Neurosarcoidosis presenting as an isolated intrasellar mass: case report and review of the literature

Abstract

Objective: Isolated neurosarcoidosis without evidence of extracranial manifestation continues to be a rare phenomenon. This case report and others in the literature demonstrate the difficulty in making the diagnosis of isolated neurosarcoidosis, as it may be indistinguishable from other pathologies on radiographic and laboratory studies. This case report and review of the literature will emphasize the need for clinical suspicion for neurosarcoidosis in patients with intrasellar lesions and the appropriate clinical history.

Case history: A 37-year-old female presented with visual field changes and a headache unresponsive to nonsteroidal anti-inflammatory medications. A history of Bell's palsy, hypothyroidism, and a history of sarcoidosis in the patient's father were noted. Imaging revealed an intrasellar mass resembling a pituitary macroadenoma. Routine neuroendocrine laboratory studies were consistent with hypopituitarism, and all other standard laboratory tests were normal. An endonasal transsphenoidal resection of the intrasellar lesion was done. The tissue was inconsistent with a typical adenoma. Intraoperative pathology reported non-caseating granulomatous disease. Based on the patient's history and intraoperative pathology she was diagnosed with neurosarcoidosis, which was confirmed by final pathologic analysis. Minimal debulking was performed to decompress the optic chiasm. The patient was then placed on corticosteroids and methotrexate and responded well to medical therapy.

Conclusion: If isolated neurosarcoidosis is diagnosed early it will save a costly and invasive work-up. Radiographic and laboratory studies may aid in diagnosis but no studies are pathognomonic. Neurosarcoidosis is diagnosed by a combination of imaging, diagnostic tests, and good clinical suspicion.