大小显著增加且形态改变。然而，脑脊液和血清中的HCG恢复正常。进行了手术切除，整个标本的组织学检查显示为成熟畸胎瘤。术后MRI显示左侧丘脑有一小片信号增强区。给予了放射治疗。诊断后15个月患儿完全缓解。成熟性畸胎瘤综合征是一种混合性生殖细胞肿瘤，其分泌部分对化疗有反应，而成熟畸胎瘤的非分泌部分在化疗下持续生长。治疗应包括针对恶性分泌部分的化疗和针对成熟畸胎瘤的手术治疗。
