Karimifar M, Ghanavat M
Isfahan Endocrine and Metabolism Research Centre, Isfahan University of Medical Sciences, Isfahan, Iran.
Cancer Prevention Research Center/ Isfahan University of Medical Sciences, Isfahan, Iran.
Acta Endocrinol (Buchar). 2023 Jan-Mar;19(1):99-103. doi: 10.4183/aeb.2023.99. Epub 2023 Aug 14.
Intracranial germ cell tumors (GCTs) include two categories: germinoma and non germinoma. The pineal gland and suprasellar are the most common site of involvement. The patient is a 14-year-old boy who presented with paralysis of upward gaze, polyuria, polydipsia and diplopia. Examination of vertical eye movements was impaired. Puberty then progressed to stage 3 of Marshall - Tanner and had stopped. In laboratory studies, the patient had anterior hypopituitarism and diabetes insipidus (DI). In pituitary and hypothalamic MRIs, a mass-like enlargement of the pituitary stalk and pineal region was seen, but due to the small size of the lesions, stereotactic biopsy was not possible. During this time, the patient developed recurrent attacks of hydrocephalus. Serum and CSF β-subunit of Human chorionic gonadotropin levels were negative. Treatment was not started because different lesions could cause disease in this area. After one year, a biopsy was performed and the germ cell tumor was diagnosed. Chemotherapy was started and after treatment the vision disorder and diplopia disappeared, but DI and pituitary dysfunction remained. Treatment of intracranial germinoma is multidisciplinary. GCTs are very sensitive to radiation therapy. They are treated with combination of chemotherapy, radiotherapy, surgery, endocrine therapy and have a good prognosis.
颅内生殖细胞肿瘤(GCTs)包括两类:生殖细胞瘤和非生殖细胞瘤。松果体区和鞍上区是最常受累的部位。该患者为一名14岁男孩,表现为向上凝视麻痹、多尿、多饮和复视。垂直眼球运动检查受损。青春期进展至马歇尔-坦纳第3阶段后停止。实验室检查显示,该患者存在垂体前叶功能减退和尿崩症(DI)。在垂体和下丘脑的磁共振成像(MRI)中,可见垂体柄和松果体区呈肿块样增大,但由于病变较小,无法进行立体定向活检。在此期间,该患者出现了脑积水的反复发作。血清和脑脊液中人绒毛膜促性腺激素β亚基水平均为阴性。由于该区域不同病变均可导致疾病,故未开始治疗。一年后进行了活检,诊断为生殖细胞肿瘤。开始化疗,治疗后视力障碍和复视消失,但尿崩症和垂体功能障碍仍然存在。颅内生殖细胞瘤的治疗是多学科的。GCTs对放射治疗非常敏感。它们采用化疗、放疗、手术、内分泌治疗相结合的方法进行治疗,预后良好。