Pediatric central nervous system primitive neuro-ectodermal brain tumors (CNS-PNETs) are rare tumors with ill-defined biological features. In this issue of Cell, Sturm et al. used state-of-the-art methods to interrogate these tumors' biology. Their integrated molecular analyses led them to propose a new molecular classification, with four new entities identified, that should get oncologists' at...

PURPOSE The aim of this study was to investigate the incidence and spectrum of malignant tumors in Korean neurofibromatosis type 1 (NF1) patients. METHODS We retrospectively reviewed 125 patients who were diagnosed with NF1 at a single institution from 1995 to 2010. The incidence, location, histologic type, and radiologic findings of malignant tumors as well as development of multiple primary...

Central neurocytomas are uncommon tumors of the CNS, representing approximately 0.1-0.5% of all primary CNS tumors. Patients most often present with symptoms of increased intracranial pressure (headache, nausea/vomiting, diplopia) due to obstructive hydrocephalus. Rarely, central neurocytomas may present with a hemorrhage. Central neurocytomas are intraventricular tumors with a predilection for...

In order to accurately assess the carcinogenicity of chemicals with regard to rare tumors such as rat CNS tumors, sufficient information about spontaneous tumors are very important. This paper presents the data on the type, incidence and detected age of CNS tumors in F344/DuCrlCrlj (a total of 1363 males and 1363 females) and Crl:CD(SD) rats (a total of 1650 males and 1705 females) collected fr...

  Ependymomas accounts for about 2%–6% of CNS and 60%–70% of spinal cord tumors. Several histological patterns of these neoplasms are well known, but little attention has been devoted to a variant composed of giant cells. In spite of apparently “worrisome” histology, giant cell ependymoma seems to be a neoplasm with a relatively good prognosis. This report presents a case of giant cell ependym...

Although these entities are histologically similar, recent advances in molecular genetics have allowed the distinction of central nervous system extraosseous Ewing sarcoma (CNS-EES) from central primitive neuroectodermal tumors (c-PNET) including medulloblastoma and supratentorial PNET. We present 2 cases of pathologically confirmed CNS-EES. Knowledge of CNS-EES as a distinct entity enables the...

Germinomas and embryonal carcinomas are central nervous system (CNS) germ cell tumors (GCT) that may overexpress the proto-oncogene c-KIT, a receptor tyrosine kinase, of which dasatinib is a potent inhibitor. This retrospective review presents the feasibility and tolerability of dasatinib administration in select patients with CNS germinoma. Between November 2008 and April 2010, six patients wi...

Traditionally, cytotoxic drugs have played a limited role in the treatment of brain tumors, but important advances in chemotherapy have occurred during the past decade. Certain central nervous system (CNS) malignancies are remarkably chemosensitive. These include primary CNS lymphoma, medulloblastoma, oligodendroglioma, and intracranial germ-cell tumors. This review focuses on advances in the c...