Revisão Revisado por pares

Clinical Heterogeneity of Desmoplastic Infantile Ganglioglioma

2012; Lippincott Williams & Wilkins; Volume: 34; Issue: 6 Linguagem: Inglês

10.1097/mph.0b013e3182580330

ISSN

1536-3678

Autores

Trent R. Hummel, Lili Miles, Francesco T. Mangano, Blaise V. Jones, James I. Geller,

Tópico(s)

Neuroblastoma Research and Treatments

Resumo

Desmoplastic infantile gangliogliomas (DIG) are intracranial tumors described in 1987 as benign lesions of infancy. A literature review and the clinical course of 3 patients reported herein suggest that the initial description should be amended. Nearly 23% of DIG cases occur in children older than 24 months. Approximately 40% of DIG cases require additional medical, radiation, and/or further surgical intervention, and 15% of infants and children develop leptomeningeal spread or die from DIG. Such adverse outcomes, combined with the recognition that DIG represents a heterogeneous disease, underscore the need for an expanded biological and molecular investigation.

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