Neuroglial choristoma presenting as congenital lid tumor

A 12-month-old boy presented with a diffuse solid tumor of the nasal portions of the lower lid, which had caused almost complete narrowing of the interpalpebral fissure since birth. Clinically, it was suspected to be a mesenchymal tumor. Radiological studies revealed no bony defects in the orbit. The computerized tomographic (CT) scan disclosed a porencephalic cyst in the ipsilateral cerebral hemisphere. Excisional biopsy of the lid tumor revealed ectopic neuroglial tissue. The immunocytochemical demonstration of glial fibrillary acidic protein (GFAP) was consistent with the electron microscopic findings that disclosed fibrillary astrocytes with an abundance of typical 9- to 10-nm glial filaments. Heterotopic neuroglial tissue should be part of the differential diagnosis of congenital lid tumors; its occurrence may be associated with other anomalies of cerebral organogenesis.