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

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.

Lhermitte-Duclos disease in a 51-year old patient

“…Low rates of postoperative recurrence are associated with complete surgical resection [10] . A previous study reported a 31-year-old patient with LDD who presented with symptoms of increased intracranial cranial pressure and was managed surgically [19] . However, our patient improved symptomatically with antiepileptic medication in the emergency department, and her admission course was uneventful.…”

Lhermitte-Duclos disease with excessive calcification in a septuagenarian: A case report

“…[ 7 , 8 , 15 , 16 ] Remarkably, the lesion often slow growing in nature with unclear boundaries with the adjacent cerebellum making total resection difficult, if not impossible. [ 16 , 17 ] Nevertheless, total resection of the tumor is still considered the most anticipated surgical strategy. [ 16 , 18 ] However, some authors advocate both maximal safe resection as well as preventive C1 laminectomy.…”

“…[ 16 , 18 ] However, some authors advocate both maximal safe resection as well as preventive C1 laminectomy. [ 17 ] Interestingly, intraoperative ultrasound has been used to determine the exact location, specified the lesion resection range, as well as analyzed the blood supply and the venous sinus patency aiding in accurate resection of the lesion. [ 17 ] We attained total resection of the lesion in our case with no neurological deficits.…”

Lhermitte-Duclos disease: A series of six cases

“…Hence, subtotal and partial resection of LDD may be acceptable due to its remarkably slow-growing nature. [15] Radiation therapy has been reported as non-effective in avoiding the growth of the lesion. [2,9] Prognosis is favorable and mortality is low, though recurrences may occur due to subtotal resection.…”

“…Hence, subtotal and partial resection of LDD may be acceptable due to its remarkably slow-growing nature. [ 15 ] Radiation therapy has been reported as non-effective in avoiding the growth of the lesion. [ 2 , 9 ]…”