Naruse Yu, Jinguji Shinya, Hiruta Ryo, Toda Ayako, Nagai Kenichiro, Kudo Shingo, Sano Hideki, Sekine Rei, Suzuki Osamu, Bakhit Mudathir, Fujii Masazumi
1Departments of Neurosurgery.
2Pediatric Oncology, and.
J Neurosurg Case Lessons. 2024 Mar 4;7(10). doi: 10.3171/CASE23718.
Bifocal germ cell tumors, with primarily identical tissue composition, occur concurrently in the neurohypophyseal and pineal regions.
A 16-year-old male patient exhibited increased intracranial pressure symptoms, with concurrent tumors in the pineal and neurohypophyseal regions, causing obstructive hydrocephalus. His serum human chorionic gonadotropin level was elevated, measuring 506.6 mIU/mL. Upon gross endoscopic examination, the pineal tumor appeared white, whereas the neurohypophyseal tumor appeared red and hemorrhagic. Because of the limited sample size of the latter, a frozen section biopsy was feasible only for the pineal lesion, which indicated the presence of a germinoma. Subsequently, carboplatin and etoposide were administered, resulting in the reduction of the pineal tumor, but no effect was observed in the neurohypophyseal tumor. Histopathological analysis confirmed the pineal lesion as a germinoma, whereas the neurohypophyseal lesion was an embryonal carcinoma. Thus, the treatment was altered to ifosfamide, carboplatin, and etoposide (ICE), leading to a response in both tumors. The patient underwent three additional cycles of ICE therapy and high-dose chemotherapy, followed by whole craniospinal irradiation, achieving complete remission.
Although most bifocal germ cell tumors share the same histological tissue, occasional differences may arise, necessitating separate biopsies for accurate assessment.
双灶性生殖细胞肿瘤主要具有相同的组织构成,同时发生于神经垂体和松果体区域。
一名16岁男性患者出现颅内压升高症状,松果体和神经垂体区域同时存在肿瘤,导致梗阻性脑积水。其血清人绒毛膜促性腺激素水平升高,为506.6 mIU/mL。大体内镜检查时,松果体肿瘤呈白色,而神经垂体肿瘤呈红色且有出血。由于后者样本量有限,仅对松果体病变可行冰冻切片活检,结果显示为生殖细胞瘤。随后给予卡铂和依托泊苷治疗,松果体肿瘤缩小,但神经垂体肿瘤未见疗效。组织病理学分析证实松果体病变为生殖细胞瘤,而神经垂体病变为胚胎性癌。因此,治疗方案改为异环磷酰胺、卡铂和依托泊苷(ICE),两肿瘤均有反应。患者又接受了三个周期的ICE治疗和大剂量化疗,随后进行全颅脊髓照射,实现完全缓解。
尽管大多数双灶性生殖细胞肿瘤具有相同的组织学类型,但偶尔也会出现差异,因此需要分别进行活检以准确评估。