Rare Primary Central Nervous System Tumors

Abstract

There are close to 70,000 new cases of primary central nervous system tumors diagnosed annually in the United States. Meningiomas, gliomas, nerve sheath tumors and pituitary tumors account for 85% of them. There is abundant literature on these commonly occurring tumors but data from the literature on infrequently encountered tumors such as atypical teratoid/rhabdoid tumor, choroid plexus carcinoma, ganglioglioma, hemangiopericytoma, and pleomorphic xanthoastrocytoma are limited. This review provides an overview of the clinicopathologic and therapeutic aspects of these rare primary central nervous system tumors.

A rare case of choroid plexus carcinoma that led to the diagnosis of Lynch syndrome (hereditary nonpolyposis colorectal cancer)

“…The invasive nature of this disease tends to preclude gross total resection but our patient was fortunate enough to undergo complete resection. Currently, there are no established guidelines to help determine whether adjuvant radiation and/or chemotherapy should be offered following surgery [5]. We discussed the case with our pediatric oncology colleagues.…”

Extraventricular Intraparenchymal Choroid Plexus Tumors in Cerebral Hemisphere: A Series of 6 Cases

“…The common locations are lateral ventricles, the fourth ventricle, the third ventricle and the cerebellopontine angle (CPA) (6,16). These tumors predominantly arise in supratentorial locations such as lateral ventricles in children and the fourth ventricle in adults (22,24).…”

Rare Primary Central Nervous System Tumors Encountered in Pediatrics

“…3 About 50% of atypical teratoid rhabdoid tumors arise in the posterior fossa, with up to 40% demonstrating leptomeningeal spread at the time of diagnosis. 4 …”