Open AccessCase Report Synchronous Bilateral Carotid Body and Bilateral Vagal Paragangliomas: A Case Report and Literature Review 1 Department of Surgery, College of Medicine, Imam Mohammad Ibn Saud Islamic University (IMSIU), Riyadh 11564, Saudi Arabia 2 Department of Surgery, Division of Vascular Surgery, Security Forces Hospital, Riyadh 11564, Saudi Arabia 3 Division of Vascular Surgery and Endovascular Therapy, Department of Surgery, King Faisal Specialist Hospital and Research Center (KFSH&RC), Riyadh 12713, Saudi Arabia 4 Department of Otolaryngology-Head and Neck Surgery, Security Forces Hospital Program, Riyadh 11564, Saudi Arabia 5 College of Medicine, Alfaisal University, Riyadh 11533, Saudi Arabia 6 Division of Vascular Surgery, Department of Surgery, University of Toronto, Toronto, ON M5S 1A1, Canada * Author to whom correspondence should be addressed. J. Clin. Med. 2026, 15(12), 4463; https://doi.org/10.3390/jcm15124463 (registering DOI) Submission received: 10 May 2026 / Revised: 5 June 2026 / Accepted: 6 June 2026 / Published: 9 June 2026 Abstract Background: Head and neck paragangliomas (HNPGLs) are rare neuroendocrine neoplasms that may be sporadic or hereditary and may occur as isolated, multifocal, or syndromic disease within the pheochromocytoma-paraganglioma spectrum. Synchronous bilateral carotid body tumors (CBTs) and bilateral vagal paragangliomas (VPGLs) are exceptional, particularly when biochemical activity and metastatic nodal involvement coexist. Case Presentation: We report a 33-year-old man with a 2-year history of enlarging bilateral neck masses, positive family history, and markedly elevated noradrenaline (6430 pg/mL; reference range <750 pg/mL). Initial CT angiography suggested bilateral CBTs, and Metaiodobenzylguanidine (MIBG) scintigraphy did not demonstrate abnormal adrenal or distant uptake. After alpha- and beta-blockade, staged surgery was performed. Right CBT excision demonstrated metastatic PGL, with two of four lymph nodes positive. Subsequent MRI and operative reassessment revealed synchronous bilateral CBTs and bilateral VPGLs. Left-sided surgery required partial debulking of a vagal-adherent mass to preserve nerve continuity; pathology confirmed PGL with Ki-67 index approximately 2% and left neck nodes were negative. Postoperatively, profound bradycardia required temporary then permanent pacing, together with bilateral vocal cord paralysis. During follow-up, swallowing and voice improved, the pacemaker was removed, and late imaging showed stable residual cervical disease without visceral metastasis on chest/abdominal CT. Conclusions: This case highlights the diagnostic and therapeutic complexity of multicentric, biochemically active HNPGLs and supports individualized multidisciplinary management, genetic counseling, biochemical surveillance, and long-term follow-up. 1. Introduction Synchronous involvement of CBTs and vagal PGLs (VPGLs) in the same patient remains rare, and reports combining bilateral CBTs with bilateral VPGLs are exceptional [ 12, 13]. The objective of this report was to describe a rare case of synchronous bilateral CBTs and bilateral VPGLs with biochemical activity, histologically confirmed regional nodal metastasis, severe postoperative bradyarrhythmia, and delayed functional recovery, and to contextualize this case against the available literature on multicentric cervical PGLs. Current evidence supports a hereditary framework for a substantial proportion of HNPGLs, and guideline-oriented studies recommend genetic counseling and consideration of germline testing for all patients with PGL, particularly when the disease is bilateral, multifocal, metastatic, or presents at a relatively young age [ 3, 28]. Up to 40% of HNPGLs are associated with genetic mutations, predominantly in the succinate dehydrogenase (SDH) complex genes (SDHA, SDHB, SDHC, SDHD) [ 3, 29, 30]. SDHD mutations are classically associated with multifocal parasympathetic HNPGL and demonstrate a parent-of-origin effect, with disease manifesting predominantly after paternal inheritance. In contrast, SDHB mutations are more often associated with extra-adrenal sympathetic PGLs and a higher risk of metastatic disease than SDHD-associated tumors [ 31, 32, 33]. In the large Papaspyrou series of 175 patients, multifocality and mutation-positive disease were important recurring themes in clinical evaluation [ 17]. More recently, Bellamkonda et al. analyzed 49 patients with bilateral HNPGLs comprising 116 tumors, including 90 CBTs and 15 VPGLs, further highlighting that bilateral disease is a distinct management subset [ 34]. These observations are highly relevant to the present patient because he presented at 33 years of age with bilateral cervical masses and a positive family history of relatives with neck masses. Although formal molecular testing had not been performed, this phenotype strongly supports referral for genetic counseling, germline testing, family evaluation, and long-term surveillance [ 35 Standard anatomical evaluation relies on contrast-enhanced CT and MRI, which define carotid bifurcation splaying, cranial extension, skull-base proximity, and vascular encasement [ 3, 37]. However, in multifocal or closely adjacent lesions, anatomical distinction may be difficult when one tumor distorts the tissue planes of another. In the present case, the diagnosis of synchronous bilateral CBTs and bilateral VPGLs was established only after integration of operative findings, MRI, and detailed radiological re-review. The modern diagnostic pathway has also evolved beyond anatomic imaging alone. Lin et al. recommended 68Ga-DOTATATE PET/CT as first-line functional imaging when multifocal or metastatic disease is suspected [ 3]. Janssen et al. demonstrated that 68Ga-DOTATATE PET/CT localized more HNPGL lesions than conventional anatomical imaging and other functional modalities in parasympathetic HNPGLs [ 10]. Although MIBG scintigraphy did not demonstrate adrenal or distant uptake at initial work-up, current evidence supports 68Ga-DOTATATE PET/CT as the preferred functional imaging modality when multifocal or metastatic HNPGL is suspected [ 11 Management of HNPGLs has shifted from a uniformly aggressive surgical approach toward individualized decision-making that balances tumor control against long-term function [ 14]. Factors influencing treatment choice include age, comorbidity, tumor size and site, bilaterality, growth, secretory status, baseline cranial nerve function, metastatic behavior, and patient preference [ 14, 28]. CBTs and VPGLs do not carry equivalent operative risk. In the bilateral HNPGL series by Bellamkonda et al., CBTs had the lowest risk of postoperative cranial nerve deficits after surgery compared with vagal and jugular lesions [ 34]. Del Guercio et al. reported preoperative cranial nerve palsy in 36% of patients, new postoperative nerve dysfunction in 16%, and persistent deficits in 12% in a mixed cervical series managed at an experienced center [ 43]. For catecholamine-secreting tumors, preoperative alpha-adrenergic blockade before beta-blockade is recommended to reduce perioperative adrenergic risk [ 9]. For bilateral tumors, concurrent bilateral resection is generally avoided because of the risk of bilateral lower cranial nerve palsy and baroreflex failure [ 44]. The staged, single-center multidisciplinary strategy used in the present case, with right-sided resection performed first and left-sided surgery undertaken subsequently after recognition of dense vagal adherence, is consistent with a function-conscious approach in bilateral or multicentric HNPGLs [ 45]. Complete resection was not pursued at the cost of definite nerve sacrifice; instead, partial debulking was undertaken to preserve nerve continuity. This approach aligns with literature supporting selective resection, radiotherapy, or surveillance in anatomically high-risk lesions rather than routine radical excision [ 28, 40]. Functional morbidity remains one of the most important determinants of outcome in HNPGL management, especially when the vagus nerve is involved. Lower cranial nerve dysfunction may manifest as hoarseness, dysphagia, aspiration risk, shoulder dysfunction, or the need for voice and swallowing interventions. Neskey et al. emphasized that postoperative outcomes after HNPGL resection should be judged not only by tumor control, but also by functionally meaningful endpoints such as feeding-tube or tracheostomy dependence, need for vocal fold medialization, and cerebrovascular complications [ 46]. In the current case, the postoperative bilateral vocal cord paralysis and transient swallowing difficulty reflected the recognized functional burden of surgery for vagal-region disease. The most distinctive complication was the severe postoperative bradyarrhythmia requiring temporary and then permanent pacing, followed by later pacemaker removal after recovery. Major bradycardic events of this severity are uncommon in the HNPGL literature but biologically plausible through carotid sinus hypersensitivity, vagal reflex activation, or manipulation of autonomic structures during surgery [ 47]. Bauer et al. described carotid sinus hypersensitivity after preoperative embolization of a CBT that required temporary pacemaker placement before definitive resection [ 48], and Duan et al. reported intraoperative cardiac arrest during CBT resection attributable to carotid sinus hypersensitivity [ 25]. The present case indicates that profound postoperative bradycardia may occur in parallel with vagal-related laryngeal dysfunction after complex HNPGLs surgery and that both complications may improve over prolonged multidisciplinary follow-up. Limitations of the Current Case Report Several limitations should be acknowledged. First, endocrine characterization was incomplete; biochemical evaluation showed markedly elevated noradrenaline, but plasma or urinary metanephrines, normetanephrine, dopamine, methoxytyramine, serotonin/5-hydroxyindoleacetic acid, and serial postoperative biochemical values were not measured. Second, germline testing of the patient and endocrine/genetic evaluation of relatives were not performed despite young age, family history, bilateral disease, multifocality, and metastatic nodal involvement. Third, radiological and pathological characterization relied on formal reports because source MIBG images, pathological slides, detailed immunohistochemistry, and pathological micrographs were not provided for publication. Additionally, recognized predictors of difficult resections, such as the distance of the tumor to the base of the skull [ 49], were not assessed. Fourth, the T2 vertebral lesion was radiologically suspicious but not histologically confirmed. These limitations reinforce the need for comprehensive biochemical testing, genetic counseling, functional imaging, family assessment, and structured long-term surveillance in similar cases. This real-world case demonstrates a rare presentation of synchronous bilateral CBTs with bilateral VPGLs in a young patient with positive family history, biochemical activity, histologically confirmed regional nodal metastasis, staged surgical management, severe postoperative bradyarrhythmia, bilateral vocal cord dysfunction, and delayed functional recovery. Several practical messages can be drawn. First, bilateral or multifocal HNPGLs should prompt consideration of hereditary disease, genetic counseling, family evaluation, and long-term surveillance. Second, careful re-review of imaging and, where available, contemporary functional imaging may reveal multicentric disease not fully appreciated on initial assessment. Third, treatment should be individualized, particularly when vagal involvement creates a high risk of permanent lower cranial nerve morbidity. Finally, rare cardiovascular complications such as carotid sinus hypersensitivity or vagally mediated bradyarrhythmia should remain within the perioperative differential in complex HNPGL surgery. Despite the severe presentation, the patient’s subsequent functional improvement, pacemaker removal, stable residual cervical disease, and absence of visceral metastasis on late chest/abdominal imaging suggest a favorable clinical course to date, while continuing to justify prolonged multidisciplinary surveillance. Author Contributions N.A. and T.A. drafted the main manuscript text. Y.A., M.D., H.G., M.A. and O.T.A. contributed to the literature review and preparation of the figures. All authors have read and agreed to the published version of the manuscript. The work was supported and funded by the Deanship of Scientific Research at Imam Mohammad Ibn Saud Islamic University (IMSIU) (grant number IMSIU-DDRSP2601). Institutional Review Board Statement Institutional ethics approval was waived for this study, due to it being a single-case report and in accordance with local institutional policy. Informed Consent Statement Written informed consent has been obtained from the patient(s) to publish this paper. Data Availability Statement All data generated or analyzed during this study are included in this published article. Additional details are available from the corresponding author upon reasonable request. Conflicts of Interest The authors declare no conflicts of interest. Abbreviations The following abbreviations are used in this manuscript: ARDS acute respiratory distress syndrome CBT Carotid body tumor CT Computed tomography CTA Computed tomography angiography MRI Magnetic resonance imaging HNPGL Head and neck paraganglioma VPGL Vagal paraganglioma PGL Paraganglioma MIBG Metaiodobenzylguanidine VMA Vanillylmandelic acid PTFE Polytetrafluoroethylene References Graham, N.J.; Smith, J.D.; Else, T.; Basura, G.J. Paragangliomas of the head and neck: A contemporary review. Endocr. Oncol. 2022, 2, R153. [ Google Scholar] [ CrossRef] [ PubMed] Prasad, S.C.; Paties, C.T.; Pantalone, M.R.; Mariani-Costantini, R.; Sanna, M. Carotid Body and Vagal Paragangliomas: Epidemiology, Genetics, Clinicopathological Features, Imaging, and Surgical Management. In Paraganglioma: A Multidisciplinary Approach; Exon Publications: Brisbane, Australia, 2019; pp. 81–98. [ Google Scholar] [ CrossRef] Lin, E.P.; Chin, B.B.; Fishbein, L.; Moritani, T.; Montoya, S.P.; Ellika, S.; Newlands, S. Head and Neck Paragangliomas: An Update on the Molecular Classification, State-of-the-Art Imaging, and Management Recommendations. Radiol. Imaging Cancer 2022, 4, E210088. [ Google Scholar] [ CrossRef] Benn, D.E.; Robinson, B.G.; Clifton-Bligh, R.J. 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1–5. Endocr. Relat. Cancer 2015, 22, T91. [ Google Scholar] [ CrossRef] Offergeld, C.; Brase, C.; Yaremchuk, S.; Mader, I.; Rischke, H.C.; Gläsker, S.; Schmid, K.W.; Wiech, T.; Preuss, S.F.; Suárez, C.; et al. Head and neck paragangliomas: Clinical and molecular genetic classification. Clinics 2012, 67, 19–28. [ Google Scholar] [ CrossRef] Smith, J.D.; Ellsperman, S.E.; Basura, G.J.; Else, T. Re-evaluating the prevalence and factors characteristic of catecholamine secreting head and neck paragangliomas. Endocrinol. Diabetes Metab. 2021, 4, e00256. [ Google Scholar] [ CrossRef] Zheng, X.; Wei, S.; Yu, Y.; Xia, T.; Zhao, J.; Gao, S.; Li, Y.; Gao, M. Genetic and clinical characteristics of head and neck paragangliomas in a Chinese population. Laryngoscope 2012, 122, 1761–1766. [ Google Scholar] [ CrossRef] Richter, S.; Qiu, B.; Ghering, M.; Kunath, C.; Constantinescu, G.; Luths, C.; Pamporaki, C.; Bechmann, N.; Meuter, L.; Kwapiszewska, A.; et al. Head/neck paragangliomas: Focus on tumor location, mutational status and plasma methoxytyramine. Endocr. Relat. Cancer 2022, 29, 213–224. [ Google Scholar] [ CrossRef] Lenders, J.W.M.; Duh, Q.-Y.; Eisenhofer, G.; Gimenez-Roqueplo, A.-P.; Grebe, S.K.G.; Murad, M.H.; Naruse, M.; Pacak, K.; Young, W.F., Jr. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J. Clin. Endocrinol. Metab. 2014, 99, 1915–1942. [ Google Scholar] [ CrossRef] [ PubMed] Janssen, I.; Chen, C.C.; Taieb, D.; Patronas, N.J.; Millo, C.M.; Adams, K.T.; Nambuba, J.; Herscovitch, P.; Sadowski, S.M.; Fojo, A.T.; et al. 68Ga-DOTATATE PET/CT in the Localization of Head and Neck Paragangliomas Compared with Other Functional Imaging Modalities and CT/MRI. J. Nucl. Med. 2016, 57, 186–191. [ Google Scholar] [ CrossRef] Maurice, J.B.; Troke, R.; Win, Z.; Ramachandran, R.; Al-Nahhas, A.; Naji, M.; Dhillo, W.; Meeran, K.; Goldstone, A.P.; Martin, N.M.; et al. A comparison of the performance of 68Ga-DOTATATE PET/CT and 123I-MIBG SPECT in the diagnosis and follow-up of phaeochromocytoma and paraganglioma. Eur. J. Nucl. Med. Mol. Imaging 2012, 39, 1266–1270. [ Google Scholar] [ CrossRef] [ PubMed] Kataria, T.; Bisht, S.S.; Mitra, S.; Abhishek, A.; Potharaju, S.; Chakarvarty, D. Synchronous malignant vagal paraganglioma with contralateral carotid body paraganglioma treated by radiation therapy. Rare Tumors 2010, 2, 57–59. [ Google Scholar] [ CrossRef] Karatas, E.; Sirikci, A.; Baglam, T.; Mumbuc, S.; Durucu, C.; Tutar, E.; Kanlikama, M. Synchronous bilateral carotid body tumor and vagal paraganglioma: A case report and review of literature. Auris Nasus Larynx 2008, 35, 171–175. [ Google Scholar] [ CrossRef] Cleere, E.F.; Martin-Grace, J.; Gendre, A.; Sherlock, M.; O’Neill, J.P. Contemporary management of paragangliomas of the head and neck. Laryngoscope Investig. Otolaryngol. 2021, 7, 93. [ Google Scholar] [ CrossRef] Thabet, M.H.; Kotob, H. Cervical paragangliomas: Diagnosis, management and complications. J. Laryngol. Otol. 2001, 115, 467–474. [ Google Scholar] [ CrossRef] Sobol, S.M.; Dailey, J.C. Familial multiple cervical paragangliomas: Report of a kindred and review of the literature. Otolaryngol. Head Neck Surg. 1990, 102, 382–390. [ Google Scholar] [ CrossRef] [ PubMed] Papaspyrou, K.; Mewes, T.; Rossmann, H.; Fottner, C.; Schneider-Raetzke, B.; Bartsch, O.; Schreckenberger, M.; Lackner, K.J.; Amedee, R.G.; Mann, W.J. Head and neck paragangliomas: Report of 175 patients (1989–2010). Head. Neck 2012, 34, 632–637. [ Google Scholar] [ CrossRef] [ PubMed] Kawanabe, S.; Katabami, T.; Oshima, R.; Yanagisawa, N.; Sone, M.; Kimura, N. A rare case of multiple paragangliomas in the head and neck, retroperitoneum and duodenum: A case report and review of the literature. Front. Endocrinol. 2023, 13, 1054468. [ Google Scholar] [ CrossRef] Lemaire, M.; Persu, A.; Hainaut, P.; De Plaen, J.F. Hereditary paraganglioma. J. Intern. Med. 1999, 246, 113–116. [ Google Scholar] [ CrossRef] Velegrakis, G.; Kalikakis, G.; Karampekios, S.; Stefanaki, K.; Helidonis, E. Bilateral paraganglioma of the vagus nerve. HNO 2001, 49, 471–475. [ Google Scholar] [ CrossRef] Pandey, M.; Chandramohan, K.; Sebastian, P.; Ramachandran, K. An unusual bilateral cervical paraganglioma: A case report. Int. J. Oral. Maxillofac. Surg. 2002, 31, 334–336. [ Google Scholar] [ CrossRef] Bakshi, J.; Goyal, A.K.; Vir, D.; Panda, N.K. Multiple paragangliomas involving carotid body and vagal region: A sporadic case. Otorhinolaryngol. Head Neck Surg. 2019, 4, 1–3. [ Google Scholar] [ CrossRef] Yepuri, N.; Vanga, G.R.; Naous, R.; Kinthala, S. Thyroid paraganglioma in a patient with a history of carotid and vagal paraganglioma: Metastatic or primary tumor? J. Surg. Case Rep. 2021, 2021, rjab102. [ Google Scholar] [ CrossRef] Moscona-Nissan, A.; A Saldívar-Rodea, C.; Enríquez-García, R.; Rincón-Ángel, L.I.; Calzada, A.N.; Seidman-Sorsby, A.; Cruz-Zermeño, M. Bilateral carotid body tumor case: A novel preoperative management. Radiol. Case Rep. 2022, 17, 1021–1029. [ Google Scholar] [ CrossRef] [ PubMed] Duan, H.Y.; Guan, Q.; Guo, Y.J.; Liang, N. Case report: Cardiac arrest during carotid body tumor resection indicating carotid sinus hypersensitivity. Front. Cardiovasc. Med. 2022, 9, 996644. [ Google Scholar] [ CrossRef] Sakaran, K.R.C.; Tiew, T.; Kamil, K.; Khan, H.A.H.; Idris, M.A.; Safri, L.S. Bilateral carotid body tumours: A case report from surgeon’s perspective. J. Surg. Case Rep. 2024, 2024, rjae011. [ Google Scholar] [ CrossRef] Cidral, L.S.R.; Penzo, L.D.; Merida, K.B.; Marques, M.; Andrade, A.C.; Aragão, A.H.; Ruschel, L.G. Bilateral Carotid Body Paraganglioma: A Case Report and Review of Management Strategies. J. Neurol. Surg. Rep. 2025, 86, e175–e179. [ Google Scholar] [ CrossRef] Cass, N.D.; Schopper, M.A.; Lubin, J.A.; Fishbein, L.; Gubbels, S.P. The Changing Paradigm of Head and Neck Paragangliomas: What Every Otolaryngologist Needs to Know. Ann. Otol. Rhinol. Laryngol. 2020, 129, 1135–1143. [ Google Scholar] [ CrossRef] [ PubMed] Palade, D.O.; Hainarosie, R.; Zamfir, A.; Vrinceanu, D.; Pertea, M.; Tusaliu, M.; Mocanu, F.; Voiosu, C. Paragangliomas of the Head and Neck: A Review of the Latest Diagnostic and Treatment Methods. Medicina 2024, 60, 914. [ Google Scholar] [ CrossRef] Vimawala, S.N.; Graboyes, A.Z.; Bennett, B.; Bonanni, M.; Abbasi, A.; Oliphant, T.; Alonso-Basanta, M.; Rassekh, C.; Cohen, D.; Brant, J.A.; et al. Head and Neck Paragangliomas: Overview of Institutional Experience. Cancers 2024, 16, 1523. [ Google Scholar] [ CrossRef] Else, T.; Greenberg, S.; Fishbein, L. Hereditary Paraganglioma-Pheochromocytoma Syndromes. GeneReviews ପ୍ପ. 2023. Available online: https://www.ncbi.nlm.nih.gov/books/NBK1548/ (accessed on 15 March 2026). Neumann, H.P.; Pawlu, C.; Peczkowska, M.; Bausch, B.; McWhinney, S.R.; Muresan, M.; Buchta, M.; Franke, G.; Klisch, J.; Bley, T.A.; et al. Distinct Clinical Features of Paraganglioma Syndromes Associated With SDHB and SDHD Gene Mutations. JAMA 2004, 292, 943–951. [ Google Scholar] [ CrossRef] Kantorovich, V.; King, K.S.; Pacak, K. SDH-related Pheochromocytoma and paraganglioma. Best Pract. Res. Clin. Endocrinol. Metab. 2010, 24, 415. [ Google Scholar] [ CrossRef] [ PubMed] Bellamkonda, N.; Tooker, E.L.; Naumer, A.; Buchmann, L.O.; Kohlmann, W.; McCrary, H.C.; Patel, N.S.; Espahbodi, M. Management of bilateral head and neck paragangliomas at a single-institution across four decades. Head Neck 2025, 47, 386–393. [ Google Scholar] [ CrossRef] Gupta, N.; Strome, S.E.; Hatten, K.M. Is routine genetic testing warranted in head and neck paragangliomas? Laryngoscope 2019, 129, 1491–1493. [ Google Scholar] [ CrossRef] Jansen, J.C.; Van Den Berg, R.; Kuiper, A.; Van Der Mey, A.G.L.; Zwinderman, A.H.; Cornelisse, C.J. Estimation of Growth Rate in Patients with Head and Neck Paragangliomas Influences the Treatment Proposal. Cancer 2000, 88, 2811–2816. [ Google Scholar] [ CrossRef] [ PubMed] Sandow, L.; Thawani, R.; Kim, M.S.; Heinrich, M.C. Paraganglioma of the Head and Neck: A Review. Endocr. Pract. 2023, 29, 141–147. [ Google Scholar] [ CrossRef] Fang, F.; Ding, L.; He, Q.; Liu, M. Preoperative Management of Pheochromocytoma and Paraganglioma. Front. Endocrinol. 2020, 11, 586795. [ Google Scholar] [ CrossRef] Shamblin, W.R.; ReMine, W.H.; Sheps, S.G.; Harrison, E.G. Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am. J. Surg. 1971, 122, 732–739. [ Google Scholar] [ CrossRef] Richter, S.; Constantinescu, G.; Fancello, G.; Paties, C.T.; Mariani-Costantini, R.; Sanna, M. Head and neck paragangliomas: Recent advances in translational and clinical research and guidelines for patient care. Best Pract. Res. Clin. Endocrinol. Metab. 2024, 38, 101951. [ Google Scholar] [ CrossRef] Magliulo, G.; Zardo, F.; Varacalli, S.; D’Amico, R. Multiple paragangliomas of the head and neck. Otorrinolaringol. Ibero Am. 2003, 30, 31–38. [ Google Scholar] Hamidi, O.; Young, W.F., Jr.; Gruber, L.; Smestad, J.; Yan, Q.; Ponce, O.J.; Prokop, L.; Murad, M.H.; Bancos, I. Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: A systematic review and meta-analysis. Clin. Endocrinol. 2017, 87, 440–450. [ Google Scholar] [ CrossRef] Del Guercio, L.; Narese, D.; Ferrara, D.; Butrico, L.; Padricelli, A.; Porcellini, M. Carotid and Vagal Body Paragangliomas. Transl. Med. UniSa 2013, 6, 11. [ Google Scholar] [ PubMed] Bobadilla-Rosado, L.O.; Garcia-Alva, R.; Anaya-Ayala, J.E.; Peralta-Vazquez, C.; Hernandez-Sotelo, K.; Luna, L.; Cuen-Ojeda, C.; Hinojosa, C.A. Surgical Management of Bilateral Carotid Body Tumors. Ann. Vasc. Surg. 2019, 57, 187–193. [ Google Scholar] [ CrossRef] Piazza, C.; Montenegro, C.; Rampinelli, V. Bilateral carotid body tumor management: Tips, tricks, strategies, and problems. Curr. Opin. Otolaryngol. Head Neck Surg. 2025, 33, 123–130. [ Google Scholar] [ CrossRef] Neskey, D.M.; Hatoum, G.; Modh, R.; Civantos, F.; Telischi, F.F.; Angeli, S.; Weed, D.; Sargi, Z. Outcomes after surgical resection of head and neck paragangliomas: A review of 61 patients. Skull Base 2011, 21, 171–176. [ Google Scholar] [ CrossRef] [ PubMed] Suhail, K.; Kuldeep, R.; Ajaz, W.; Farzana, F. Heart rhythm irregularities during laparoscopy: Description of a novel technique to manage severe intra-operative bradycardia—A case report. Int. J. Surg. Case Rep. 2023, 105, 107997. [ Google Scholar] [ CrossRef] Bauer, A.M.; Smith, R.B.; Thorell, W.E. Implications of carotid sinus hypersensitivity following preoperative embolization of a carotid body tumor. An indication for prophylactic intraoperative cardiac pacing. JAMA Otolaryngol. Head. Neck Surg. 2014, 140, 459–463. [ Google Scholar] [ CrossRef] [ PubMed] Alanezi, T.; Alomran, F.; Koussayer, S.; Abdulrahim, O.; Dahman, M.; Alsuhaibani, E.; Alokaili, R.; Al-Omran, M. Preoperative radiological features in predicting complications of carotid body tumor resection. J. Vasc. Surg. 2025, 81, 665–671.e2. [ Google Scholar] [ CrossRef] [ PubMed] Figure 1. CT angiography of the neck on 8 March 2020 showing bilateral hypervascular carotid bifurcation masses with splaying of the carotid vessels, larger on the right side. Figure 1. CT angiography of the neck on 8 March 2020 showing bilateral hypervascular carotid bifurcation masses with splaying of the carotid vessels, larger on the right side. Figure 2. Magnetic resonance imaging (MRI) of the neck demonstrates bilateral paraganglioma-related lesions. ( a) Axial contrast-enhanced MRI showing bilateral hypervascular masses at the carotid bifurcations with characteristic vascular flow voids. ( b) Coronal view demonstrating the extent of the lesions and their relationship to adjacent vascular structures, with separation of right-sided lesions suggesting distinct tumors. ( c) Sagittal view highlighting mass effect on the upper aerodigestive tract, including indentation of the hypopharynx. Figure 2. Magnetic resonance imaging (MRI) of the neck demonstrates bilateral paraganglioma-related lesions. ( a) Axial contrast-enhanced MRI showing bilateral hypervascular masses at the carotid bifurcations with characteristic vascular flow voids. ( b) Coronal view demonstrating the extent of the lesions and their relationship to adjacent vascular structures, with separation of right-sided lesions suggesting distinct tumors. ( c) Sagittal view highlighting mass effect on the upper aerodigestive tract, including indentation of the hypopharynx. Figure 3. Schematic illustration of the distribution of synchronous bilateral carotid body and bilateral vagal paragangliomas. Enlarged right cervical lymph nodes represent histologically confirmed nodal metastatic involvement, and the T2 vertebral body lesion indicates a radiologically suspected site of possible metastasis. This schematic is not drawn to scale. The schematic was initially hand-drawn by the authors and subsequently enhanced using FigureLabs ( https://www.figurelabs.ai/) and BioRender ( https://www.biorender.com/). Created in BioRender. Alanezi et.al. (2026) https://BioRender.com/h42n006. Figure 3. Schematic illustration of the distribution of synchronous bilateral carotid body and bilateral vagal paragangliomas. Enlarged right cervical lymph nodes represent histologically confirmed nodal metastatic involvement, and the T2 vertebral body lesion indicates a radiologically suspected site of possible metastasis. This schematic is not drawn to scale. The schematic was initially hand-drawn by the authors and subsequently enhanced using FigureLabs ( https://www.figurelabs.ai/) and BioRender ( https://www.biorender.com/). Created in BioRender. Alanezi et.al. (2026) https://BioRender.com/h42n006. Figure 4. ( a) Axial Magnetic Resonance Angiography (MRA) of the neck demonstrating postoperative status with no residual tumor at the carotid bifurcation, consistent with complete resection of the carotid body tumor. ( b) Coronal MRA view showing persistent residual mass along the expected course of the vagus nerve, consistent with residual vagal paraganglioma following prior partial debulking, with no interval progression or new lesions identified. Figure 4. ( a) Axial Magnetic Resonance Angiography (MRA) of the neck demonstrating postoperative status with no residual tumor at the carotid bifurcation, consistent with complete resection of the carotid body tumor. ( b) Coronal MRA view showing persistent residual mass along the expected course of the vagus nerve, consistent with residual vagal paraganglioma following prior partial debulking, with no interval progression or new lesions identified. Timeline of clinical presentation, diagnostic evaluation, surgical management, pathology, postoperative course, follow-up imaging, and approximate intervals in a patient with bilateral cervical paraganglioma-related masses. Timeline of clinical presentation, diagnostic evaluation, surgical management, pathology, postoperative course, follow-up imaging, and approximate intervals in a patient with bilateral cervical paraganglioma-related masses. Date/Approximate Interval Clinical Milestone Key Documented Details Before 8 March 2020 (approximately 2-year symptom history before baseline imaging) Initial presentation The patient presented with bilateral neck masses that had gradually increased in size over 2 years. 8 March 2020 (baseline imaging) Initial CT angiography Bilateral enhancing lesions/tumors were identified at the bifurcation of both carotid arteries, encasing the internal carotid arteries and splaying the external carotid arteries bilaterally. 13 August 2020 (approximately 5 months after baseline CT) First operation Excision of a right CBT was performed. Intraoperatively, a large circumscribed CBT with multiple enlarged lymph nodes was identified. After 13 August 2020 (post-first operation) First histopathology Histopathological review, including second-opinion assessment, showed metastatic PGL, with two of four lymph nodes positive for tumor. 7 October 2020 (approximately 8 weeks after first operation) MRI neck Bilateral lobulated mass. The right-sided mass indented the right hypopharynx and mildly narrowed the oropharyngeal lumen. Multiple signal-void areas were present within the tumors. Slightly prominent posterior triangle lymph nodes were noted. A small enhancing T2 vertebral body lesion was also reported as concerning for possible metastasis. After MRI review (October 2020) Revised anatomical interpretation Initial cross-sectional imaging had suggested a single large parapharyngeal mass; however, intraoperative findings and subsequent detailed radiologic review established two anatomically distinct lesions, consistent with a CBT and a VPGL. 1 October 2020 (approximately 7 weeks after first operation) Second admission The patient was admitted for the second surgery. Hematology, endocrinology, ENT, and cardiology teams were consulted, and he was cleared for surgery. 12 October 2020 (approximately 2 months after first operation) Second operation A well-circumscribed tumor at the carotid bifurcation was resected, consistent with a CBT, and a second, anatomically distinct mass was identified that was predominantly adherent to the left vagus nerve. Debulking was performed, and a remnant was left in situ to preserve nerve continuity. Full neck dissection was completed. After 12 October 2020 (post-second operation) Second histopathology Histopathology showed PGL with a Ki-67 index of approximately 2%. All left-sided neck lymph nodes harvested were uninvolved by tumor. Immediate postoperative period (October 2020) ICU course The patient was monitored in the intensive care unit and was initially extubated successfully. Early postoperative period (October 2020) Major complication After drinking water, he developed severe coughing followed by severe bradycardia with near arrest, necessitating reintubation and continued monitoring. Cardiac management Several episodes of bradycardia down to 30 beats/min were documented. Cardiology review was obtained, and a temporary pacemaker was inserted and maintained for several days. Later postoperative period (late 2020 onward) Permanent pacing Cardiology follow-up led to recommendation for permanent pacemaker insertion, which was subsequently performed. Recovery status Clinical condition gradually improved. The patient reported some dizziness but was able to ambulate without severe bradycardia; heart rate was documented as 70 beats/min. The wound was healing well. Procyclidine 2.5 mg orally twice daily was prescribed and later increased to 5 mg orally twice daily. Laryngeal complication Postoperative hoarseness was documented, and bilateral vocal cord paralysis was diagnosed. 19 November 2020 (approximately 5 weeks after second operation) Follow-up CT neck There was a reduction in the size of both left- and right-sided lesions compared with the initial imaging, with no residual tumor identified at the carotid bifurcation on either side 2023 (approximately 3 years after surgery) MRI The permanent pacemaker was removed, and the patient reported no further hoarseness or choking episodes, with good swallowing. Jan 2026 (approximately 5 years after surgery) Late follow-up imaging and staging MRA neck reportedly showed no change in size compared with the most recent prior imaging (2023), and CT chest/abdomen showed no evidence of metastasis. CT, computed tomography; ENT, ear, nose, and throat; ICU, intensive care unit; MRI, magnetic resonance imaging; MIBG, metaiodobenzylguanidine; MRA, Magnetic Resonance Angiography; PGL, paraganglioma. Published case reports of bilateral or multicentric cervical paragangliomas involving carotid body and/or vagal tumors. Published case reports of bilateral or multicentric cervical paragangliomas involving carotid body and/or vagal tumors. Study; Country Age/Sex FHx/Genetics Functional Status Presentation Imaging/Distribution Metastasis Management Complications Pathology/Outcome ARDS, acute respiratory distress syndrome; CBT, carotid body tumor; CECT, contrast-enhanced computed tomography; CN, cranial nerve; CTA, computed tomography angiography; DSA, digital subtraction angiography; ECA, external carotid artery; FHx, family history; ICA, internal carotid artery; IHC, immunohistochemistry; IGRT, image-guided radiotherapy; IMRT, intensity-modulated radiotherapy; JTP, jugulotympanic paraganglioma; MI, myocardial infarction; NF, non-functional; NR, not reported; PE, pulmonary embolism; PGL, paraganglioma; POD, postoperative day; PTFE, polytetrafluoroethylene; PVA, polyvinyl alcohol; RT, radiotherapy; SDH, succinate dehydrogenase; VPGL, vagal paraganglioma; VMA, vanillylmandelic acid. 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Synchronous Bilateral Carotid Body and Bilateral Vagal Paragangliomas: A Case Report and Literature Review. Journal of Clinical Medicine. 2026; 15(12):4463. https://doi.org/10.3390/jcm15124463 Chicago/Turabian Style Alanezi, Nahar, Yazeed Alofisan, Mohammed Dahman, Hassan Gado, Majid Althobaiti, Omar Tawfiq Abualnasr, and Tariq Alanezi. 2026. "Synchronous Bilateral Carotid Body and Bilateral Vagal Paragangliomas: A Case Report and Literature Review" Journal of Clinical Medicine 15, no. 12: 4463. https://doi.org/10.3390/jcm15124463 APA Style Alanezi, N., Alofisan, Y., Dahman, M., Gado, H., Althobaiti, M., Abualnasr, O. T., & Alanezi, T. (2026). Synchronous Bilateral Carotid Body and Bilateral Vagal Paragangliomas: A Case Report and Literature Review. Journal of Clinical Medicine, 15(12), 4463. https://doi.org/10.3390/jcm15124463 Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here. 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