[Dignity of carotid body tumors. Review of the literature and clinical experiences]

Abstract

Introduction: Tumors of the carotid body are rare paragangliomas (incidence 0.012%) originating from sympathetic fibres of the carotid bifurcation. Growth is slow and they frequently become symptomatic through local mechanical compression of neighboring vascular and neural structures. The aim of this study is to present the diagnosis, therapy and course in patients with a carotid body tumor treated at our department of the Düsseldorf University Hospital and to discuss rates of recurrence and also dignity during the long-term follow-up.

Patients and methods: Included in this retrospective study were all patients treated for a carotid body tumor between January 1988 and June 2008. At follow-up examination the current history was recorded and a physical examination, sonography and duplex sonography were carried out. Furthermore each patient completed the questionnaires QLQ-C30 of the European Organisation for Research and Treatment of Cancer (EORTC) and the module for head and neck QLQ-H&N35 to assess quality of life.

Results: In our collective of 36 patients consisting of 13 men (36%) and 23 women (64%) with an average age of 48.33 years (range 17-78 years), 16 patients presented with a local neck swelling and 5 patients each had difficulties swallowing or hoarseness, respectively. Preoperatively Horner's syndrome was found in one patient. A total of 22 tumors were found on the right side of the neck (52.38%), 20 were found on the left side (47.62%) and 6 patients showed a bilateral carotid body tumor (16.67%), 3 of which were bilaterally excised. The other 3 patients are still under surveillance without surgery. Altogether surgery of 39 carotid body tumors was performed in 36 patients. In all 39 cases (primary surgery n=34, recurrence surgery n=5) the tumors were macroscopically excised in toto. Parts of the vagus nerve had to be resected in 3 patients (7.69% Shamblin type II n=1, Shamblin type III n=1) and resection of blood vessels was necessary during 10 operations. The survival rate after 1 year was 100%, after 2 years 96.3% and after 5 years 92.6%. A local recurrence was diagnosed in 2 patients (5.13%). In one patient a second operation was necessary and in the other patient there was a non-progressive swelling in the carotid bifurcation which had existed for 14 years and which was conservatively left untreated. Peripheral neural lesions could be found in 12% (3/25) at long-term follow-up. None of the patients showed evidence of local or remote metastasization of a carotid body tumor.

Conclusions: Surgical extirpation of carotid body tumors can be regarded as the only curative option with an overall mortality of 0%. Morbidity is low when applying vascular surgical techniques (2.56% for central lesions). The incidence of peripheral nervous lesions is high reflecting the radicality of the resection (64.10%) but is outweighed by the benefits. In the long-term follow-up the rate of permanent peripheral neural lesions decreased to 12%. Due to a potentially infiltrating and disseminating growth, carotid body tumors should be regarded as semi-malignant and should therefore be indicated for surgery at the time of diagnosis. Whether the incidence of carotid body tumors will rise due to increased routine diagnostic examination of the head and neck region using sonography and tomography remains to be seen.