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Neurological emergency from rare spinal metalloma: Case report and literature review.

ABSTRACT: Metallosis is a rare and poorly understood long-term complication of instrumented surgery that can result in an inflammatory pseudotumor termed metalloma. We describe a particularly unique case and compare it to 6 analogous cases identified by PubMed and/or Medline search through July 2020. A 79-year-old male with multiple prior spinal lumbar fusion procedures presented with progressive weakness and pain. Imaging revealed a large mass surrounding the right-sided paraspinal rod with extension into the spinal canal, neural foramina, extraforaminal spaces, psoas muscle, marrow spaces, and right sided pedicles. The case presented is a unique example of a unilateral metalloma with mixed-metal instrumentation that created a progressive neurologic deficit without infection, pseudoarthrosis, or hardware failure. This case highlights the lack of understanding regarding the pathophysiology of metallosis and metalloma in spinal instrumentation. We highlight the imaging findings of metalloma to encourage early identification for removal and decompression.

SUBMITTER: Mazur-Hart DJ

PROVIDER: S-EPMC8908052 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Neurological emergency from rare spinal metalloma: Case report and literature review.

Mazur-Hart David J DJ Larson Erik W EW Yaghi Nasser K NK Halfpenny Aaron M AM Pettersson David R DR Yam David A DA

Radiology case reports 20220307 5

Metallosis is a rare and poorly understood long-term complication of instrumented surgery that can result in an inflammatory pseudotumor termed metalloma. We describe a particularly unique case and compare it to 6 analogous cases identified by PubMed and/or Medline search through July 2020. A 79-year-old male with multiple prior spinal lumbar fusion procedures presented with progressive weakness and pain. Imaging revealed a large mass surrounding the right-sided paraspinal rod with extension int ...[more]

PMID: 35282321

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Project description:Background Mesenchymal chondrosarcoma (MCS) is a rare malignant chondrosarcoma with a high propensity for recurrence and distant metastasis. MCS usually arises from bone tissue, and rarely occurs outside the bone. MCS in the subdural and extramedullary regions of the spinal cord is especially rare. In this article, we report a case of spinal intradural extramedullary MCS with herpes virus infection, which is the first such case reported in East China. Case Description A 13-year-old male complained of intermittent low-grade fever, sweating, progressive constipation with weakness of both lower extremities and bilateral hypoesthesia after a 5-month history of herpes virus infection. Spinal magnetic resonance imaging (MRI) revealed a subdural-extramedullary solid nodular mass with isointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging that was located behind the superior margin of the T5 vertebral body. The patient was initially diagnosed with thoracic meningioma and underwent spinal cord tumour resection followed by adjuvant chemotherapy. Histopathological examination revealed that the tumour was mainly composed of round or oval cells and mesenchymal chondroid matrix, and gene analysis showed the fusion of HEY1 exon 4 to NCOA2 exon 13. Both test results were consistent with the diagnosis of primary intraspinal MCS. At the 1-year follow-up, the patient received adjuvant chemotherapy, and the reexamination images revealed no evidence of tumour in situ tumour recurrence or distant metastasis. Conclusions As more research has been done on MCS, it has been found that the disease is more likely to occur in adolescents, but is often overlooked due to its lack of imaging characterization. Therefore, the misdiagnosis rate can be reduced only by closely considering clinical manifestations with pathology and imaging findings. Although MCS is a highly malignant tumour, early primary spinal intradural extramedullary MCS can cause neurological symptoms, early detection and treatment can achieve basic total surgical resection. Postoperative adjuvant chemoradiotherapy can further reduce recurrence.

Project description:BackgroundClassic cerebrotendinous xanthomatosis (CTX; OMIM #213700) manifests with chronic diarrhea, juvenile cataracts, tendon xanthomas and neurological symptoms. It is due to biallelic inactivation of CYP27A1 wich leads to cholestanol accumulation in the central nervous system, eyes and tendons. Less commonly, the disease can present in young adults as spastic paraparesis in the absence of xanthomas.Case presentationWe report a 38-year old woman who presented with chronic diarrhea and progressive spastic paraparesis in her twenties. Brain magnetic resonance imaging (MRI) showed cerebral atrophy with diffuse periventricular white matter hyperintensities. Spinal MRI was normal. CYP27A1 gene sequencing confirmed the diagnosis of CTX. Chenodeoxycholic acid (CDCA) treatment was introduced with remission of diarrhea. Unfortunately, the treatment had to be discontinued several times and the patient developed psychosis and an severe ataxospastic gait. Spinal MRI revealed new linear hyperintensities of the corticospinal and gracile tracts. Thirty-three spinal CTX patients were identified by searching in Pubmed, EMBASE™ and Web of Science databases. All patients presented pyramidal signs and 48% had dorsal column signs. Juvenile cataracts were described in 78% of patients, chronic diarrhea in 65%, and tendon xanthomas in 31%. Disease improvement or stabilization with chenodeoxycholic acid was observed in 69% of patients. A higher prevalence of the Arg395Cys allele was observed in patients with spinal CTX as compared to CTX in general (ᵡ2; p < 0.00001).ConclusionsThe diagnosis of spinal CTX can be easily missed or delayed in absence of xanthomas. There is a higher prevalence of the Arg395Cys allele in spinal CTX as compared to classic childhood-onset CTX. CDCA treatment seems to stabilize or improve clinical symptoms in most patients. However, as seen in our patient and in two previously reported cases, sudden interruption of CDCA may lead to irreversible neurological complications.

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Neurological emergency from rare spinal metalloma: Case report and literature review.

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Neurological emergency from rare spinal metalloma: Case report and literature review.

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