Experience with carotid paragangliomas at the National Rehabilitation Institute

Abstract

Introduction
Head and neck paragangliomas are rare neuroendocrine tumors, accounting for only 3% of neoplasms in this region. Carotid body tumors (CBTs) are the most common type, with an estimated incidence of 1 in 30,000 individuals, predominantly affecting women between 40 and 60 years of age [1–5]. Up to 35% are hereditary, associated with SDHx gene mutations, mainly SDHD and SDHB, which are linked to multifocality and malignancy [1,5]. Histologically, they exhibit the classic "Zellballen" pattern, and malignancy can only be confirmed by the presence of metastases in non-neuroendocrine tissues [1–3,5]. Clinically, they present as a painless, pulsatile lateral neck mass, which may cause compressive symptoms as it grows. Less than 3% are catecholamine-secreting tumors [1,2,5]. Diagnosis is based on imaging (US, CT, MRI, PET-CT) and biochemical testing. Shamblin classification predicts surgical complexity according to the tumor’s relationship with the carotid vessels [1,5]. Surgical resection is the treatment of choice, though it carries risks of neurologic and vascular morbidity. Radiotherapy is an alternative in inoperable cases. Long-term follow-up is essential due to the risk of recurrence [1–6].

Objective
To describe the institutional experience with patients diagnosed with carotid body paragangliomas at the Instituto Nacional de Rehabilitación between 2012 and 2025, focusing on clinical characteristics, diagnostic approach, treatment strategies, and postoperative outcomes.

Methodology
Data were collected from medical records. Clinical, demographic, radiological, surgical, and follow-up variables were documented using a structured data collection form. Shamblin classification, histopathological findings, and postoperative outcomes were also recorded. Descriptive analysis was performed using absolute and relative frequencies for categorical variables and measures of central tendency and dispersion for quantitative variables.

Results
A total of 13 patients with confirmed carotid body paraganglioma were included. The median age was 54 years (IQR: 47–65), with a female predominance (10 women vs. 3 men). Seven tumors were located on the left side (53.8%) and six on the right (46.2%). The average symptom duration was 12 months, and all patients were symptomatic at the time of diagnosis. Most tumors were classified as benign (84.6%), while 2 cases (15.4%) were malignant. Only one patient required lymph node dissection; two received adjuvant radiotherapy (RT), and one patient received chemotherapy (CT). Adjuvant treatment was required in 7 patients (53.8%), primarily in those with features suggestive of malignancy or advanced disease.

Conclusions
Carotid body tumors are rare but clinically relevant neoplasms that predominantly affect middle-aged women, with a generally benign behavior and symptomatic clinical course. Surgery was the mainstay of treatment, with low rates of adjuvant radiotherapy or chemotherapy. Multicenter prospective studies with larger sample sizes are needed to evaluate predictors of malignancy and to standardize the use of complementary therapies.