Aggressive enterogenous cyst with extensive craniospinal spread: Case report

OBJECTIVE AND IMPORTANCE: Considered benign and malformative in nature, ent erogenous cysts are predominantly spinal lesions. Although recurrences have been reported, especially in the rare intracranial examples, craniospinal dissemination has never been described. CLINICAL PRESENTATION: We report a 63-year-old woman who presented 16 years previously with a histologically typical enterogenous cyst of the cerebell um. Fourteen years after its incomplete excision, numerous supratentorial, infratentorial, and spinal subarachnoid cysts were detected. A histological examination revealed multiple enterogenous cysts without evidence of malig nancy. Ki-67 (MIB-1) proliferative indices were less than 1% in all except one specimen, wherein it was 4%; immunohistochemistry demonstrated rare p53 protein overexpression. INTERVENTION: During the last 2 years, four subtotal resections of spinal e nterogenous cysts have been performed for progressive paraplegia, pain, par esthesia, and bladder dysfunction. Considering the multiple recurrences and progressive neurological deficits, craniospinal radiation therapy was give n. CONCLUSION: To our knowledge, this is the first report of widespread cranio spinal dissemination associated with the incomplete resection of an enterog enous cyst. Although a rare complication, it emphasizes the advantages of e arly, aggressive surgery.