IgG4-negative pituitary inflammatory pseudotumor with sphenoidal involvement resembling a macroadenoma

Fawwaz Almajali, Mohammad Almajali, Kate Schwetye, Georgios Alexopoulos, Maheen Khan, Jeroen Coppens, Philippe Mercier

Research output: Contribution to journalArticlepeer-review


Background: Inflammatory pseudotumors (IPTs) are rare benign conditions of unknown etiology that can affect any part of the body. IPTs are most commonly associated with Immunoglobulin G4 (IgG4)-related disease. Central nervous system IPTs, especially with pituitary involvement, are even rarer entities. The presence of an IgG4-negative pituitary IPT with simultaneous extracranial involvement has not been reported. Case report: We present the case of a 41-year-old female with past medical history of rheumatoid arthritis and a diagnosis of pituitary IPT with coexisting sphenoidal (extracranial) involvement mimicking a pituitary macroadenoma at presentation. The patient underwent multiple consecutive biopsies, and an extensive workup prior to establishing the diagnosis. Laboratory work-up showed normal serum IgG4 and unremarkable liver function tests. Conclusion: Pituitary lesions with simultaneous sphenoidal involvement in patients with IgG4-negative systemic inflammatory disease should raise the clinical suspicion for intracranial IPTs, as these tumors can mimic aggressive counterparts causing adjacent bony erosion, and local invasion.

Original languageEnglish
JournalBritish Journal of Neurosurgery
StateAccepted/In press - 2020


  • Intracranial inflammatory pseudotumor
  • pituitary mass
  • pituitary pseudotumor
  • sellar pseudotumor
  • sphenoiditis


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