Dysplastic gangliocytoma of the cerebellum: Rare differential diagnosis in space occupying lesions of the posterior fossa

Buhl, Ralf; Barth, Harald; Hugo, Heinz-Hermann; Straube, Torsten; Mehdorn, H. M.

doi:10.1007/s00701-003-0040-3

Cited by 25 publications

(7 citation statements)

References 19 publications

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“…Recent published data suggest that LDD is a hamartomatous lesion related to a phakomatosis, rather than a neoplasm. 2 LDD is often asymptomatic in early life, but usually becomes clinically apparent in the third and fourth decades. Reported cases have presented from birth to 74 years old.…”

Section: Introductionmentioning

confidence: 99%

CT and MRI findings in Lhermitte-Duclos disease

Bayat

Sarawan

2005

S. Afr. j. radiol.

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Section: Introductionmentioning

confidence: 99%

CT and MRI findings in Lhermitte-Duclos disease

Bayat

Sarawan

2005

S. Afr. j. radiol.

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“…GFAP expression is restricted to reactive astrocytes that may occupy the involved cortex and white matter. 1,[5][6][7]13,16,[18][19][20] Differential diagnosis includes gangliocytoma, which determines a nodular tumoral mass, ganglioma, which exhibits a complex architecture and a solid / cystic lesion, and an infiltrating glioma with trapped Purkinje cells that is composed by atypical glia and presence of mitoses. Surgical resection is usually curative, but recurrence is not uncommon in long-term follow-up.…”

Section: Discussionmentioning

confidence: 99%

“…Surgical resection is usually curative, but recurrence is not uncommon in long-term follow-up. 1,7,9,12,14,16,18,[20][21][22] Conflict of Interests The authors declare that there are no conflict of interests.…”

Section: Discussionmentioning

confidence: 99%

A rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior - Dysplastic Gangliocytoma of the Cerebellum: Case Report

Almeida

Schiavo

Santos

et al. 2020

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.

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“…Lhermitte-Duclos disease (LDD) is a rare condition usually affecting patients aged 30-50 years, though it may occur in infants or adults over 60 years. There is no sex (M:F=1) or race preference 6,7 . Lhermitte and Duclos reported the first case of cerebellar ganglion cell tumour in 1920.…”

Section: Discussionmentioning

confidence: 99%

“…There is considerable controversy over the cause of this disease: it may have a hamartomatous, neoplastic, or congenital malformative origin 5,7 . The condition is often associated with different types of malformation such macrocephaly, megacephaly, syringomyelia, polydactyly, multiple haemangiomas and mucocutaneous lesions as well as breast, thyroid, genitourinary and gastrointestinal malignancies 2 .…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos Disease

et al. 2013

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Lhermitte-Duclos disease is a rare pathologic condition consisting of a dysplastic gangliocytoma of the cerebellum. Its association with phacomatosis and an autosomal dominant neoplastic syndrome, Cowden's syndrome is also known. Modern neuroimaging contributes to a correct diagnosis and pre- and postoperative evaluation. Here we describe the morphologic and metabolic aspects of the disease as shown by conventional MRI, diffusion imaging and spectroscopy in a 31-year-old woman. In addition, the specific neuroradiologic characteristics are presented and discussed in the light of the main pathologic and clinical features, such as hypertrophy of the cerebellar folia associated with white matter atrophy.

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Dysplastic gangliocytoma of the cerebellum: Rare differential diagnosis in space occupying lesions of the posterior fossa

Cited by 25 publications

References 19 publications

CT and MRI findings in Lhermitte-Duclos disease

CT and MRI findings in Lhermitte-Duclos disease

A rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior - Dysplastic Gangliocytoma of the Cerebellum: Case Report

Lhermitte-Duclos Disease

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