Desmoplastic infantile astrocytoma with metastases at presentation

A 4-month-old child presented with nystagmus and macrocephaly. He had a large tumor in the suprasellar and hypothalamic region, as well as two smaller similar masses in the posterior fossa and one in the spinal canal. A biopsy of the suprasellar mass revealed it to be a desmoplastic infantile cerebral astrocytoma. Cerebrospinal fluid obtained at surgery before tumor manipulation showed clusters of malignant cells immunopositive for glial fibrillary acidic protein. In our opinion, the smaller tumors were metastases from the large suprasellar primary astrocytoma. Review of all of the previously reported cases of desmoplastic infantile cerebral astrocytoma and of the related neoplastic entity desmoplastic infantile ganglioglioma suggested that this was a unique case, but we still recommend caution with respect to the previously accepted notion that desmoplastic infantile neuroepithelial tumors are virtually benign neoplasms.