Clinical and pathological study of dysplastic gangliocytoma

Abstract

A case of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is reported. Computerized tomography revealed a nonenhancing mass lesion surrounded by areas of calcification. Surgical excision resulted in complete resolution of the patient's symptoms. The histological findings support the concept that this tumor represents a congenital abnormality in granule-cell migration and is not a true neoplasm.

Uncommon glial tumors

“…In patients with epilepsy, surgical treatment commonly significantly reduces or eliminates seizures. As would be expected from CT and MR, the tumor on angiography is an avascular mass (Roski et al 1981;Sabin et al 1988;Faillot et al 1990;Murata et al 1999). Gangliocytomas associated with endocrine dysfunction may require medical management of the endocrinopathy (McCowen et al 1999;Isidro et al 2005).…”

Lhermitte-Duclos disease associated to Cowden syndrome: de novo diagnosis and management of these extremely rare syndromes in a patient

“…However, complete resection may be difficult because of poorly defined margins, leading to a high risk of recurrence. For this reason and given the slowly-growing nature of LDD, a long-term follow-up is required 30 31. However, our case highlights that prompt and aggressive surgical treatment can prevent significant visual loss, normalise the peripapillary retinal nerve fibre layer and preserve the visual field.…”

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

“…[7][8][9] In a few instances, patients with Lhermitte-Duclos disease have undergone radiation therapy, but the effect of posterior fossa irradiation has been variously reported to either result in several symptomfree years 31 or progressive neurological deterioration. 14 Decompressive surgery for symptomatic patients has been successful both in relieving symptoms 9,12,25,32 and in improving long-term survival from 2.5 to 11 years. 12,13,30,32 The lesion usually blends into normal cerebellar parenchyma.…”

“…[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] The recognition of the coexistence of Lhermitte-Duclos disease and multiple hamartoma-neoplasia syndrome, also called Cowden syndrome, by Padberg et al 17 suggested a genetic association between the two entities. 1 Today Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is still a highly unusual and controversial condition.…”