Epidermoid cysts of the pineal region

Localization of epidermoid cysts to the pineal region is rare. The 7-year-o ld boy now reported presented with an 18-month history of progressive ataxi a. CT and MRI scans demonstrated a 2.5 x 2.5 cm cyst at the pineal region w ith obstructive hydrocephalus. At surgery via an occipital transtentorial a pproach, a characteristic "pearly tumour" was encountered, and complete res ection was achieved. We present the management of this child with pineal re gion epidermoid cyst and review 11 cases reported in the literature since 1 968. In all, 8 of the 12 patients were males. The age at the time of diagno sis ranged from 7 years to 69 years. Parinaud's syndrome and hydrocephalus are the most common presenting findings. All but 1 patient underwent direct surgical resection; 1 had stereotactic decompression. Surgical treatment b rought about complete resolution of the presenting symptoms and signs in 10 of the 12 cases. One patient had persistent upgaze palsy. One patient died from progression of the pineal region mass. This patient presented with he miparesis, which is a marker of clinical aggressiveness. The authors advoca te direct surgical attack as opposed to stereotactic diagnosis and aspirati on to: (1) obtain maximal resection and thereby limit the potential for rec urrence and delayed complications of the cyst; (2) possibly avoid shunt pla cement in patients who present with hydrocephalus; and (3) decrease the lik elihood of sampling error.