Carotid body paragangliomas – clinical variety and management (RCD code: I-O)

Jakub Chmiel, Piotr Loska, Andrzej Brzychczy, Paweł Iwaszczuk, Jerzy Krzywoń, Katarzyna Zbierska-Rubinkiewicz, Tomasz Kwiatkowski, Julia Krzywoń, Jakub Giliavas, Mariusz Trystuła

Full Text:



Paragangliomas (PGLs) are a group of rare, slow-growing tumours which are found between the base of the skull and the pelvis. The tumour may be asymptomatic, although a lump on the neck, cranial nerve palsy, or neck pain may be present. The treatment of choice is surgical resection. We present the cases of 7 patients (6 females) diagnosed with 9 PGLs of the carotid body (carotid body tumours – CBTs). These include: one case with known genetic burden, one of an advanced bilateral and recurrent tumour, and one with a malignant
tumour. All presented CBTs were surgically removed. The size of a tumour correlates with postoperative complications. Resection of the largest lesion was associated with persistent left recurrent laryngeal, hypoglossal, and partial facial nerve paralysis. Other complications included single cranial nerve palsy and temporary Horner syndrome. JRCD 2018; 4 (1):


rare disease; carotid body paraganglioma; carotid body tumour; carotid body; paraganglioma; bifurcation of the common carotid artery


Wieneke JA, Smith A. Paraganglioma: Carotid Body Tumor. Head Neck Pathol 2009; 3: 303–306.

Moore MG, Netterville JL, Mendenhall WM, et al. Head and Neck Paragangliomas: An Update on Evaluation and Management. Otolaryngol Head Neck Surg 2016;154: 597–605.

P ȩ cak M, Pluta E, Hetnał M, et al. Role of irradiation in combined treatment of head and neck paragangliomas at the Centre of Oncology in Krakow between 1970–2005. Wspolczesna Onkol 2014;18:182–186.

Sajid MS, Hamilton G, Baker DM. A multicenter review of carotid body tumor management. Eur J Vasc Endovasc Surg 2007; 34: 127–130.

Pellitteria PK, Rinaldo A, Myssiorekm D, et al. Paragangliomas of the head and neck. Oral Oncol 2004; 40: 563–575.

Dixon JL, Atkins MD, Bohannon WT, et al. Surgical management of carotid body tumors: a 15-year single institution experience employing an interdisciplinary approach. Proceedings (Baylor University Medical Center) 2016; 29:16–20.

Gad A, Sayed A, Elwan H, et al. Carotid Body Tumors: A Review of 25 Years Experience in Diagnosis and Management of 56 Tumors. Ann Vasc Dis 2014;7: 292–299.

Dorobisz K, Dorobisz T, Temporale H, et al. Diagnostic and therapeutic dif- ficulties in carotid body paragangliomas, based on clinical experience and a review of the literature. Adv Clin Exp Med 2016; 25: 1173–1177.

Pacheco-Ojeda LA. Carotid body tumors: Surgical experience in 215 cases. J Craniomaxillofac Surg 2017; 45: 1472–1477.

Luna-Ortiz K, Rascon-Ortiz M, Villavicencio-Valencia V, et al. Carotid body tumors: review of a 20-year experience. Oral Oncol 2005; 41: 56–61

Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab 200; 86: 5210–5216.

Amato B, Serra R, Fappiano F, et al. Surgical complications of carotid body tumors surgery: a review. Int Angiol 2015; 34(6 Suppl 1):15–22.

Hu K, Persky MS. Treatment of Head and Neck Paragangliomas. Cancer Control 2016; 23: 228–241.

Davila VJ, Chang JM, Stone WM, et al. Current surgical management of carotid body tumors. J Vasc Surg 2016; 64:1703–1710.

Dahia PL. Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity. Nat Rev Cancer 2014;14:108–119.

Williams MD. Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics. Head Neck Pathol 2017;11: 278–287.

Boedeker CC, Neumann HPH, Ridder GJ, et al. Paragangliomas in patients with mutations of the SDHD gene. Otolaryngol Head Neck Surg 2005;132: 467–470.

Shamblin WR, ReMine WH, Sheps SG, et al. Carotid body tumor: Clinicopathologic analysis of ninety cases (chemodectoma). Am J Surg 1971;122:732–739.

Jena A, Reddy GV, Kadiyala V, et al. A case of large carotid body tumor: surgical challenge. Indian J Vasc Endovasc Surg 2016; 3: 96–98.

Biscoe TJ. Carotid body: structure and function. Physiol Rev 1971; 51: 437.

Jaźwiec P, Gać P, Niewinski P. Current opinions on clinical significance of carotid bodies. Przegląd lekarski 2013; 7: 211–214.

Luna-Ortiz K, Rascon-Ortiz M, Villavicencio-Valencia V, et al. Does Shamblin’s classification predict postoperative morbidity in carotid body tumors? A proposal to modify Shamblin’s classification. Eur Arch Otorhinolaryngol 2006; 263:171–175.

Jansen JC, van Berg R, Kuiper A, et al. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 2000; 88: 2811.

Hallett Jr JW, Nora JD, Hollier LH, et al. Trends in neurovascular complications of surgical management for carotid body and cervical paragangliomas: a fifty-year experience with 153 tumors. J Vasc Surg 1988; 7: 284–291.



  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659