MON-423 Unique Neuroendocrine Manifestation of Necrotizing Sarcoid Granulomatosis

Abstract

Introduction: Necrotizing granulomatous disease is a rare entity most commonly due to infection. Sarcoidosis accounts for <3% of necrotizing granulomatous disease, and to our knowledge, necrotizing sarcoid granulomatosis (NSG) infiltrating the pituitary has never been reported. We present a unique case of biopsy proven necrotizing sarcoidosis that manifested as necrotizing granulomatous hypophysitis mimicking a pituitary macroadenoma. Case report: A 17-year-old woman, previously healthy, was evaluated at the University of Utah’s Pituitary Clinic for new onset headaches without visual changes. The headaches were solely responsive to steroids. Brain MRI revealed pituitary enlargement measuring 9.3x12.6x19.3 mm abutting the optic chiasm. Based on imaging characteristics and normal laboratory evaluation, it was interpreted to be either a non-functioning pituitary macroadenoma or an infiltrative process. After offering therapy with steroids versus resection, the patient and her family opted for transsphenoidal resection of the pituitary mass. Pathology revealed necrotizing granulomatous inflammation with adenomatous tissue. Subsequent laboratory evaluation was negative for ANA, ANCA, PR3, MPO, HIV, fungal infection, and TB. No hormonal deficiencies were found postoperatively. During the steroid taper, headaches recurred and four months into the taper, she developed new onset pleuritic chest pain and dyspnea. Chest X-ray showed bilateral consolidation and nodular opacities. Chest CT revealed multiple lung nodules, hilar and mediastinal lymphadenopathy. The differential diagnosis included granulomatosis with polyangiitis vs rare necrotizing variant of sarcoidosis. CT guided biopsy of a pulmonary nodule confirmed necrotizing granulomatous sarcoidosis. Prednisone 60 mg daily was initiated resulting in significant improvement of her headaches, chest pain, dyspnea, and near resolution of her lung nodules. Currently, she is doing well on Prednisone 5 mg/day and Methotrexate 15 mg/week. Conclusion: Necrotizing granulomatous sarcoidosis is an uncommon disease very rarely presenting only with pituitary involvement. A high index of clinical suspicion is required for accurate diagnosis and implementation of appropriate treatment.