Germ Cell Tumors

Abstract

Germ cell tumors (GCTs) are a rare group of pediatric brain tumors that occur in the midline and often present with endocrinopathies including central diabetes insipidus (DI) or anterior pituitary deficiencies. Symptoms of central DI may be present in patients for months to years prior to definitive diagnosis. Patients may require long-term serial imaging and monitoring of tumor markers to ensure appropriate diagnosis and treatment. Germ cell tumors are subdivided into germinomas and nongerminomatous germ cell tumors (NGGCT) based on histology and tumor markers. Germinomas are quite radiosensitive, and historically have been treated with radiation therapy alone. NGGCTs are less responsive to radiation and require a more aggressive treatment approach with the addition of chemotherapy and sometimes surgery. Due to the complexities of diagnosis and management, these patients require a multidisciplinary approach to management including endocrinology, neurosurgery, neuro-oncology, radiation oncology, and neuro-ophthalmology.