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This is a rare combined presentation of Tetralogy of Fallot and carotid body tumor (CBT). Hypotheses and further discussion provides data for the development of CBT as a response to chronic hypoxemia. This present study demonstrates and discusses such an occurrence.
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... Though CBT is a rare tumor but it constitutes about 65% of the head and neck paragangliomas [3]. It occurs in middle aged people with higher predisposition in females [4] and those living at high altitudes or with diseases causing chronic hypoxemia like Fallot's tetralogy (TOF) or Chronic Obstructed Pulmonary Disease (COPD) [5]. ...
Objectives:
The reported risk of a cranial nerve (CN) injury is up to one in four patients in large registries of carotid body tumor (CBT) resection. Functional outcome for this population is unknown.
Methods:
We evaluated consecutive patients who underwent CBT resection from November 2013 through October 2020. Demographics, intraoperative details, complications, and out- comes were recorded from the medical record. Permanent CN nerve injury was defined as deficits lasting >6 months. Frequency statistics, averages, chi-square test, and multiple logistic regression were completed for primary end points of complications and disease-free survival. Patient reported outcomes were gathered via telephone survey of patients conducted in September 2021.
Results:
Fifty-one patients presented with CBTs and the following Shamblin classes: I (n=7; 14%), II (n = 36; 69%), and III (n = 9; 17%). Head and neck oncology and vascular surgery jointly did 52% of CBT resections, including six of nine Shamblin III cases. Eight patients (15.3%, all Shamblin II or III) suffered a total of 12 CN injuries - eight CN XII (five temporary and three permanent), three CN X (all permanent), and one CN XI (permanent). Seven of the CN injury subgroup had preoperative embolization and five were joint oncology/vascular cases. Additionally, four separate carotid injuries required repair. Notably, all patients had disease-free survival postoperatively at a mean follow-up of 6 months. Patient reported outcomes (PRO) obtained in 70.6% of patients one year or more from index operation demonstrated that two-thirds of patients live without any permanent functional deficits, and the majority of those with continued deficits rate the symptoms as daily but mild in severity.
Conclusions:
In a series of complex CBT patients treated with preoperative embolization capabilities and multidisciplinary surgical approach, disease free survival was achieved in all patients despite a high rate of iatrogenic CN injuries, most commonly CN XII. PRO survey results indicate that injuries identified on clinical exam underreport patients' true post-operative CN deficits - especially branches of CN X. This data supports the practice of aggressive primary resection of CBTs while providing guidance for expected functional outcomes due to CN injury risk.
Carotid body tumors (CBTs) are rare paragangliomas, comprising 0.5% of all head and neck tumors, and 65% of head and neck paragangliomas. A majority of CBTs occur sporadically, while 15% are familial or hyperplastic in the setting of chronic hypoxia. They usually present as unilateral, well-circumscribed rubbery masses, arising at the level of the carotid bifurcation. A majority of CBTs are painless and therefore may evade diagnosis for months to years. Symptomatic lesions occur due to progressive cranial nerve IX, X, or XII dysfunction, manifesting as hoarseness, dysphagia, vertigo, coughing, or odynophagia. Other local symptoms include neck discomfort, pulsatile tinnitus, hearing loss, or carotid sinus syndrome. Appropriate workup includes a thorough physical exam followed by radiographic imaging, vascular studies, and biochemical workup with 24 h urinary catecholamine or metanephrine analysis. The management of these tumors, which involves standalone surgical resection or following embolization for larger and more vascular tumors, will also be discussed.
Herein we report the management of a giant, high-grade and vascular carotid body tumor in a young woman. She presented with slowly progressive neck swelling. Vascular imaging revealed a left-sided, high-grade giant carotid body tumor (> 8cm). The tumor was completely excised by caudocranial subadventitial dissection. Histology of the tumor revealed a characteristic Zellballen pattern of the lesion, suggestive of a paraganglioma. The patient made an uneventful recovery. We also discuss newer insights regarding the management of such highly vascular lesions.
