Hormonal and metabolic abnormalities associated with central nervous system germinoma in children and adolescents and the effect of therapy: report of 10 patients.
We describe the results of clinical and endocrinological investigations performed on 10 children and adolescents (5 males and 5 females) with a primary central nervous system germinoma. Eight of 10 patients were between 10-20 yr of age at the time of initial presentation. Polyuria (7 of 10) and a decrease in or cessation of linear growth (5 of 10) were the most common presenting symptoms, while only 2 of 10 patients complained of visual problems. Two patients presented with the syndrome of polyuria, adipsia, hypernatremia, profound muscle weakness, and hyperlipidemia. Initial physical exam revealed abnormal eye findings in 60%, short stature (greater than or equal to 2.5 SD) in 50%, and abnormal pubertal development in 30% of the patients. The neoplasm was located in the suprasellar-hypothalamic region in 8, caudate nucleus in 1, and pineal region in 1. Biopsy performed in 7 patients revealed the classic two-cell germinoma in all cases. Assessment of endocrine function before radiotherapy documented pituitary deficits in all patients studied. Antidiuretic hormone was deficient in 8 of 10 patients and was associated with hypoadipsia in 4. GH was deficient in al patients tested (7 of 7). TSH (5 of 8), ACTH (3 of 7), and gonadotropin (1 of 1) deficiencies were also common before treatment. Plasma PRL concentrations were elevated in 5 of 8 patients, all with suprasellar tumors. The hCG values were elevated only in the patient with sexual precocity (1 of 10). Endocrine evaluation during the postirradiation period revealed additional instances of GH (1), ACTH (1), and gonadotropin (5) deficiencies. All 10 patients are alive without evidence of active disease 6 months to 10 yr after radiation therapy (4500-5100 R). Evidence of hypothalamic-pituitary dysfunction is an early and almost universal feature of central nervous system germ cell tumors. The importance of careful evaluation and follow-up of children with acquired anterior or combined anterior and posterior pituitary dysfunction for a suprasellar tumor is stressed.
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000172:deficiency*; D001932:Brain Neoplasms / Q000378:metabolism* / Q000532:radiotherapy; D002421:Caudate Nucleus; D002648:Child; D002675:Child, Preschool; D004407:Dysgerminoma / Q000378:metabolism* / Q000532:radiotherapy; D005260:Female; D006065:Gonadotropins, Pituitary / Q000172:deficiency*; D013006:Growth Hormone / Q000172:deficiency*; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000378:metabolism; D008297:Male; D010871:Pinealoma / Q000378:metabolism; D011388:Prolactin / Q000097:blood; D013972:Thyrotropin / Q000172:deficiency; D014057:Tomography, X-Ray Computed; D014667:Vasopressins / Q000172:deficiency
D006065:Gonadotropins, Pituitary; D014667:Vasopressins; D000324:Adrenocorticotropic Hormone; D011388:Prolactin; D013972:Thyrotropin; D013006:Growth Hormone