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Abstract.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. IntroductionPrimary intracranial germ-cell tumors (GCT) are rare tumors. They account for ~0.5-3% of all pediatric primary central nervous system (CNS) tumors in Western regions, but are observed at higher frequencies among pediatric CNS tumors in Asia, accounting for up to 10% (1). The incidence in the Far East area of Japan is 0.1-0.17 per 100,000 per year, according to the Brain Tumor Registry of Japan (2), slightly higher than the incidence rate of 0.1 per 100,000 per year in the United States (3). Primary intracranial GCT typically occurs in children or young adults, with the majority of patients (60-70%) aged under 20 (4). The peak incidence of intracranial germ-cell tumors is in the early pubertal period with a median age of diagnosis at 10-12 years (5). The disease occurs primarily in males, with the ratio of male to female between 2:1 and 3:1 (6). The World Health Organization had classified intracranial germ-cell tumors into three groups as follows: Germinomas, non-germinomatous germ-cell tumors (NGGCTs) and mixed germ-cell tumors (7). The diseases are heterogeneous in terms of histology, tumor characteristics, treatment response and tumor marker secretion. Among NGGCTs, choriocarcinoma secretes β-human chorionic gonadotropin (β-HCG) into the serum and/or the cerebrospinal fluid (CSF), with high levels detected, while yolk sac tumors secrete α-fetoprotein (AFP). Elevation of AFP levels, combined with characteristic magnetic resonance imaging (MRI) results, is diagnos...