Unusual Occurrence of Multifocal Desmoplastic Infantile Astrocytoma: A Case Report and Review of the Literature

Abstract

Desmoplastic infantile gliomas are rare, benign tumors of the early infancy period. Two histological subtypes - desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma - have been described. The characteristic features of DIAs are lobar location, glial histology, and excellent prognosis after complete surgical excision. DIAs usually present as solitary, cortical-surfacing, solid-cystic neoplasms; however, atypical, aggressive, and multifocal variants of DIA have been reported in the literature. These rare DIAs presenting with multiple lesions pose a diagnostic as well as a therapeutic dilemma. We report an unusual case of an 8-month-old female infant diagnosed with multifocal (cranial and spinal) DIA and obstructive hydrocephalus and discuss the radiological and histological features of this rare variant of DIA. The patient underwent ventriculoperitoneal shunt placement to relieve the hydrocephalus, excisional biopsy from a surfacing lesion in the right frontal lobe, and multiple cycles of chemotherapy; however, the lesions continued to progress, and the patient is likely to have an unfavorable outcome.

Keywords: Chemotherapy; Desmoplastic infantile astrocytoma; Desmoplastic infantile glioma; Infantile brain tumors; Multifocal localization; Pathology; Treatment.