Dysplastic cerebellar gangliocytoma and Cowden syndrome

Abstract

Objective To describe the clinical features, diagnosis, treatment and the association between dysplastic cerebellar gangliocytoma (Lhermitte- Duclos disease, LDD) and Cowden Syndrome.Methods Out of 7 LDD patients admitted to Tiantan Hospital from August 2001 to December 2009, 6 patients had undergone tumor resection.Medical records and radiological images of each case were reviewed.Follow - up period ranged from 5 months to 105 months.Results MRI images of all patients were unique to make the diagnosis of LDD preoperatively.Tumor removal was performed in 6 out of 7 patients.Ventriculoperitoneal shunt without tumor removal was performed for one patient.Tumor removal was gross total in 5 patients, subtotal in one patient.4 out of 6 adult LDD patients had diseases which could be found in Cowden Syndrome such as facial multiple macula or papules, acral keratoses, fibroblastic disease, endometrial carcinoma, uterine fibroid, et al.One patient died of complication of diabetes mellitus two years later.Conclusions MRI images of LDD are unique for preoperative diagnosis in almost every patient Tumor removal is the only effective treatment method.Most patients could recover well postoperatively.For adult LDD patients, the possibility combined with Cowden syndrome is very great.Patients need thorough examinations to rule out other diseases of Cowden syndrome. Key words: Lhermitte- Duclos disease; Cowden syndrome; Magnetic resonance imaging; Neurosurgical procedures