Sylvian Fissure Desmoplastic Infantile Ganglioglioma: A Case Presentation and Review of Literature


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Abstract

Childhood tumors arising from the Sylvian fissure are extremely rare and provide not only a diagnostic imaging challenge in localization and characterization, but also complicate neurosurgical resection. Magnetic resonance imaging plays an important role in characterizing these tumors and forms an important adjunct in preoperative and perioperative neurosurgical planning. Desmoplastic infantile ganglioglioma (DIG) is a rare distinct clinico-pathologic entity with a predominantly benign prognosis that usually presents in the first 18 months of life. These tumors are invariably supratentorial in location but none have been described in the current literature to arise within the Sylvian fissure. The clinical course, imaging, and histologic/immunohistochemical features of a 3-month-old boy presenting with a Sylvian fissure DIG are described. Additionally, the authors present a literature review of DIGs and Sylvian fissure tumors. Although rare, the characteristic imaging findings of the DIG despite arising from a previously undescribed location of origin help confirm the diagnosis, resulting in a complete resection of the tumor with a favorable prognostic outcome.

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