Primary Presentation of Neurosarcoidosis as a Pituitary Mass with Sinonasal Involvement

ABSTRACT

Objective: Sarcoidosis is a systemic granulomatous disease identified by noncaseating granulomas that can have central nervous system (CNS) involvement but rarely presents with primary CNS involvement. Neurosarcoidosis can present with or without systemic disease and can present with mass effect, endocrinopathies, and neuropathic symptoms. We report on an unusual case of neurosarcoidosis involving a pituitary adenoma. Case report A 45-year-old African American man presented to ophthalmology clinic with a chief complaint of worsening field of vision. Magnetic resonance imaging (MRI) showed a 3.8-cm macroadenoma containing cystic and calcified components with optic chiasm compression, near-complete opacification of the maxillary sinuses, and mucosal thickening in the frontal sinuses. Due to the COVID-19 pandemic, follow-up was delayed for 4 months while the vision loss progressed to near blindness in his right eye. After thorough evaluation, the patient was found to have near-complete right sided blindness, diffuse lymphadenopathy. After interdisciplinary discussions surgery was recommended. The patient underwent endoscopic transsphenoidal resection of the pituitary tumor and concurrent endoscopic sinus surgery. During the approach sinus mucosa was grossly inflamed. Frozen section of the sinus mucosa revealed granulomatous disease. The suprasellar mass had both soft contents which could be suctioned and fibrotic tumor with dense septations. Final pathology showed a pituitary adenoma with non-necrotizing granulomas within in the pituitary adenoma. Post-operatively, the patient was started on steroids and reported gradual improvement in his visual fields. At 3-months post-operative, MRI showed significant reduction in macroadenoma with a 1.5 cm residual tumor remaining in the sella and a decompressed optic chiasm which retracted inferiorly without any evidence of other intercranial anomalies. Literature review Sarcoidosis occurs mostly in African Americans and Northern European women in their 3rd and 4th decades and can affect any part of the body such as the lymphatic systems, skin, lungs, and liver. Sarcoidosis is estimated to be prevalent in up to 80 per 100,000 people. CNS involvement occurs in 5 to 15% of patients with systemic sarcoidosis and can present with cranial neuropathy such as 7th nerve palsy, anticonvulsant refractory seizures, visual changes, and headaches. Pituitary involvement occurs 0.5% of patients with sarcoidosis and can present with endocrine and water metabolism dysfunction while sinonasal sarcoidosis can occur in up to 4%. Sinonasal sarcoidosis most usually presents as chronic crusting rhinitis, nasal obstruction, anosmia, and epistaxis and can show clinically as mucosal hypertrophy and external nose deformity in ~10% of these patients. Discussion and Conclusion: Symptomatic pituitary mass as the initial presentation of sarcoidosis is extremely rare. This case of primary neurosarcoidosis is unusual with its initial presentation mimicking non-functional pituitary macroadenoma with optic chiasm compression and associated vision loss. Primary presentation of neurosarcoidosis as a pituitary mass is rare but should be included in the differential diagnosis of a patient presenting with a combination of a macroadenoma, chronic sinusitis, and lymphadenopathy.