Intradural extracerebral choristoma. Case report and review of the literature.

Abstract

BACKGROUND: Intracranial extracerebral neuroglial choristoma is a very rare entity. A cleft palate or a nasopharyngeal teratoma has been described as accompanying choristoma, but there are no observations of both anomalies being simultaneously present and combined.

METHODS: An 8-month-old girl presented with seizures, without evidence of any other neurological abnormalities. Brain magnetic resonance imaging demonstrated a 2.0-cm-sized, round-shaped mass in the frontal midline. The T(1)- and T(2)-weighted images showed a well-circumscribed iso-signal intensity lesion with no gadolinium enhancement.

RESULTS: Surgical exploration revealed a yellowish tumor mass with whitish soft tissue material within the tumor, adherence to the surrounding blood vessels, and therefore subtotal removal was performed. Pathologic examination was neuroglial choristoma with 1.5% of MIB-1 labeling index. The patient also had a cleft palate with a pinkish palatal mass and underwent repair of the cleft palate, and excision of mass that was a mature cystic teratoma on pathologic examination. Follow-up at 18 months later showed that there was no evidence of tumor regrowth or neurological deficit, and the neurological development was normal.

CONCLUSION: The authors described a rare case of intracranial extracerebral neuroglial choristoma accompanied by both nasopharyngeal teratoma and cleft palate and suggested its benign nature from the low MIB-1 labeling index.