A rare case of neurosarcoidosis occurred only in the medulla oblongata mimicking malignant brain tumor
- Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan.
- Department of Neurology and Geriatric Medicine, Ehime University School of Medicine, Toon, Ehime, Japan.
- Department of Diagnostic Pathology, Ehime University Hospital, Toon, Ehime, Japan.
DOI:10.25259/SNI_195_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Kentaro Murayama1, Akihiro Inoue1, Yawara Nakamura1, Masayuki Ochi2, Seiji Shigekawa1, Hideaki Watanabe1, Riko Kitazawa3, Takeharu Kunieda1. A rare case of neurosarcoidosis occurred only in the medulla oblongata mimicking malignant brain tumor. 31-May-2021;12:243
How to cite this URL: Kentaro Murayama1, Akihiro Inoue1, Yawara Nakamura1, Masayuki Ochi2, Seiji Shigekawa1, Hideaki Watanabe1, Riko Kitazawa3, Takeharu Kunieda1. A rare case of neurosarcoidosis occurred only in the medulla oblongata mimicking malignant brain tumor. 31-May-2021;12:243. Available from: https://surgicalneurologyint.com/?post_type=surgicalint_articles&p=10840
Background: Sarcoidosis is a multisystem disorder characterized by noncaseating epithelioid granulomas. However, neurosarcoidosis occurring only in the medulla oblongata is very rare and lacks specific imaging and clinical features. We report a rare case of neurosarcoidosis arising from the medulla oblongata alone, suggesting the significance of pathological findings for accurate diagnosis.
Case Description: A 78-year-old woman with a history of rheumatoid arthritis was admitted to our hospital with a 3-month history of progressive numbness in bilateral lower extremities and gait disturbance. Neurological examination on admission showed mild bilateral paired paralysis of the lower limbs (manual muscle test: right 2/V; left 4/V) and marked numbness in the right lower limb. Neuroimaging revealed a solid mass with clear boundaries in the dorsal medulla oblongata appearing hypointense on T1-weighted imaging (WI), hyperintense on T2-WI, and hypointense on diffusion WI (DWI), with strong enhancement on gadolinium-enhanced T1-WI. Cerebrospinal fluid analysis showed moderately elevated levels of protein and lymphocytic cells. Biopsy to determine the exact diagnosis revealed histological findings of noncaseating epithelioid granulomas and inflammatory infiltration, consistent with sarcoidosis. Postoperatively, corticosteroid therapy with prednisolone was initiated as soon as possible, resulting in marked reductions in lesion size. Follow-up neuroimaging after 12 months showed no signs of recurrence.
Conclusion: Neurosarcoidosis is difficult to diagnose from routine neuroimaging and laboratory findings. Accurate diagnosis requires careful identification of clinical signs, hypointensity on DWI, and morphological findings from surgical biopsy.
Keywords: Medulla oblongata, Neurosarcoidosis, Noncaseating epithelioid granuloma, Pathological finding
Sarcoidosis is a multisystemic disorder of unknown etiology.[
Neurosarcoidosis in the medulla oblongata is particularly rare, with only four cases reported previously [
A 78-year-old woman with a history of rheumatoid arthritis was admitted to our hospital with a 3-month history of progressive numbness in both lower extremities and gait disturbance. Neurological examination on admission showed mild paralysis of both lower limbs (manual muscle test: right 2/V; left 4/V) and pronounced numbness in the right lower limb. Magnetic resonance imaging (MRI) revealed a solid mass with clear borders in the dorsal medulla oblongata, appearing hypointense on diffusion-weighted imaging (DWI), fluid-attenuated inversion recovery, and T1-WI (WI), and hyperintense on T2-WI, with strong enhancement on gadolinium (Gd)-enhanced T1-WI [
Preoperative axial T1-weighted (a), T2-weighted (b), fluid-attenuated inversion recovery (c), diffusion-weighted (d) and gadolinium-enhanced T1-weighted (e) magnetic resonance imaging shows a solid mass at the dorsal medulla oblongata (f). The tumor shows prominent homogeneous enhancement with gadolinium.
Intraoperative microscopic findings from craniotomy using a midline suboccipital approach. (a) The neuronavigation system demonstrates locations of the lesion in the microscopic view. (b) Microscopic examination of this lesion shows a solid, rubbery firm mass (black arrow). (c) The cut surface (white dashed circle) is gray to yellowish in color.
(a) Histopathology of the resected lesion shows a noncaseating granuloma comprising epithelioid cells without evidence of cellular atypia or mitotic figures (hematoxylin and eosin staining). Staining shows infiltration of lymphocytes and macrophages. (b-d) Immune cells show positive staining for CD68 (b) and CD3 (c), but very slight staining for CD20 (d). Magnification: (a-1) x200; a-2, (b-d) x400. Scale bars, 100 μm.
Sarcoidosis is a granulomatous disease that is usually systemic and may include the CNS, as so-called neurosarcoidosis. [
Clinically, neurosarcoidosis tends to be more common than ordinary sarcoidosis in older women.[
CSF examination yields results similar to common subacute meningitis.[
Microscopically, the histopathological characteristics of sarcoidosis are demonstrated by noncaseating epithelioid granuloma formation.[
Finally, regarding the treatment of neurosarcoidosis, the optimal treatment remains to be determined.[
We suggest that neurosarcoidosis should be included as an important differential diagnosis for intramedullary malignant brain tumor. Even if the patient shows no evidence of systemic sarcoidosis, consideration of neurosarcoidosis is very important. Careful identification of clinical signs, MRI findings including hypointensity on DWI and detailed histopathological evaluation of specimens from surgical biopsy are necessary for accurate diagnosis of neurosarcoidosis. In addition, accurate recognition of these pathological findings may expedite appropriate treatment with steroids while avoiding unnecessary extensive surgery.
1. Caneparo D, Lucetti C, Nuti A, Cipriani G, Tessa C, Fazzi P. A case of sarcoidosis presenting as a non-specific intramedullary lesion. Eur J Neurol. 2007. 14: 346-9
2. Chen XY, Ren ZC, Huang XJ. Sarcoidosis of the medulla oblongata causing intractable hiccoughs and numbness of extremities: A case report. Medicine (Baltimore). 2018. 97: e13667
3. Dennis AN, Darius CW. Neurosarcoidosis: A review of its intracranial manifestation. J Neurol. 2001. 248: 363-72
4. Ferriby D, Seze JD, Stojkovic T, Hachulla E, Wallaert B, Destee A. Long-term follow-up of neurosarcoidosis. Neurology. 2001. 57: 927-9
5. Jarnier D, Series C. Neurosarcoidosis: Review of the literature. Neurochirurgie. 1999. 45: 214-8
6. John S, Parambil J, Culver D, Tavee J. Medullary neurosarcoidosis presenting with intractable hiccoughs. J Clin Neurosci. 2012. 19: 1193-5
7. Lee JH, Takai K, Ota M, Shimizu T, Komori T, Taniguchi M. Isolated neurosarcoidosis in the medulla oblongata involving the fourth ventricle: A case report. Br J Neurosurg. 2013. 27: 393-5
8. Mahadewa TG, Nakagawa H, Watabe T, Inoue T. Intramedullary neurosarcoidosis in the medulla oblongata: A case report. Surg Neurol. 2004. 61: 283-7
9. Negi M, Takemura T, Guzman J, Uchida K, Furukawa A, Suzuki Y. Localization of propionibacterium acnes in granulomas supports a possible etiologic link between sarcoidosis and the bacterium. Mod Pathol. 2012. 25: 1284-97
10. Okamura M, Hamaguchi M, Suzuki K, Nakamura T, Fujita H, Hirata K. A patient with neurosarcoidosis presenting with easy falling and dysphagia. Rinsho Shinkeigaku. 2020. 60: 346-50
11. Pawate S, Moses H, Sriram S. Presentations and outcomes of neurosarcoidosis: A study of 54 cases. QJM. 2009. 102: 449-60
12. Uchino M, Nagao T, Harada N, Shibata I, Hamatani S, Mutou H. Neurosarcoidosis without systemic sarcoidosis-case report. Neurol Med Chir (Tokyo). 2001. 41: 48-51
13. Vinas FC, Rengachary S. Diagnosis and management of neurosarcoidosis. J Clin Neurosci. 2001. 8: 505-13