Unusual presentation of sarcoidosis: solitary intracranial mass lesion mimicking an intracranial neoplasm: a case report

Abstract

Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease however is a rare. In this report, we present an unusual case of neurosarcoidosis with intra cranial mass mimicking radiologically a glioma. Pathological examination revealed intraparenchymatous necrotising granulomatous lesions. After clinicopathological correlation, the diagnosis of a necrotizing cerebral granulomatosis (neurosarcoidosis) with atypical systemic involvement was made. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease.

Case report

“…[ 4 , 17 , 25 , 26 ] NS is reported in 5% to 10% of patients with sarcoidosis and is frequently associated with systemic disease; however, 10% to 17% of patients with NS have no apparent systemic involvement. [ 7 , 16 , 17 , 21 , 22 ] The leptomeninges are primarily affected, but parenchymal spread occurs through Virchow–Robin spaces (at base of brain), making cranial nerves most susceptible. [ 8 , 21 , 22 , 25 , 27 , 28 ]…”

“…Treatments may differ dramatically and be harmful if an improper diagnosis is made. [ 1 , 4 , 11 , 13 , 15 , 16 ] Isolated NS, devoid of other organ involvement, is a rare but often significant cause of morbidity and mortality. [ 2 , 5 , 11 , 15 , 17 – 23 ] Necrotizing NS is prone to vasculitic patterns.…”

Recurrent Bifacial Neuropathy in a Case of Steroid Responsive Neurosarcoidosis: A Case Report

“…However, it is nonspecifi c. About one-third of patients with NS will have normal CSF analysis fi ndings as was the situation in our present case (10). CSF analysis will be helpful in excluding CNS infections or malignancy (2,5,7). An elevated CSF protein level typically accompanies oligoclonal bands in patients with NS, but which was not found in this present case.…”

“…This may present as progressive, painless or acute visual loss, papilledema, or optic atrophy (1,2). It is not present in our patient, but other presentations of NS may be the following: hydrocephalus and chronic meningitis (2,8), mass lesions (2), seizure (9), hypothalamic and pituitary involvement, and neuropsychiatric manifestations (5,9).…”

“…The process is either subacute or chronic in nature mimicking other granulomatous lesions and neoplasms. When the nervous system is involved as it is in about 5-13% of cases, NS can occur either in isolation or along with other features of systemic sarcoidosis (5,6). In a review by Lacomis (6), the mean age of onset is from 33 to 41 years.…”