ABSTRACT
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.
Subject(s)
Brain Neoplasms , Central Nervous System Neoplasms , Meningeal Neoplasms , Female , Humans , Child , Meningeal Neoplasms/pathology , Central Nervous System Neoplasms/pathologyABSTRACT
PURPOSE: Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature. CASE ARTICLE: In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course.
