De novo germinoma in the brain in association with Klinefelter's syndrome: Case report and review of the literature

BACKGROUND There are no previous reports about de novo germ cell tumors without any past history of germ cell tumor. We describe a case of de novo cerebral germinoma in association with Klinefelter's syndrome. CASE DESCRIPTION A boy had undergone growth hormone therapy for dwarfism because of hypopituitarism from 10 to 17 years old. The result of karyotyping at the age of 13 was 47,XXY. Magnetic resonance images (MRI) of the brain at the age of 17 years showed no lesions. Two years later, at the age of 19, the patient noticed onset of mild right hemiparesis. MR imaging revealed the existence of a brain tumor in the left temporal lobe and hypothalamus. The patient underwent an operation and the histologic diagnosis of the lesion was germinoma. After postoperative chemotherapy and radiation therapy, the lesion disappeared and the patient was discharged uneventfully. CONCLUSION To the best of the authors' knowledge, this is the first reported case of a germ cell tumor to be de novo without any past history of other germ cell tumor and the seventh case in which it occurred in association with Klinefelter's syndrome. (C) 2003 Elsevier Inc. All rights reserved.