Desmoplastic infantile astrocytoma with multiple intracranial and intraspinal localizations at presentation.

PURPOSE: Desmoplastic infantile gliomas (DIG) are rare tumors that occur in infants aged between 1 and 24 months. The tumor in general has a favorable prognosis after surgical resection. There are no treatment algorithms, however, for patients with multiple intracranial and intraspinal presentations.

CASE REPORT: In an 11-month-old girl with a history of nystagmus, magnetic resonance imaging (MRI) demonstrated contrast-enhancing lesions in the suprasellar region, the cerebellar vermis, and the spinal axis. The tumor in the cerebellar vermis was removed via a suboccipital midline approach. The histological examination revealed a desmoplastic infantile astrocytoma (DIA) WHO grade I. Postoperatively, it was decided to adopt a wait-and-see strategy. Further development, up to 16 months after surgery, was unremarkable. Follow-up MRI showed no recurrence of the posterior fossa tumor, mild progress of the suprasellar tumor, and significant regression of the spinal tumors.

CONCLUSION: DIA is a rare mostly benign brain tumor found in infants. The final diagnosis always relies on histology. Surgical resection is the recommended therapy for symptomatic tumors; however, more experience is needed to develop treatment recommendations for multiple-site tumors.