Carotid body paraganglioma metastatic to spine causing cord compression: a case report

Diagnostic Pathology

Case Report

Kenneth B. Fallon¹  Jared A. Maas²  Michael C. Dobelbower²  Andrew M. McDonald²  Bhargav P. Earla³  Jazmyne N. Tabb⁴ 

[1] Department of Pathology, University of Alabama at Birmingham, Birmingham, USA;Department of Radiation Oncology, University of Alabama at Birmingham, Birmingham, USA;UAB Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, USA;University of South Alabama College of Medicine, Mobile, USA;

关键词: Carotid body tumor;    Paraganglioma;    Metastatic;    Cord compression;    Radiotherapy;   

DOI  :  10.1186/s13000-023-01314-y

 received in 2022-10-11, accepted in 2023-02-10,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundCarotid body tumors (CBTs) are rare neuroendocrine neoplasms arising near the carotid bifurcation with a reported incidence of 1 to 2 cases in 100,000 patients. Most CBTs are sporadic, benign, slow-growing, and non-secreting, but untreated CBTs can grow locally to compress the nearby blood vessels, esophagus, and airway. Regional metastases can occur in 5% to 10% of cases, but distant metastases are exceedingly rare, occurring in roughly 1–2% of cases. As such, the optimal treatment for metastatic CBTs is not well-defined. We report a rare case of a patient with CBT distant metastases causing spinal cord compression.Case presentationA 40-year-old African American female presented with a right neck mass, headaches, vertigo, tinnitus, hoarseness, and dysphagia. Imaging demonstrated a Shamblin II right neck mass; subsequent transcervical resection and pathology showed a carotid body paraganglioma. The patient recurred locally near the carotid bifurcation, so she underwent Stereotactic Body Radiation Therapy to the recurrent right neck disease. She later re-presented with new onset bilateral lower extremity weakness, dysmetria, and numbness. She was found to have metastatic disease to the thoracic spine causing spinal cord compression. She underwent laminectomy, tumor resection, and posterior fixation followed by adjuvant radiation therapy. She was started on systemic therapy with sunitinib. She eventually progressed with metastatic disease to the right iliac bone, which was treated with palliative radiotherapy. Second line systemic therapy with capecitabine and temozolomide was started. At last follow up, the patient was asymptomatic with stable persistent disease.ConclusionsParagangliomas often exhibit a prolonged interval to the development of progression; locoregional recurrences or rare distant metastases have been reported to occur as many as 20 years from diagnosis. The natural course of CBTs in other cases as well as the present case call into question the idea that CBTs are truly benign; instead CBTs may be indolent tumors with metastatic potential. Treatment choices for CBTs include surgical resection, radiation therapy, and systemic therapy, though the optimal treatment regimen for metastatic CBTs is not well-defined. A more advanced understanding of CBT pathophysiology, disease classification, risk stratification, and treatment options is needed to improve outcomes for patients.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

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Fig. 5