Sarcoid Infiltration of the Skull with Epidural Extension: Case Report and Literature Review

World Neurosurg. 2020 Jul:139:253-259. doi: 10.1016/j.wneu.2020.04.008. Epub 2020 Apr 16.

Abstract

Background: Sarcoidosis is a granulomatous disease most often affecting the lungs, but extrapulmonary manifestations are also common. While virtually any organ system can be involved, skeletal manifestations are present in 1%-13% of cases. Skull lesions are even more rare. We found 14 case reports describing symptomatic skull lesions in patients with no prior history of sarcoid to better understand the symptomology and disease progression.

Case description: Here we present the case of a 57-year-old female with history of sarcoidosis and new-onset forgetfulness, confusion, and headaches who was found to have multiple skull lesions with epidural extension. On histopathologic examination of the excised lesion, noncaseating granulomas were observed and diagnoses of skull sarcoidosis and neurosarcoid were made. Since the patient was actively treated with corticosteroids, the plan was to initiate infliximab.

Conclusion: Sarcoidosis affecting the skull and central nervous system is exceedingly rare and can mimic many pathologies including metastatic bone disease, multiple myeloma, and eosinophilic granulomatosis. With a wide differential, surgical specimen is needed for concrete diagnosis and treatment. While the rates of skeletal involvement are low, a skeletal survey might be an important step in monitoring disease burden in patients, especially as lesions can be asymptomatic.

Keywords: Neurosarcoid; Sarcoid; Sarcoidosis; Skeletal sarcoid; Skull lesion.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Bone Diseases / pathology*
  • Central Nervous System Diseases / pathology*
  • Female
  • Granuloma / pathology
  • Humans
  • Middle Aged
  • Sarcoidosis / pathology*
  • Skull / pathology*

Supplementary concepts

  • Neurosarcoidosis