Literature DB >> 36131570

The surgical resection of dysplastic cerebellar gangliocytoma assisted by intraoperative sonography: illustrative case.

Yiming Li1, Jiahe Guo1, Huijie Wei1, Cuiyun Sun1,2, Yan Chai3, Xiuwei Fu4, Kai Zhang5, Shengping Yu1, Xuejun Yang1.   

Abstract

BACKGROUND: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is an extremely rare, slow-growing hereditary mass lesion that is mainly characterized by both specific neuroradiological features and secondary hydrocephalus. Patients may present with symptoms of cerebellar mass lesion and increased intracranial pressure. As an important part of Cowden syndrome, Lhermitte-Duclos disease in adults is typically marked by PTEN gene mutation. OBSERVATIONS: The clinical management of a 31-year-old woman who suffered Lhermitte-Duclos disease was introduced in this case report. Subtotal resection was performed with the assistance of intraoperative sonography to relieve obstructive hydrocephalus, and prophylactic C1 laminectomy was performed to prevent possible postoperative progression of the residual lesion. Perioperative care and surgical process were clearly revealed in an accompanying video. Intraoperative sonography of Lhermitte-Duclos disease presents hyperechoic distorted thickening cortices surrounded by hypoechoic edema belt. The patient did not report any significant neurological complications or sequelae after the lesion resection. LESSONS: The authors first reported the use of intraoperative sonography in resection of adult-onset Lhermitte-Duclos disease. Hopefully, the educative case report can provide a feasible experience in the diagnosis and treatment of Lhermitte-Duclos disease.

Entities:  

Keywords:  Cowden syndrome; Lhermitte-Duclos disease; PTEN mutation; dysplastic gangliocytoma of the cerebellum; intraoperative sonography; surgical strategy

Year:  2021        PMID: 36131570      PMCID: PMC9563950          DOI: 10.3171/CASE21451

Source DB:  PubMed          Journal:  J Neurosurg Case Lessons        ISSN: 2694-1902


  34 in total

Review 1.  Recurrent Lhermitte-Duclos disease: report of two cases and association with Cowden's disease.

Authors:  D W Williams; A D Elster; L E Ginsberg; C Stanton
Journal:  AJNR Am J Neuroradiol       Date:  1992 Jan-Feb       Impact factor: 3.825

Review 2.  The value of intraoperative ultrasound in neurosurgery.

Authors:  J C Sutcliffe
Journal:  Br J Neurosurg       Date:  1991       Impact factor: 1.596

3.  Updated Imaging Features of Dysplastic Cerebellar Gangliocytoma.

Authors:  Radhika Dhamija; Christopher P Wood; Alyx B Porter; Leland S Hu; Steven M Weindling; Joseph M Hoxworth
Journal:  J Comput Assist Tomogr       Date:  2019 Mar/Apr       Impact factor: 1.826

4.  Central positional nystagmus associated with cerebellar tumors: Clinical and topographical analysis.

Authors:  Bang-Hoon Cho; Sang-Hoon Kim; Sung-Sik Kim; Yun-Ju Choi; Seung-Han Lee
Journal:  J Neurol Sci       Date:  2016-12-27       Impact factor: 3.181

Review 5.  The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.

Authors:  David N Louis; Arie Perry; Guido Reifenberger; Andreas von Deimling; Dominique Figarella-Branger; Webster K Cavenee; Hiroko Ohgaki; Otmar D Wiestler; Paul Kleihues; David W Ellison
Journal:  Acta Neuropathol       Date:  2016-05-09       Impact factor: 17.088

6.  Lhermitte-Duclos disease: Clinical study with long-term follow-up in a single institution.

Authors:  Qiguang Wang; Si Zhang; Jian Cheng; Wenke Liu; Xuhui Hui
Journal:  Clin Neurol Neurosurg       Date:  2017-09-09       Impact factor: 1.876

7.  Lhermitte-Duclos disease and Cowden disease: a single phakomatosis.

Authors:  G W Padberg; J D Schot; G J Vielvoye; G T Bots; F C de Beer
Journal:  Ann Neurol       Date:  1991-05       Impact factor: 10.422

8.  Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease).

Authors:  N G Rainov; H J Holzhausen; W Burkert
Journal:  Clin Neurol Neurosurg       Date:  1995-05       Impact factor: 1.876

9.  Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology.

Authors:  B Thomas; T Krishnamoorthy; V V Radhakrishnan; C Kesavadas
Journal:  Neuroradiology       Date:  2007-06-05       Impact factor: 2.804

10.  Characterization of cryptic splicing in germline PTEN intronic variants in Cowden syndrome.

Authors:  Hannah Jinlian Chen; Todd Romigh; Kaitlin Sesock; Charis Eng
Journal:  Hum Mutat       Date:  2017-07-17       Impact factor: 4.878

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