Cervical epidural neurosarcoidosis: A case report and literature review

INTRODUCTION

Sarcoidosis is a multisystem disease attributed to an accelerated immune response of unknown etiology.[7] Systemic sarcoidosis occurs in 1–3/100,000 individuals; only 5% of lesions involve the nervous system (e.g., neurosarcoidosis). Just 10% of those with neurosarcoidosis, which have spinal lesions that are typically located within the cord itself (i.e., intramedullary).[7]

Here, we present a 29-year-old female with left-sided C2–C7 epidural cervical neurosarcoidosis diagnosed utilizing an open biopsy, followed by appropriate medical management.

CASE REPORT

A 29-year-old female presented with a 22-month history of progressive dysphagia, neck pain, and cervical myelopathy. Although she had no motor deficit, she exhibited impaired pin appreciation on the left side from C2–C7, bilateral Hoffman’s signs, and diffuse upper and lower extremity hyperreflexia.

The computed tomography (CT) and full neuraxis enhanced MR studies showed a left-sided epidural enhanced mass from C2 to C7 compressing the spinal cord and displacing it to the right with a paravertebral extension [Figure 1a-d].

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Figure 1:: Cervical CT with contrast (a: sagittal and b: axial) and MRI (c: sagittal and d: axial) shows a longitudinally oriented epidural enhancing soft-tissue mass lesion occupying the left side of the spinal canal opposite C2 down to the C7 vertebra. MRI (c: sagittal and d: axial) shows intraspinal epidural and paraspinal avidly enhancing cervical lesions with multiple neural foraminal extensions and cord compression, which was most severe at C5.

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Three days after the presentation, a CT-guided biopsy was performed. However, due to the initiation of steroids (dexamethasone 4 mg every 6 h), the tumor had significantly decreased in size (e.g., repeat MRI showed that the lesion had profoundly regressed), and a biopsy was no longer feasible/ reliable [Figure 2a-d].

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Figure 2:: Contrasted cervical CT (a: sagittal and b: axial) during the CT-guided biopsy, preprocedure scan demonstrated improvement of the prevertebral tissue, likely related to steroids treatment. Due to the lack of enough safety margin to perform biopsy, the procedure was cancelled. Repeated cervical MRI (c: sagittal and d: axial), showed the lesion response to the steroids more profoundly.

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The next MR performed 2 weeks after cessation of steroids revealed significant tumor recurrence [Figure 3a and b]. The repeated CT-guided biopsy revealed a granuloma in a background of nonneoplastic skeletal muscular and fibrous tissues. This was followed by an open anterior C5–C6 biopsy of the large recurrent extradural lesion; the latter confirmed the diagnosis of neurosarcoidosis (i.e., nonnecrotizing granulomatous lymphadenitis) neurosarcoidosis [Figure 4a and b]. She was later discharged on 60 mg prednisone/day and placed on an 8-week taper. Three months later, she exhibited substantial clinical improvement.

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Figure 3:: The cervical spine MRI (a: sagittal and b: axial) was repeated after 2 weeks from the previous MRI [Figure 2] while stopping the steroids and showed a significant recurrence.

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Figure 4:: (1) Low magnification – H&E stain (a) shows a well- demarcated granuloma. (2) High magnification – H&E stain (b) the granuloma consists of epithelioid histiocytes and mature lymphocytes.

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DISCUSSION

Sarcoidosis is a chronic systemic inflammatory disease characterized by noncaseating granulomas on pathological examination. It most commonly affects the lungs and mediastinal lymph nodes.[7] Only approximately 5% of all sarcoidosis patients have central nervous system involvement; most of these lesions are intracranial.[7,6]

Just 10% of neurosarcoidosis involve the spine. Seven similar cases found in the literature. Of interest, most are intramedullary lesions, and few involve the extradural compartment [Table 1].[2,5,6]

Table 1:: Cases of spinal epidural neurosarcoidosis.

Most cases of neurosarcoidosis have normal levels of ACE, and the histology demonstrates nonnecrotizing granulomas with an inflammatory background [Table 1].[1,7,8]

Although medical management is the standard of care for neurosarcoidosis, most patients initially undergo biopsy\ surgical decompression or debulking to obtain pathology [Table 1]. Medical management usually includes steroids (e.g., in some cases, require a prolonged course of steroid utilization) along with immunosuppressive medications(e.g., methotrexate).[3,4]

CONCLUSION

Epidural spinal neurosarcoidosis is very rare. Establishing a pathological diagnosis warrants cessation of steroids (i.e., if previously administered), and the performance of a biopsy\ or open surgical debulking. Once pathology has been established, these lesions can be typically managed with short-\long-term steroids and/or other medical regimens.