Primary cervical spine AL-kappa amyloidoma: A case report and review of the literature.

MedStar author(s):
Citation: Neuropathology. 39(3):231-239, 2019 Jun.PMID: 31044465Institution: MedStar Washington Hospital CenterDepartment: Neurosurgery | PathologyForm of publication: Journal ArticleMedline article type(s): Case ReportsSubject headings: *Amyloid | *Amyloidosis/dg [Diagnostic Imaging] | *Amyloidosis/su [Surgery] | *Cervical Vertebrae/dg [Diagnostic Imaging] | *Cervical Vertebrae/su [Surgery] | Amyloid/ip [Isolation & Purification] | Humans | Male | Middle AgedYear: 2019ISSN:
  • 0919-6544
Name of journal: Neuropathology : official journal of the Japanese Society of NeuropathologyAbstract: Copyright (c) 2019 Japanese Society of Neuropathology.Of the myriad of variants of amyloidoses where abnormally folded proteins damage native tissue, primary cervical spine amyloidoma represents one of the rarest forms. Since clinical presentations and imaging findings appear similar to other pathologies, including abscesses, metastatic lesions, and inflammatory lesions, a definitive diagnosis requires a biopsy with specific immunohistochemical stains. We present the first known case of primary cervical amyloid light-chain (AL)-kappa subtype amyloidoma and compare the clinical presentations, imaging findings, treatment options, and immunohistochemical subtypes of primary, hemodialysis, and multiple myeloma cervical amyloidomas. Our case is of a 58-year-old man who developed neck pain radiating to the left arm with bilateral upper extremity weakness over several months. Magnetic resonance imaging revealed a circumferential C1-C2 mass extending into the neural foramina inducing severe mass effect. The patient underwent C2 laminectomy and resection of the lesion which was discovered during surgery to be completely epidural. Postoperatively, his pain and weakness improved. A complete work-up was negative for systemic amyloidosis or inflammatory conditions. In the setting of a long clinical history of hemodialysis, this patient required specific staining and laboratory testing to correctly diagnose his primary cervical AL-kappa subtype amyloidoma. Cervical amyloidomas comprise a very small minority of amyloid pathology with an exceptional prognosis following successful surgical resection and stabilization. It is recommended these patients undergo surgical resection with appropriate characterization and a complete work-up to rule out systemic disease.All authors: Deng T, Dowlati E, Jha RT, Mason RB, Rotter JOriginally published: Neuropathology. 2019 May 01Fiscal year: FY2019Digital Object Identifier: ORCID: Date added to catalog: 2019-05-21
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Journal Article MedStar Authors Catalog Article 31044465 Available 31044465

Copyright (c) 2019 Japanese Society of Neuropathology.

Of the myriad of variants of amyloidoses where abnormally folded proteins damage native tissue, primary cervical spine amyloidoma represents one of the rarest forms. Since clinical presentations and imaging findings appear similar to other pathologies, including abscesses, metastatic lesions, and inflammatory lesions, a definitive diagnosis requires a biopsy with specific immunohistochemical stains. We present the first known case of primary cervical amyloid light-chain (AL)-kappa subtype amyloidoma and compare the clinical presentations, imaging findings, treatment options, and immunohistochemical subtypes of primary, hemodialysis, and multiple myeloma cervical amyloidomas. Our case is of a 58-year-old man who developed neck pain radiating to the left arm with bilateral upper extremity weakness over several months. Magnetic resonance imaging revealed a circumferential C1-C2 mass extending into the neural foramina inducing severe mass effect. The patient underwent C2 laminectomy and resection of the lesion which was discovered during surgery to be completely epidural. Postoperatively, his pain and weakness improved. A complete work-up was negative for systemic amyloidosis or inflammatory conditions. In the setting of a long clinical history of hemodialysis, this patient required specific staining and laboratory testing to correctly diagnose his primary cervical AL-kappa subtype amyloidoma. Cervical amyloidomas comprise a very small minority of amyloid pathology with an exceptional prognosis following successful surgical resection and stabilization. It is recommended these patients undergo surgical resection with appropriate characterization and a complete work-up to rule out systemic disease.

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