The management of pineal tumors is a model for multidisciplinarity. Apart from an emergency situation that requires immediate shunting of cerebrospinal fluid (CSF), the initial discussion should involve at least a radiologist, a surgeon, a neurologist and an oncologist. The initial decision is whether obtaining a histological proof is obligatory. It depends on age and ethnicity, site (mono- or bifocality), presence of markers in serum as well as CSF, and/or of malignant cells in the CSF. In cases of marker elevation indicating a germ cell tumor, front line chemotherapy can avoid dangerous immediate surgery. When histological proof is required, the extent of surgery should be discussed, aiming either only at obtaining tissue or removal. If a germ cell tumor is detected, treatment will include a cisplatin-containing chemotherapy followed by focal or ventricular irradiation. Tumors of the pineal parenchyma will be treated according to grade, either by surgery alone (pinealocytoma) or chemo-radiotherapy (pinealoblastomas). Similarly, gliomas will be treated depending on their grade with several different possible lines in low grade, and usually radio-chemotherapy in high grade. A careful balance between improved survival rates and decreased long-term side effects will guide the decisions of all these specialists.
Keywords: Germ cell tumors; Germinoma; Germinome; Glande pinéale; Gliomas; Gliomes; Pineal; Pinealoblastoma; Pinealocytoma; Pinéaloblastome; Pinéalocytome; Tumeurs germinales.
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