Long-term outcome of endocrine function in patients with neurohypophyseal germinomas

Since neurohypophyseal germinomas occur at the pituitary and hypothalamic a xis in children and adolescents, the endocrinopathy is one of the common an d critical QOL determinants. We carried out a retrospective study on the ou tcome of endocrine function in patients with neurohypophyseal germinoma, in order to improve or preserve pituitary function after treatment. Sixteen p atients (7 men and 9 women), aged 6 to 26 years were admitted and followed up for 95.3 (14-197) months. DI was noted in 12 patients in pretreatment an d 16 in posttreatment regardless of tumor size. We carried out the replacem ent of GH in all 8 patients, presenting the symptoms under 15 years of age. Gonadal or gonadotropic, thyroid and adrenal hormones were replaced in 9, 12 and 15 patients, respectively. Patients with large tumor compressing chi asm or hypothalamus needed hormonal replacement such as gonadal or gonadotr opic and thyroid hormones more frequently (<0.01) than those with small one . In addition, two patients with a small tumor at the pituitary stalk and t he 3rd ventricle floor showed the improvement of secretion pattern in gonad otropins and ACTH after chemotherapy, although they later needed radiation therapy to control the tumor. Based on our study and review of literature, the endocrinological studies before and after treatment demonstrated that p ituitary dysfunction present before treatment persisted or worsened even af ter tumor remission, except for patients with small and localized ones. The poor endocrine results is considered to be largely radiation-related. Chem otherapy alone seems to be insufficient to obtain complete response (CR). T o avoid radiation related pituitary injury, combination of 24 Gy or less do sage of radiation and appropriate chemotherapy is essential. The earlier di agnosis by repeatedly using neuroimaging and serum and CSF tumor markers an d earlier initiation of treatment, before irreversible pituitary-hypothalam ic damage occurs, contributes to improvement of the outcome of pituitary fu nctions in patients with neurohypophyseal germinomas.