Lhermitte-duclos disease (dysplastic cerebellar gangliocytoma): a case report

Authors

  • Aribah Atiq Department of Histopathology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan
  • Usman Hassan Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan
  • Noreen Akhtar Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan
  • Sajid Mushtaq Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan

DOI:

https://doi.org/10.47391/JPMA.591

Abstract

Abstract

Lhermitte-Duclos disease (LDD) is a relatively uncommon condition of the cerebellum. It is generally characterised as a hamartomatous lesion of posterior fossa and is common in the third and fourth decades of life. According to the World Health Organisation, it is classified as a grade I tumour with potential for recurrence. Otherwise, this disease is generally associated with good prognosis. Malignant transformation of LDD has not yet been reported. However, genetic counselling of the patient is recommended with active surveillance. Since LDD is believed to be a pathognomonic feature of Cowden syndrome, which is a multi-system autosomal dominant hereditary disorder characterised by multiple hamartomas and an elevated risk of benign and malignant neoplasms, we decided to report this important entity considering its rarity and high clinical significance.

Keywords: Lhermitte-Duclos disease, Cowden syndrome, dysplastic cerebellar gangliocytoma, PTEN

Published

2020-10-17

How to Cite

Aribah Atiq, Usman Hassan, Noreen Akhtar, & Sajid Mushtaq. (2020). Lhermitte-duclos disease (dysplastic cerebellar gangliocytoma): a case report. Journal of the Pakistan Medical Association, 1-7. https://doi.org/10.47391/JPMA.591

Issue

Section

Case Series