Abstract
Background Carotid body paragangliomas are rare neoplasms usually benign, however sometimes presenting as highly aggressive tumors. Surgery is the main line of treatment. Purpose To study and describe clinical presentations, surgical approaches, postoperative complications, and treatment outcomes. Materials and Methods A single-institution retrospective analysis of 19 cases with carotid body paragangliomas who were candidates for surgery from January 2009 through January 2019 with a mean follow-up period of 58.8 months. Results The mean age was 46 years with the female predominance of 63%. The mean size of the tumor was 4.3 cm. All cases were presented with a painless pulsating neck lump located anteriorly at the level of the hyoid bone. Neck US was done in all cases as a primary screening investigation. CT scanning was the second main investigation performed in 17 cases (89.5%) revealing tumors attached to the carotid artery at its bifurcation. Urinary catecholamine metabolites were measured in all cases to rule out familial functioning types. 5 cases (26.3%) were malignant. All cases were surgically approached through transcervical transverse incision. 11, 5, and 3 cases were classified as Shamblin's type II, III, and I, respectively. All tumors were R0 resected with nodal neck dissection conducted in the malignant group. Major complications occurred in 4 cases (21%) during tumor dissection from the adventitia of carotid bifurcation. ECA ligation was performed in one case (5.3%). 2 patients (10.5%) suffered XII nerve paralysis. Carotid artery blowout occurred in one patient (5.3%) and was immediately controlled. No operative mortality occurred. All patients were free of disease during the follow-up period. 4 malignant cases (21%) suffered a systemic relapse to bone and lung metastasis justifying adjuvant chemotherapy, radiotherapy, or both. Conclusions Surgery is the treatment of choice for carotid body paragangliomas. Complete R0 resection should be justified especially in case of malignancy. Adjuvant chemotherapy or radiotherapy is an option for patients with primary malignancy or relapse.
Highlights
Paragangliomas (PGs) are paraganglionic tumors of chemoreceptor cell origin. is term “paraganglia” was first described by Kohn in the early 20th century and is the most appropriate nomenclature from the embryologic aspect [1].ey are distributed paraxially in the trunk mainly related to major arteries and cranial nerves of the pharyngeal arches origin
Carotid body paragangliomas (CBPs) are more prevalent in middle-aged females and typically present as slowly growing mass. ey can remain asymptomatic for years
Fine-needle aspiration (FNA) of CBPs is generally not recommended as it may result in significant hemorrhage; aspirations in unsuspected cases may show moderate cellularity of small groups arranged in a “pseudorosette” pattern [9]
Summary
Paragangliomas (PGs) are paraganglionic tumors of chemoreceptor cell origin. is term “paraganglia” was first described by Kohn in the early 20th century and is the most appropriate nomenclature from the embryologic aspect [1].ey are distributed paraxially in the trunk mainly related to major arteries and cranial nerves of the pharyngeal arches origin. CBPs are rare highly vascular tumors with an incidence of only 0.3% of all paragangliomas and 60% of head and neck paragangliomas, followed by jugulotympanic and vagal PGs [4]. Carotid body paragangliomas are rare neoplasms usually benign, sometimes presenting as highly aggressive tumors. A single-institution retrospective analysis of 19 cases with carotid body paragangliomas who were candidates for surgery from January 2009 through January 2019 with a mean follow-up period of 58.8 months. CT scanning was the second main investigation performed in 17 cases (89.5%) revealing tumors attached to the carotid artery at its bifurcation. Major complications occurred in 4 cases (21%) during tumor dissection from the adventitia of carotid bifurcation. 4 malignant cases (21%) suffered a systemic relapse to bone and lung metastasis justifying adjuvant chemotherapy, radiotherapy, or both. Adjuvant chemotherapy or radiotherapy is an option for patients with primary malignancy or relapse
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