Sarcoidosis of the pineal gland: an unusual presentation of neurosarcoidosis

Abstract

Introduction Sarcoidosis is an inflammatory disease characterized by noncaseating granulomas that is rarely found as primary CNS pathology. We report an unusual case of sarcoidosis involving the pineal gland with radiographic, histopathology, and clinical data. Case report A 45-year-old man without evidence of systemic sarcoidosis presented with a history of gradual onset of blurry vision and diplopia that progressed over 3 months. MR imaging demonstrated an enhancing mass in the pineal region. A suboccipital craniotomy was performed with resection of the mass through a supra-cerebellar infratentorial approach. Histopathologic analysis did not reveal a pineoblastoma but instead revealed noncaseating granulomas within the pineal gland. Extensive hematologic laboratory examinations, cerebral spinal fluid studies, and cultures for infection were all negative. This mass lesion was diagnosed as solitary neurosarcoidosis of the pineal gland, without dissemination. The patient was treated with steroids and at 4-year follow-up is asymptomatic with an unremarkable MRI scan. Conclusion This is an unusual case of pineal sarcoidosis mimicking a tumor with associated MRI, CT and histopathologic findings reported together. Although rare, sarcoidosis of the pineal gland should not be excluded from a comprehensive differential diagnosis of an enhancing pineal region mass.

The Isolated Pituitary Neurosarcoidosis: A Case Report and Review of The Literature

“…In our case the CSF cell count was normal but the protein was slightly elevated to 0.89 gm/dL and there were identical oligoclonal bands in CSF and in blood pointing to a systemic disorder with CNS involvement rather than a pure CNS disorder. Both computed tomography and MRI scans are helpful in disease evaluation; however MRI scan is the modality of choice 5,12 . MRIs show a wide range of CNS abnormalities including hypothalamic-pituitary infiltrating lesions, cerebral parenchyme masses, leptomeningeal lesions, and focal white-matter lesions 5,13 .…”

“…The clinical presentation of neurosarcoidosis is widely variable, it can be manifested in a maltitude of ways including cranial neuropathy, aseptic meningitis, encephalopathy, vasculopathy, seizures, psychiatric manifestations, hydrocephalus, hypothalamic pituitary disorders, myelopathy and peripheral neuropathy 1,[4][5][6][7] . The disease has a predilection to spread from the leptomeninges to the Virchow-Robin spaces leading to invasion and thrombosis of associated blood vessels resulting in granulomatous angitis.…”

“…ACE levels in the serum and cerebrospinal fluid may be increased, decreased or normal 2 . Serum or CSF ACE levels are found to be elevated in approximately 70-80% of patients with sarcoidosis, hypercalcemia may be found in 2-15% of the patients due to enhanced sensitivity to vitamin D, however the diagnosis of sarcoidosis is confirmed by histopathological examination 5,10,11 . In our case the CSF cell count was normal but the protein was slightly elevated to 0.89 gm/dL and there were identical oligoclonal bands in CSF and in blood pointing to a systemic disorder with CNS involvement rather than a pure CNS disorder.…”

“…Both computed tomography and MRI scans are helpful in disease evaluation; however MRI scan is the modality of choice 5,12 . MRIs show a wide range of CNS abnormalities including hypothalamic-pituitary infiltrating lesions, cerebral parenchyme masses, leptomeningeal lesions, and focal white-matter lesions 5,13 . The use of gadolinium improves the sensitivity of detecting leptomeningeal lesions.…”

“…The disease is associated with intrathoracic lesions in about 70% of cases; therefore, an intensive search for enlarged pulmonary lymph nodes should be performed 14 . MRI is the modality of choice in neurosarcoidosis evaluation 5,12 and although any technique can be used to diagnose suprasellar lesions, tissue biopsy taken from the lesion is required for definitive diagnosis and to exclude other cerebral pathologies.…”