Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review

Khandpur, Umang; Huntoon, Kristin; Smith-Cohn, Matthew; Shaw, Andrew; Elder, J. Bradley

doi:10.1016/j.wneu.2019.03.131

Cited by 22 publications

(23 citation statements)

References 45 publications

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“…4 Accompanied with megalencephaly, syringomyelia, polydactyly, cranial asymmetry and mucocutaneous lesions, LDD is presented with Cowden syndrome that is characterized by autosomal dominantly-inherited hereditary hamartoma and neoplasia. 9 Our patient did not have a history of cancer and skin lesions. LDD diagnosis is characterized by a tiger-striped pattern caused by abnormal cerebellar thickening that is hyperintense and hypointense on T2-and T1-weighted scans of cranial MRI, respectively.…”

Section: Discussionmentioning

confidence: 80%

“…4 As in our case, recurrence is observed postoperatively, although it is rare and benign. 9 In addition, there are cases that do not present any clinical findings and are followed-up without surgery. 2,6 In our case, the location of the lesion at the cerebellopontine angle, which is very rare, led to the consideration of the pathologies of this region.…”

Section: Discussionmentioning

confidence: 99%

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Lhermitte–Duclos Disease Mimicking Cerebellopontine Angle Tumor: A Case Report

Abbasoğlu

Zaimoğlu

Eroğlu

et al. 2021

Indian Journal of Neurosurgery

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Lhermitte-Duclos disease (LDD) is dysplastic gangliocytoma of the cerebellum, which is rare, tends to grow slowly, usually has good prognosis, and wherein the cerebellar cortex becomes thick and the central white matter is not observed. On MRI of the brain, it has hyperintense and hypointense presentations on T2- and T1-weighted scans, respectively. It has nonhomogenous contrasting pattern on T1-contrast scans. LDD can be seen unilaterally or bilaterally in the cerebellum. When the disease is located at the cerebellopontine angle, it can be mistaken for the tumors located in this region. Recurrence can occur postoperatively, which is rare. The present case is a rare one because the tumor was located at the cerebellopontine angle and radiologically mimicked cerebellopontine angle tumors.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Lhermitte–Duclos Disease Mimicking Cerebellopontine Angle Tumor: A Case Report

Abbasoğlu

Zaimoğlu

Eroğlu

et al. 2021

Indian Journal of Neurosurgery

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“…However, assuming the non-neoplastic etiology of LDD, irradiation is unlikely beneficial, and, therefore, it is not recommended-even in subtotal resections. 19,23 Moreover, in the histopathological examination, which is the gold standard for diagnosis, the white mass atrophy and the granular cell layer thickening can be observed. 2,6 The absence of the Purkinje, the laminar cytoarchitecture destruction of the cerebellar cortex, and the presence of hypertrophic and dysplastic neurons in the internal granular layer are common findings.…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

et al. 2019

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Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group of genetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are considered major criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.

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“…24,25 Lhermitte-Duclos disease grows slowly and resection is often curative, with a few cases of recurrence. 3,26 Jiang et al 27 reported that 18 patients with Lhermitte-Duclos disease received total or subtotal lesion resection in a single institute, and no patient had lesion recurrence during an average of 52 months of follow-up. In addition, as stated by Wang et al, 11 experienced postoperative recurrence.…”

Section: Surgical Strategymentioning

confidence: 99%

“…5 Computed tomography (CT) may show a nonspecific hypoattenuating cerebellar mass in the posterior fossa, although calcification is occasionally seen. 3,6 Magnetic resonance imaging (MRI) is considered a technique of choice for diagnosis of Lhermitte-Duclos disease, producing widened cerebellar folia with the characteristic striated pattern cerebellum (also described as tigroid appearance) in T2-weighted images. [7][8][9][10] With both imaging techniques, hydrocephalus is demonstrated frequently.…”

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confidence: 99%

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

Li¹,

Guo²,

Wei³

et al. 2021

Journal of Neurosurgery: Case Lessons

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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.

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Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review

Cited by 22 publications

References 45 publications

Lhermitte–Duclos Disease Mimicking Cerebellopontine Angle Tumor: A Case Report

Lhermitte–Duclos Disease Mimicking Cerebellopontine Angle Tumor: A Case Report

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

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

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