<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Objective:</strong> To describe the histological and immunohistochemical findings observed when ...<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Objective:</strong> 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. <strong>Material and Methods:</strong> Radiological images (MRI) and histological and immunohistochemical preparations obtained from the cerebellar tissue were received in our laboratory. <strong>Results:</strong> 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. <strong>Conclusions:</strong> Verify the diagnosis of a rare entity, clearly related in scientific publications with Cowden’s Syndrome.</span> </div>展开更多
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 ...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.展开更多
Objectives: Sellar gangliocytomas are extremely rare. Since they present clinically and radiologically as pituitary adenomas, the preoperative diagnosis of these mixed tumors is very difficult. Here, we report three c...Objectives: Sellar gangliocytomas are extremely rare. Since they present clinically and radiologically as pituitary adenomas, the preoperative diagnosis of these mixed tumors is very difficult. Here, we report three cases of gangliocytoma combined with pituitary adenoma and describe our findings. Methods: The clinical data of the three cases of gangliocytoma combined with pituitary adenoma have been retrospectively analyzed, and the published literature has also been reviewed. Results: All three patients underwent pure endonasal endoscopic surgery, and no recurrence was observed over a follow-up of at least 30 months. Growth hormone(GH)-hypersecreting adenoma and tumor calcification were detected in these mixed tumors. Conclusions: Pure endoscopic transnasal transsphenoidal surgery may be an effective way for the treatment of this kind of tumor. Gross total resection of the tumor is recommended. In addition, calcification with GH-hypersecretion may serve as a preoperative diagnostic clue for gangliocytoma in the sella turcica.展开更多
Lhermitte-Duclos disease (LDD), also called dysplastic gangliocytoma of the cerebellum, is a rare condition described in 1920. It represents a disorganization of cerebellar architecture with overgrowth of cerebellar g...Lhermitte-Duclos disease (LDD), also called dysplastic gangliocytoma of the cerebellum, is a rare condition described in 1920. It represents a disorganization of cerebellar architecture with overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells. In this report we present the case of a 62-year-old male affected by this disease, as well as literature review of the clinical, morphological and functional radiological findings.展开更多
文摘<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Objective:</strong> 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. <strong>Material and Methods:</strong> Radiological images (MRI) and histological and immunohistochemical preparations obtained from the cerebellar tissue were received in our laboratory. <strong>Results:</strong> 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. <strong>Conclusions:</strong> Verify the diagnosis of a rare entity, clearly related in scientific publications with Cowden’s Syndrome.</span> </div>
文摘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.
基金the Youth Fund of Beijing Tiantan Hospital(Grant No.2015-YQN-10)
文摘Objectives: Sellar gangliocytomas are extremely rare. Since they present clinically and radiologically as pituitary adenomas, the preoperative diagnosis of these mixed tumors is very difficult. Here, we report three cases of gangliocytoma combined with pituitary adenoma and describe our findings. Methods: The clinical data of the three cases of gangliocytoma combined with pituitary adenoma have been retrospectively analyzed, and the published literature has also been reviewed. Results: All three patients underwent pure endonasal endoscopic surgery, and no recurrence was observed over a follow-up of at least 30 months. Growth hormone(GH)-hypersecreting adenoma and tumor calcification were detected in these mixed tumors. Conclusions: Pure endoscopic transnasal transsphenoidal surgery may be an effective way for the treatment of this kind of tumor. Gross total resection of the tumor is recommended. In addition, calcification with GH-hypersecretion may serve as a preoperative diagnostic clue for gangliocytoma in the sella turcica.
文摘Lhermitte-Duclos disease (LDD), also called dysplastic gangliocytoma of the cerebellum, is a rare condition described in 1920. It represents a disorganization of cerebellar architecture with overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells. In this report we present the case of a 62-year-old male affected by this disease, as well as literature review of the clinical, morphological and functional radiological findings.
