Spinal cryptoccoma mimicking a spinal cord tumor complicated by cryptococcal meningitis in an immunocompetent patient
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.237012
Source of Support: None, Conflict of Interest: None
A 19-year old immunocompetent healthy young man presented with acute onset symptoms suggestive of a spinal cord lesion. An urgent magnetic resonance imaging (MRI) of the whole spine revealed a lesion that raised the suspicion of a spinal cord tumor, possibly an ependymoma [Figure 1]a and [Figure 1]b.
A laminectomy was done 48 hours later. The biopsy report was consistent with chronic granulomatous inflammation secondary to Cryptococcus neoformans infection [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. A thorough immunological workup proved that the patient was immunocompetent.
Nevertheless, antifungal therapy alongside steroid therapy was promptly started. Despite active antifungal therapy, he deteriorated and expired due to nosocomial infection.
The acute onset presentation of an intramedullary spinal cord cryptococcoma is a rare initial presentation of cryptococcal infection. Here, we describe an unusual case of a young immunocompetent patient with a solitary spinal cord cryptococcoma that subsequently led to cryptococcal meningitis, that occurred in the absence of a cerebral cryptococcoma. Prior to this episode, he did not have headache, vomiting, and seizures to suggest a central nervous system infection or raised intracranial pressure. This immunocompetent patient presented acutely with sudden onset of lower limb weakness as a result of the spinal cryptococcoma.
Infection with C. gattii presents with granulomatous inflammatory response in immunocompetent people, resulting in a chronic disease; the patients remain at a risk of developing an intracerebral mass lesions (e.g., a cryptococcoma). In comparison, C. neoformans has a tendency to cause infection in predominantly immunodeficient patients, leading to acute meningitis. In this case, the patient developed C. neoformans of the spinal cord despite being immunocompetent and a young man. The patient progressed to have cryptococcal meningitis despite institution of antifungal therapy and surgical intervention.
The intramedullary lesions of spinal cryptococcosis are described as cryptococcoma. Intramedullary spinal cryptococcoma are less common compared to intradural and other types of extradural lesions. In the literature, there have been a few reports of spinal intramedullary cryptococcoma. In these rare reports, the majority of the patients presented with paraparesis and pyramidal signs in the lower limbs with a sensory level in the thoracolumbar region. In some cases, similar to our case, the lesion was initially thought to be a tumor. Most of them were diagnosed after a biopsy. The cryptococcoma on MRI spine appeared as hyperintense lesions with edema and a ring enhancement, similar to the appearance in our patient. Most patients developed the lesions in the thoracic or upper lumbar regions. The survival rate for these patients, if the lesion was limited to the spine, was high., The incidence of spinal cryptococcoma with cerebral meningitis is not well documented. However, Stein et al., reported 4 patients with mycotic spinal arachnoiditis and meningitis due to cryptococcal infection. Three of these patients developed acute fungal meningitis postoperatively and 2 out of 3 of them died, which is an outcome similar to our case. None of the patients had cryptococcoma of the spine. Therefore, mortality is higher when cerebral involvement is present.
Treatment in our patient involved surgical intervention for removal of the tumor followed by intravenous administration of antifungal regiment; however, it is worth discussing possible alternative treatment options. As intravenous amphotericin B has poor central nervous system penetration, perhaps a lumbar puncture catheter drainage with an intrathecal injection of amphotericin B might have proven to be effective. In a trial conducted by Yuchong et al., all 14 patients survived with no evidence of a relapse during a 1-year follow-up with intrathecal injection of amphotericin B or with a low-dose systemic therapy. Therefore, this technique should be considered as it appears to be effective and safe.
The role of steroid was considered in this case, due to the presence of mass effect, to help in reducing the swelling caused by the tumor residing in the spinal region, which was causing significant neurological deficit. So far, there is no strong evidence showing the risks and benefits of steroid usage in severe central nervous system cryptococcal disease. However, there are multiple case reports showing therapeutic success and good neurologic outcomes with the use of adjunctive steroids among non-HIV infected individuals with severe central nervous system cryptococcal infection. Nonetheless, more randomized control trials are needed to provide better evidence.
Although rare, a spinal cryptococcoma should be thought of when MRI shows hyperintense lesions with ring enhancement involving the thoracic and upper lumbar region. This diagnosis needs to be entertained in young patients as this disease is potentially treatable. We need to be more vigilant when it comes to diagnosing spinal cryptococcoma. This is to highlight awareness regarding the disease, to help in improving clinical guidelines, and to offer better management for this disease.
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There are no conflicts of interest.
[Figure 1], [Figure 2]