Endocrine Abstracts

Background: Germ cell tumors (germinoma, teratoma, embryonal carcinoma, yolk sac tumor and choriocarcinoma) constitute 0.3–3.4% of all primary intracraneal tumors. Germinoma is the most common and of better prognosis.

Aim: Describing the behaviour of germ cell tumors of the pituitary–hypothalamic region: location, clinical characteristics, diagnosis and follow-up.

Material and methods: Review of medical records of patients with germ cell tumors of the pituitary–hypothalamic region diagnosed between 1998 and 2011 attended at Hospital Universitario Virgen del Rocío in Seville. Were determined the clinical presentation, location, process followed to achieve diagnosis, treatment and evolution.

Results: Thirteen patients were diagnosed: four male (30.7%) and nine female (69.23%). Nine of the 13 patients (69.2%) had an age of 14 or younger.

Twelve patients (92.3%) displayed diabetes insipidus, being the first symptom in 11 of them (84.6%). All subjects displayed panhypopituitarism at the beginning of the treatment.

In four patients (30.7%) with diabetes insipidus, the initial MRI was normal or only displayed thickened pituitary stalk; these patients were finally diagnosed with a radiologic follow-up and/or biopsy.

Nine of the 13 patients (69%) were diagnosed after biopsy or surgery and in the four remainders (31%) by the elevation of the serum and/or cerebrospinal fluid (CSF) tumor markers.

In nine of which pathologic anatomy records were available, eight were germinomas and one was yolk sac tumor.

The average time of evolution since diagnostic is 4.67 years. Nowadays, 12 of the 13 patients are alive.

Conclusion: Diabetes insipidus is the most frequent symptom in the germinomas of the pituitary-hypothalamic region. When diabetes insipidus is diagnosed a close clinic and radiologic follow-up is compulsory as the patient might suffer a germinal tumor or develop other hormonal deficits.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.