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

Medicine (Baltimore). 2024 Jan 5;103(1):e36212. doi: 10.1097/MD.0000000000036212.

Abstract

Rationale: Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma (DCG), is a rare tumor originating from the cerebellar cortex. LDD is a benign neuroglial tumor with uncertain prognosis. Over 200 cases have been reported in the literature mostly in the form of case reports. Thus, we present a spectacular case of LDD with excessive calcification in a female septuagenarian.

Patient concerns: A 72-year-old female presented with progressive dizziness for 8 months and suffered a head and sacrococcygeal region injury 20 days prior to her admission in our neurosurgery department.

Diagnosis: Computed tomography scan showed a right nonspecific cerebellar mass with striated calcification. Magnetic resonance imaging revealed a right "tiger-striped" alteration of the cerebellar cortex. H&E staining revealed a low grade glial neural tumor which was consistent with the diagnosis of LDD or DCG.

Intervention: The lesion was total resected.

Outcomes: The patient recovered well and the cerebellar dysfunctional symptoms subsided 3 months after the operation and 2 years follow-up revealed no recurrence of the lesion and no neurological deficits.

Lesion: We postulate that the calcification of LDD is age-related and the pathogenesis of disease often observed in young adulthood.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Brain Neoplasms*
  • Calcification, Physiologic
  • Calcinosis* / diagnostic imaging
  • Calcinosis* / surgery
  • Cerebellum
  • Female
  • Glioma*
  • Hamartoma Syndrome, Multiple* / complications
  • Hamartoma Syndrome, Multiple* / diagnosis
  • Hamartoma Syndrome, Multiple* / surgery
  • Humans
  • Young Adult