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dysplastic gangliocytoma: A Rare Example of Cerebellar Tumor with An Evident Genetic Profile (Cowden Syndrome)

Open Journal of Pathology 2021年第2期11卷 33-37页

作者： Javier Ortiz Rodríguez-Parets Luis Miguel Chinchilla Tábora Enrique Montero Mateos Elisa Muñoz Torres María Dolores Ludeña de la Cruz Department of Pathology University Hospital of Salamanca (Spain) Salamanca Spain Department of Cell Biology and Pathology The Medical School University of Salamanca Salamanca Spain

Objective: To describe the histological and immunohistochemical findings observed when studying a dysplastic cerebellar gangliocytoma observed in a 33-year-old man with a history of Thyroid Cancer. Material and Methods: Radiological images (MRI) and histological and immunohistochemical preparations obtained from the cerebellar tissue were received in our laboratory. Results: A neoplasm constituted by aberrant-looking mature neurons was observed that showed negativity for the immunohistochemical markers of the PTEN protein, expression of the activity of the PTEN gene. Conclusions: Verify the diagnosis of a rare entity, clearly related in scientific publications with Cowden’s Syndrome.

关键词： dysplastic gangliocytoma Immunohistochemistry Cowden Syndrome

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Acute Hydrocephalus Revealing Unusual Cerebellar Mass: dysplastic Cerebellar gangliocytoma or Lhermitte-Duclos Disease (LDD)

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Neuroscience & Medicine 2013年第3期4卷 161-165页

作者： C.Karekezi M.Boutarbouch O.Coulibaly S.Derraz A.El Ouahabi A.El Khamlichi Department of Neurosurgery Mohamed Vth UniversitySchool of MedicineCHU IBN SINAHopital des Spécialités ONORabatMorocco.

Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the cerebellum is a rare benign lesion of uncertain pathogenesis characterized by overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells;this results in gross thickening of the cerebellar folia. It is revealed by symptoms of raised intracranial pressure, cerebellar impairment and obstructive hydrocephalus. We reported the case of a 41-year-old male who presented with symptoms of acute raised intracranial pressure. Brain computed tomography (CT) scan revealed hydrocephalus due to compression of the 4th ventricle by a large non-enhancing left cerebellar mass. Magnetic resonance imaging (MRI) showed a space-occupying lesion within the left cerebellar hemisphere with unusual striation. Radical surgery was retained though the margins with normal cerebellar tissue were not distinct. Clinical complications after gross total or partial removal of Lhermitte-Duclos lesions have been rarely reported in the literature;herein we stress the importance of extreme caution in removing these lesions in cerebellar areas that have no distinct margins between the lesion and normal tissue.

关键词： Lhermitte-Duclos Disease dysplastic gangliocytoma MRI Cerebellar Tiger Stripes

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