Malignant carotid body tumors: What we know, what we do, and what we need to achieve. A systematic review of the literature

Cesare Piazza; Davide Lancini; Michele Tomasoni; Mark Zafereo; Vincent Vander Poorten; Ehab Hanna; Antti A Mäkitie; Veronica Fernandez-Alvarez; Luiz P Kowalski; Carlos Chiesa-Estomba; Alfio Ferlito

doi:10.1002/hed.27624

Malignant carotid body tumors: What we know, what we do, and what we need to achieve. A systematic review of the literature

Head Neck. 2024 Mar;46(3):672-687. doi: 10.1002/hed.27624. Epub 2024 Jan 5.

Authors

Affiliations

¹ Unit of Otorhinolaryngology - Head and Neck Surgery, ASST Spedali Civili of Brescia, Brescia, Italy.
² Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, School of Medicine, Brescia, Italy.
³ Department of Head and Neck Surgery, MD Anderson Cancer Center, Houston, Texas, USA.
⁴ Otorhinolaryngology - Head and Neck Surgery, Leuven Cancer Institute, University Hospitals Leuven, Leuven, Belgium.
⁵ Department of Oncology, Section Head and Neck Oncology, KU Leuven, Leuven, Belgium.
⁶ Department of Otorhinolaryngology - Head and Neck Surgery, Research Program in Systems Oncology, University of Helsinki and HUS Helsinki University Hospital, Helsinki, Finland.
⁷ Department of Vascular and Endovascular Surgery, Hospital Universitario de Torrecardenas, Almeria, Spain.
⁸ Department of Head and Neck Surgery, University of Sao Paulo Medical School and Department of Head and Neck Surgery and Otorhinolaryngology, AC Camargo Cancer Center, Sao Paulo, Brazil.
⁹ Department of Otorhinolaryngology - Head and Neck Surgery, Donostia University Hospital, Deusto University - School of Medicine, BioGuipuzcoa Research Institute, San Sebastian, Spain.
¹⁰ Coordinator of the International Head and Neck Scientific Group, Padua, Italy.

PMID: 38179805
DOI: 10.1002/hed.27624

Abstract

Malignant carotid body tumors (MCBT) are rare and diagnosed after detection of nodal or distant metastases. This systematic review (SR) focuses on MCBT initially approached by surgery. Preferred Reporting Items for SR and Meta-Analysis (MA) guided the articles search from 2000 to 2023 on PubMed, Scopus, and Web of Science. Among 3548 papers, 132 (337 patients) were considered for SR; of these, 20 (158 patients) for MA. Malignancy rate was 7.3%, succinate dehydrogenase (SDH) mutation 17%, age at diagnosis between 4th and 6th decades, with a higher prevalence of females. MCBTs were mostly Shamblin III, with nodal and distant metastasis in 79.7% and 44.7%, respectively. Malignancy should be suspected if CBT >4 cm, Shamblin III, painful or otherwise symptomatic, at the extremes of age, bilateral, with multifocal disease, and SDHx mutations. Levels II-III clearance should be performed to exclude nodal metastases and adjuvant treatments considered on a case-by-case basis.

Keywords: carotid body tumor; malignant; metastasis; surgery; systematic review.

Publication types

Meta-Analysis
Systematic Review

MeSH terms

Carotid Body Tumor* / genetics
Carotid Body Tumor* / surgery
Female
Head and Neck Neoplasms*
Humans
Male
Mutation