Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: case report and review of the literature.

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare central nervous system tumors of childhood that were recently described as a new entity. DLGNTs usually manifest with symptoms related to increased intracranial pressure or spinal cord compression. The classic radiological feature is a widespread leptomeningeal enhancement that may involve the entire neuroaxis. Microscopic examination demonstrates oligodendroglial-like cells that are positive for OLIG2, MAP2, and S100 and negative for IDH-1. Anaplastic features occur in some cases. Molecularly, DLGNTs are characterized by chromosome arm 1p deletion and alteration of a mitogen-activated protein kinase (MAPK) pathway gene, most commonly BRAF-KIAA1549 fusion. There is no established grading system for these tumors, which may have an indolent or aggressive behavior. Treatment usually involves chemotherapy and radiation therapy.

A Diffuse Leptomeningeal Glioneural Tumor Case Producing Hydrocephalus and Polyradiculopathy

The present report describes the case of a male 17-year-old patient who progressively developed a hydrocephalus and polyradiculopathy due to involvement of central nervous system (CNS) by a diffuse leptomeningeal glioneuronal tumor (DLGNT). The tumor had partial remission in response to the treatment with radiotherapy plus procarbazine, lomustine, and vincristine (PCV) chemotherapy, and the patient had improvement in function and pain levels. The current knowledge about DLGNT, including its clinical manifestations, imaging findings, histological characteristics, and treatment are revised and discussed in the present paper.

Rosette-forming glioneuronal tumor: an update.

Rosette-forming glioneuronal tumor (RGNT) is a rare and distinct primary nervous system tumor. The literature on this novel neoplasm is sparse and limited to mostly case reports. Reviews on the characteristics of this tumor are fewer and far between with the latest up to a decade old. We thus provide a comprehensive review of recent literature to characterize presenting symptoms, radiological evidence, treatment options, and prognosis of this novel neoplasm. A Medline search for case reports detailing primary rosette-forming glioneuronal tumors was performed. RGNTs are a benign tumor of indolent course with mixed glial and neurocytic components. There is a slight female predominance with mean age of presentation at 23.57 years. Headaches, visual disturbances, and nausea and vomiting are the most common symptoms. Most RGNTs have solid and cystic components, arising most frequently in the fourth ventricle or cerebellum. Management is usually through surgery with gross total resection (GTR) providing better prognosis.

Disseminated glioneuronal tumor with neuropil-like islands of the spinal cord: a distinctive entity.

BACKGROUND: Glioneuronal tumor with neuropil-like islands (GTNI) was recently added as a novel lesion in the most recent update of the World Health Organization classification of tumors of the central nervous system in 2007. Since this tumor's initial description, approximately 28 cases of GTNI have been published. In this report, we describe the ninth case of a spinal GTNI in the world literature. METHODS: We report a case arising in a 2-year-old female patient who presented with headaches associated with intermittent vomiting due to a tetraventricular hydrocephalus. RESULTS: After ventriculoperitoneal shunt placement, the patient presented with lower extremity motor weakness and sensory disturbance. A dorsolumbar spine magnetic resonance imaging scan revealed an intramedullary spinal neoplasm involving T12 through L2 in association with the thick linear enhancement of the spinal cord surfaces. A brain magnetic resonance imaging scan demonstrated focal leptomeningeal enhancement in the Sylvian fissures, the basal cistern, tentorium, and multiple small cystic-like lesions extending on the cerebellar surface, brainstem, and temporal lobes. The patient underwent a T11-L2 laminectomy for a gross total tumor resection. Histology revealed a World Health Organization grade II GTNI. CONCLUSIONS: GTNI is a rare type of glioneuronal tumor that has recently been described in the literature. The outcome of this case seems to have an unfavorable clinical course despite their low-grade morphology. However, the combination of gross total resection and adjuvant chemo-radiotherapy can enhance chances for longer survival among children with spinal GTNI associated with meningeal dissemination, and a clinical follow-up of a large series will be necessary to evaluate the long-term prognosis.