| Summary: | ABSTRACT: Objectives: Pituitary adenomas are the most common cause of sellar-suprasellar masses, but other nonadenomatous neoplastic lesions (e.g., gliomas, meningiomas, craniopharyngiomas), metastatic deposits, inflammatory lesions, and infections may also afflict the sella. Sellarsuprasellar masses can present with mass effect as well as symptoms and signs secondary to hormonal disturbances. We report a 32-year-old subject with IgG4-related sellarsuprasellar mass who remained undiagnosed for nearly 2 decades.Methods: Case report and literature review.Results: Our patient initially presented with nasal obstruction at the age of 14 years and underwent multiple surgical procedures for nasal mass with minimal relief. He subsequently developed bilateral progressive loss of vision; work-up for the same revealed sellar-suprasellar mass due to infundibulo-hypophysitis and hypertrophic pachymeningitis. In addition, he also had primary testicular failure. Histopathologic findings from sinonasal mass were consistent with IgG4-related disease. Serum IgG4 levels were also found to be raised. He was subsequently administered glucocorticoids, which resulted in significant reduction in the size of the sellar-suprasellar mass.Conclusion: In a patient with sellar-suprasellar mass along with synchronous multisystem involvement (sinonasal, meningeal, and testicular), a diagnosis of IgG4-related disease should be considered.Abbreviations: CT = computed tomography;FDG = fluorodeoxyglucose;Ig = immunoglobulin;MRI = magnetic resonance imaging
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