Yang Wen-Ping, Chien Hung-Yu, Lin Yi-Chun
Section of Endocrinology and Metabolism, Department of Medicine, Taipei City Hospital Ren-Ai Branch, Taipei 106, Taiwan, R.O.C.
Section of Endocrinology and Metabolism, Department of Medicine, Taipei Veterans General Hospital, Taipei 112, Taiwan, R.O.C.
Oncol Lett. 2017 Jul;14(1):1129-1132. doi: 10.3892/ol.2017.6213. Epub 2017 May 19.
A 38-year-old male patient presented with general weakness, polydipsia and a body weight loss of 10 kg in two years. Hypopituitarism with central hypothyroidism and central adrenal insufficiency were noted at Taipei City Hospital (Taipei, Taiwan). However, hypogonadotropic hypergonadism was also observed. The patient was diagnosed with an intracranial β-human chorionic gonadotropin (β-hCG) secreting germ-cell tumor, and brain magnetic resonance imaging revealed that the tumor involved the pineal gland, stalk, posterior pituitary gland, right basal ganglion, hypothalamus, corpus callosum and posterior hippocampus. The cerebrospinal fluid (CSF) β-hCG level was 1936 IU/l, while the α-fetoprotein (AFP) level was <0.24 ng/ml. The serum AFP level of the patient was 3.28 ng/ml, and the β-hCG level was 178 IU/l with a CSF:serum β-hCG ratio >2:1. The patient was successfully treated with chemotherapy and radiotherapy, as demonstrated by a marked decrease in size of the tumor and in the serum β-hCG levels. Intracranial β-hCG secreting germ-cell tumors are rare in adults and manifest differently compared with patients of early pubertal age. In contrast with the precocious puberty frequently observed in young patients, the diagnosis of adult patients is often delayed and the symptoms are associated with tumor size and location. The present case report described an adult male with an intracranial β-hCG secreting GCT, demonstrating hypopituitarism and asymptomatic hyperandrogenemia, and reviews and discusses the literature relevant to the case.
一名38岁男性患者出现全身乏力、多饮症状,且在两年内体重减轻了10千克。台北市立医院(台湾台北)诊断该患者患有垂体功能减退症,伴有中枢性甲状腺功能减退和中枢性肾上腺功能不全。然而,还观察到该患者存在低促性腺激素性高雄激素血症。患者被诊断为颅内β-人绒毛膜促性腺激素(β-hCG)分泌性生殖细胞瘤,脑部磁共振成像显示肿瘤累及松果体、垂体柄、垂体后叶、右侧基底神经节、下丘脑、胼胝体和海马体后部。脑脊液(CSF)β-hCG水平为1936 IU/l,而甲胎蛋白(AFP)水平<0.24 ng/ml。患者血清AFP水平为3.28 ng/ml,β-hCG水平为178 IU/l,脑脊液与血清β-hCG比值>2:1。患者通过化疗和放疗成功治愈,肿瘤大小和血清β-hCG水平均显著下降。颅内β-hCG分泌性生殖细胞瘤在成人中较为罕见,与青春期早期患者的表现有所不同。与年轻患者中常见的性早熟相反,成年患者的诊断往往延迟,症状与肿瘤大小和位置有关。本病例报告描述了一名患有颅内β-hCG分泌性生殖细胞瘤的成年男性,表现为垂体功能减退和无症状性高雄激素血症,并对与该病例相关的文献进行了综述和讨论。