Abstract
RATIONALE: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features. PATIENT CONCERNS: In this article, we report two cases of intracranial medulloepitheliomas. In the first patient, a one-month old boy, the tumor was located in in right lateral ventricle, which was the first report of such location of this disease; in the second patient, an eleven-month old girl, the tumor was in right cerebellopontine angle. DIAGNOSES: Both patients were diagnosed as medulloepithelioma by pathologists. INTERVENTIONS: Both patients underwent craniotomy to resect the lesion totally. OUTCOMES: The boy underwent chemotherapy after operation and was alive 3 months after operation. The girl died 6 months after operation, despite aggressive adjuvant chemotherapy. LESSONS: Surgical resection is safe and effective to prolong patient survival. However, despite aggressive adjuvant therapy, prognosis of medulloepithelioma remains poor, and further study is needed to improve treatment of this rare disease.