Dysplastic cerebellar gangliocytoma: a description of two cases

Wan, Xincui; Chu, Hong-Juan; Chen, Wangsheng; Wang, Fei

doi:10.21037/qims-20-627

Cited by 4 publications

(4 citation statements)

References 8 publications

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“…which are associated with abnormal vessels and areas of calcification. [12,13] In spite of these developments in the imaging modalities which have made pre-operative diagnosis of LDD easier, histopathology is still required for definitive diagnosis. Grossly, it is pale ill-circumscribed lesion and shows enlargement of cerebellar cortex on cut section.…”

Section: Discussionmentioning

confidence: 99%

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Lhermitte-Duclos disease: A series of six cases

Kolhe

Shenoy

Tayal

et al. 2023

JNRP

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The Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare lesion characterized by variable enlargement of cerebellar folia. The pathological basis of LDD has long been debated, as it has overlapping features of both, a neoplasm and hamartoma. Association between LDD and Cowden syndrome (CS) has been established based on the presence of phosphatase and tensin homologue germline mutation in both. We present a series of six cases of LDD: Four females and two males, aged between 16 and 38 years, presenting with headache and imbalance on walking of 1–7 months duration. Histomorphology showed thickening and vacuolation of the molecular layer, loss of Purkinje cells, and replacement of granular cell layer by large dysplastic ganglion cells. Awareness of histological features of this rare entity and a higher level of suspicion is required for the correct diagnosis, which, in turn, should prompt thorough investigations to exclude features of associated CS. LDD is a rare entity, awareness of its histological features and correlating them with radiology is essential, especially in tiny biopsies; to render the correct diagnosis. Diagnosis of LDD warrants further clinical workup and close follow-up for the associated features of CS.

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Section: Discussionmentioning

confidence: 99%

“…The outer thinner layer on MR images (T1 isointense and T2 iso- to hypointense) can be attributed to the outer molecular layer and the leptomeninges which are associated with abnormal vessels and areas of calcification. [ 12 , 13 ]…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos disease: A series of six cases

Kolhe

Shenoy

Tayal

et al. 2023

JNRP

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“…[ 1 , 4 , 14 ] Nonspecific histologic findings include dysplastic ganglion cells, microscopic expansion of the granular cell layer with abundant cytoplasm, large nuclei with prominent nucleoli and granular chromatin, and absence of the Purkinje cell layer. [ 3 , 17 , 19 , 20 ] Therefore, imaging is of great significance for confirming a disease diagnosis. [ 7 ] Treatment options for LDD are limited and the optimal management strategy remains controversial.…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos disease: A rare case of cerebellar tumor with successful surgical treatment

Revuelta-Gutiérrez

Serrano-Rubio

López-Rodríguez

et al. 2023

Surgical Neurology International

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Background: Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the posterior fossa is a slow-growing and extremely rare mass lesion that involves the Purkinje neurons and the granular layer of the cerebellum. It is characterized by specific neuroradiological features and secondary hydrocephalus. However, documentation of surgical experience is scarce. Case Description: A 54-year-old man with LDD manifesting as progressive headache is presented with vertigo and cerebellar ataxia. Magnetic resonance imaging demonstrated a right cerebellar mass lesion with the characteristic “tiger-striped appearance.” We decided to perform partial resection with reduction of tumor volume improving symptomatology as a result of the mass effect in the posterior fossa. Conclusion: Surgical resection is a good alternative for the management of LDD, especially when neurological compromise exists due to mass effect.

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“…This process is what occurs in autosomal dominantly inherited cases of CS and leads to unregulated cell and tumor growth, leading to the hamartoma formation of CS [5]. The manifestations of CS present during adolescence, whereas LDD is a slow-growing tumor that often does not develop until the third or fourth decade of life with slow progressive cerebellar symptoms and symptoms of increased intracranial pressure [5,[8][9][10]. LDD is typically sporadic, however, when associated with CS it is of autosomal dominant inheritance due to PTEN mutations [8].…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination

Weeden,

Mahil,

Huang

2023

Cureus

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Rosai-Dorfman disease (RDD) is a rare condition that causes massive lymphadenopathy, most commonly in the cervical area. Cowden syndrome (CS) causes hamartomas in the skin and mucosa and predisposes individuals to various malignancies. Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma, is often associated with CS. A 41-year-old female with all three conditions presented with abnormal uterine bleeding and endometrial intraepithelial neoplasia (EIN). Precautions should be considered when evaluating patients with RDD and CS preoperatively and during airway management owing to the potential for multisystem involvement, anatomical distortion, and difficult airways. The likelihood of having all three conditions is extremely rare.

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Dysplastic cerebellar gangliocytoma: a description of two cases

Abstract: Dysplastic cerebellar gangliocytoma: a description of two cases.

Cited by 4 publications

References 8 publications

Lhermitte-Duclos disease: A series of six cases

Lhermitte-Duclos disease: A series of six cases

Lhermitte-Duclos disease: A rare case of cerebellar tumor with successful surgical treatment

Anesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination

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