Revised: June 2014

Tumours of the pineal region are rare, comprising about 1% of intracranial lesions. The majority of these are germinomas in young patients. Other tumour types, arising from the parenchyma, include pineocytoma, pineoblastoma and astrocytoma.

The biologic behaviour of pineocytomas is not well known because of small patient numbers and variable behaviour. Those demonstrating neuronal differentiation have a more favorable prognosis(1). Surgery and observation may be feasible with radiation reserved for progression or recurrence. Tumours that do not show neuronal differentiation or are incompletely resected may benefit from adjuvant focal RT but evidence is limited (2).

Pineoblastomas are primitive neuroectodermal tumours (PNET). They tend to disseminate within the craniospinal axis, therefore, a staging MRI of the spine should be performed. Radiotherapy involves craniospinal radiation along with a boost to the primary. There may be a role for chemotherapy in selected patients (3).

Astrocytic tumours of the pineal are treated like astrocytomas at other intracranial sites. (see low grade astrocytoma and malignant astrocytoma.)

Reference:

1. Jouvet A, Saint-Pierre G, Fauchon F, et al. Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases. Brain Pathol 2000; 10:49.

2. J, Ramiro J, Martínez R, et al. Clinicopathological experience with pineocytomas: report of five surgically treated cases. Neurosurgery 1990; 27:612.

3. Reddy AT, Janss AJ, Phillips PC, et al. Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer 2000; 88:2189.