Background: Primary intracranial germ-cell tumors (GCTs) account for about 11.1% of all primary brain tumors of children in Taiwan. Because these tumors commonly involve the hypothalamus-pituitary gland regions and their biochemical secreting character, patients frequently display neuroendocrinological symptoms and signs. Endocrinopathy, if present, often occurs prior to other neurological or radiological manifestations. This article reviews experience here, with present results comparing them with previous reports.
Methods: Twelve children who were diagnosed with primary intracranial germ-cell tumors between 1983 to 1995 were studied retrospectively. Their clinical presentations, laboratory results and treatment modalities as well as the current status were collected for presentation here.
Results: There were seven boys and five girls. The age distribution was from 5 to 15 years old. The most common symptom was increased intracranial pressure (9/12), followed by diabetes insipidus (8/12), vision deficit (8/12) and sexual precocity in 3 boys. In 11 patients the tumors arose from the suprasellar or pineal regions. In two patients the tumors arose synchronously in the suprasellar and pineal regions. Pure germinoma was found in six patients. Only one had an elevated tumor marker. These six patients all received radiation with or without operation therapy, and all are still alive. Six patients, each with a non-germinomatous malignant germ-cell tumor, had a poorer prognosis. Although they received aggressive treatment, including operation, radiation and chemotherapy, three patients died, with a mean survival period of 3.3 years.
Conclusions: In cases of diabetes insipidus in children or sexual precocity in boys, a thorough investigation for intracranial germ-cell tumors is recommended. The treatment and outcome are different for germinomas and non-germinomatous malignant germ-cell tumors. A thorough pathological diagnosis is recommended for planning of treatment protocol in order to improve prognosis.