Unusual presentation of a skull base mass lesion in sarcoidosis mimicking malignant neoplasm: a case report

Katsunori Shijo,Mitsuru Watanabe,Nobuhiro Moro,Atsuo Yoshino,Hiroshi Yagasaki,Taku Homma,Shori Takahashi,Mari Sasano

doi:10.1186/s12883-018-1076-6

Abstract

BackgroundSarcoidosis is a multi-organ disease of unknown etiology characterised by the presence of epithelioid granulomas, without caseous necrosis. Systemic sarcoidosis is rare among children, while neurosarcoidosis in children is even rarer whether it is systemic or not.Case presentationWe described the case of a 12-year-old boy who presented with monocular vision loss accompanied by unusual MRI features of an extensive meningeal infiltrating mass lesion. The patient underwent surgical resection (biopsy) via a frontotemporal craniotomy to establish a definitive diagnosis based on the histopathology, since neurosarcoidosis remains a very difficult diagnosis to establish from neuroradiogenic imagings. Based on the histopathology of the resected mass lesion, neurosarcoidosis was diagnosed. On follow-up after 3 months of steroid therapy, the patient displayed a good response on the imaging studies. MRI revealed that the preexisting mass lesion had regressed extremely. We also conducted a small literature review on imaging studies, manifestations, appropriate treatments, etc., in particular neurosarcoidosis including children.ConclusionAlthough extremely rare, neurosarcoidosis, even in children, should be considered in the differential diagnosis of skull base mass lesions to avoid unnecessary aggressive surgery and delay in treatment, since surgery may have little role in the treatment of sarcoidosis.

Highlights

Sarcoidosis is a multi-organ disease of unknown etiology characterised by the presence of epithelioid granulomas, without caseous necrosis
Even fewer cases of sarcoidosis have been reported in children, especially cases affecting the central nervous system (CNS) [5]
Baumann and Robertson [6] reviewed pediatric neurosarcoidosis and concluded that childhood neurosarcoidosis differs in its presenting signs and symptoms from neurosarcoidosis in adults; children are more likely to have seizures, less likely to have cranial nerve palsies, and perhaps more likely to

Summary

Conclusion

Neurosarcoidosis, even in children, should be considered in the differential diagnosis of skull base mass lesions to avoid unnecessary aggressive surgery and delay in treatment, since surgery may have little role in the treatment of sarcoidosis.

Background

Findings

Discussion and conclusions