Isolated fourth ventricle craniopharyngioma: Representative case illustration and review of literature

Sella and suprasellar areas are frequently affected by craniopharyngiomas. In this article, eight occurrences have been documented. One is new, and the remaining seven are from previously published articles. Their prevalence in the posterior fossa without expansion from the suprasellar area is unusual. We present a case of a primary 4th ventricular craniopharyngioma of the posterior fossa in a 16-year-old male with no association with Gardner’s syndrome. He presented with sudden deterioration of consciousness level and was diagnosed as having a homogeneously contrast enhancing lesion occupying the 4th ventricle with obstructive hydrocephalus. The patient underwent emergency placement of external ventricular drain followed by complete removal of the tumor on the next day by midline suboccipital craniotomy with telovelar approach. Histopathological features were consistent with the adamantinomatous variety of craniopharyngioma. He had complete neurological recovery and no evidence of tumor recurrence in 1-year follow-up. The craniopharyngioma in our case was distinct because it was a solid tumor with no cystic component, exhibited homogeneous contrast enhancement in neuroimaging, developed in the fourth ventricle, and reached the level of foramen magnum, features that had rarely been documented previously. We also reviewed the literature on reported cases of 4th ventricular craniopharyngioma to strengthen knowledge in this area and highlight the embryological basis of ectopic craniopharyngioma.