Exclusive CNS Involvement by Lymphomatoid Granulomatosis in a 12-year-old Boy: A Case Report

Surg Neurol. 1999 Mar;51(3):258-60. doi: 10.1016/s0090-3019(98)00017-2.

Abstract

Background: Lymphomatoid granulomatosis [LYG] is an angiocentric, angiodestructive disease at the higher grade end of the spectrum of angiocentric immunoproliferative lesions. It primarily involves the lungs, but it may also involve several extrapulmonary sites including the central nervous system (CNS), skin, and kidneys.

Case description: Clinical presentation, radiology and treatment of LYG in a 12-year-old male child with multiple intracranial extraaxial lesions is described. A 12-year-old boy presented with sudden onset of left focal motor seizures with associated history of headache and vomiting. Computerized tomographic scan of the brain suggested high-density, bilateral, parietal extraaxial lesions. On magnetic resonance imaging, the lesions were iso- to hyperintense on T1-weighted images and hyperintense on T2-weighted images. The lesions were excised in two stages and histopathological examination confirmed the diagnosis.

Conclusion: LYG seldom involves the CNS exclusively. The present case demonstrates exclusive CNS involvement by LYG in a young boy.

Publication types

  • Case Reports

MeSH terms

  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / surgery
  • Child
  • Diagnosis, Differential
  • Humans
  • Lymphomatoid Granulomatosis / diagnosis*
  • Lymphomatoid Granulomatosis / surgery
  • Male