Intramedullary ependymoma associated with Lhermitte-Duclos disease and Cowden syndrome.
The authors describe the case of a 45-year-old man with progressive gait ataxia and sensorimotor deficits of the upper and lower extremities. The patient had been diagnosed earlier with Lhermitte-Duclos disease (LDD) in the left cerebellar hemisphere and Cowden syndrome (CS). MR imaging studies revealed an intraspinal tumor at C6-C7. Microsurgical gross total resection of the tumor was achieved. Histolopathological examination revealed an intramedullary ependymoma. Postoperatively, neurological deficits gradually improved. This is the first reported case of ependymoma in a patient with LDD and CD. Coexistence of an intraspinal ependymoma with cerebellar LDD and CS appears to be rare, but can lead to treatment failure if missed.
Farhadi
Mohammad R
MR
Department of Neurosurgery, Medical School Hannover, MHH, Carl-Neuberg street 1, 30625 Hannover, Germany.
Rittierodt
Marion
M
Stan
Alexandru
A
Capelle
Hans-Holger
HH
Tham-Mücke
Barbara
B
Krauss
Joachim K
JK
D002574:Cervical Vertebrae; D004806:Ependymoma / Q000209:etiology* / Q000473:pathology* / Q000601:surgery; D006223:Hamartoma Syndrome, Multiple / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013120:Spinal Cord Neoplasms / Q000209:etiology* / Q000473:pathology* / Q000601:surgery