Introduction

The multiple paragangliomas are rare tumours, with slow evolution, posing diagnostic and therapeutic problems. The carotid and jugulo-tympanic localization are the most frequent(1). The diagnosis of CBT is typically made with radiological studies(2). The literature is reviewed and the details of the history, physical findings, results of laboratory tests, and the surgical management of the patient are presented.

Case Report

A 72-year-old woman was admitted because of 4 months history of tenderness and palpable left neck masse. Otolaryngologic examination was negative except 4 cm × 4 cm left neck masse. These masse was pulsating with limited vertical mobility. Cranial nerve examinations were intact. Past medical history and family history were unremarkable. The color Doppler ultrasonography (USG) revealed the hypointense, homogenously opaque, vascularized 2 cm × 2 cm × 1.5cm mass on the right carotid bifurcation and a 4cm × 4 cm × 4.5cm mass on the left carotid bifurcation. She was in sinus rthym with a blood pressure of 130/85 mm Hg. Repeatedly measured plasma and urinary catecholamine concentration was normal. Angiography showed vascularized bilateral carotid body masses with characteristic tumor blush and broadening of the carotid bifurcation(Figures 1,2 and 3).

Figure 1

Figure 1.View of bilateral carotid body masses in DSA.

Figure 2

Figure 2. View of right carotid mass in DSA.

Figure 3

Figure 3. View of left carotid mass in DSA.

Excision of the enlarged mass was planned regarding the patient’s age as 72 years. Under endotracheal general anesthesia, classical incision medial to sternocleidomastoid muscle to explore carotid artery was made. Common, internal and external carotid arteries were suspended respectively. Tumoral mass was observed to be quite large (Figure 4).

Figure 4

Figure4.

Although there were minimal hemorrhagic foci, it was observed that no adventitial invasion by the tumaoral mass was evident. In the operation 4 cm × 4 cm, 4 cm × 3 cm, encapsulated mass which was thought to be a carotid body tumor was dissected from carotid bifurcation(Figure 5).

Figure 5

Figure5

It was shaven appropriately and extirpated (Figure 6).

Figure 6

Figure6

After extirpation, it was revealed that internal carotid artery was kinking due to removal of the mass (Figure 7).

Figure 7

Figure7

A segment of about 4 centimeters in length was removed and end-to-end anastomosis of the remaining internal carotid artery segments was carried out. The anatomical layers were closed properly (Figure 8).

Figure 8

Figure8.

We planned six months after this first operation, the carotid body tumor on the right side will be totally excised. During perioperative and early postoperative period no mortality did occur. There was no need for perioperative blood transfusion since no massive hemorrhage was seen. There weren’t any injuries to the neighboring vascular and cranial nerve structures experienced. Histopathological evaluation concluded that this mass was a paraganglioma (carotid body tumor). Her hospital stay took 5 days long. Her late postoperative follow-up is still free of symptoms.

Discussion

Multiple paragangliomas of the head and neck are rare conditions. The incidence of multiple paragangliomas is reported to the approximately 10% of the total patients, but in familial cases it increases up to 35-50%. In the head and neck region, the most common association is represented by bilateral carotid body tumors or by carotid body tumor associated with tympanic-jugular glomus(3).

Carotid body tumors can result in compression in carotid arteries, nerves like vagus, hypoglossus, glossopharyngeus, sympathetic chain and other neighboring structures. Vascular and neural damages during operative excision are more likely, especially if there is massive and/or bilateral tumor(4).

The need for an exhaustive preoperative evaluation is stressed so that the proper surgery can be planned(5). Doppler ultrasound is proved to be an exclusive method in demonstrating the localization of tumor and defining its vascular origin (6). CT can reveal the skull base invasion and intracranial entension(4,7). Digital substraction angiography is the best imaging technique before carotid body tumor operations(4,7). Multiple tumors can be detected by angiography.

Carotid body (CB) is a round to ovoid or flattened structure situated within the adventitia of the common carotid artery bifurcation on both sides of the neck(2). CB contains two basic types of cells: chief cells (or glomus type 1) and sustentacular cells (glomus type 2). Carotid body tumor (CBT) or paraganglioma arises from the chief cells of the carotid body(2).

Carotid body tumors are classified into three groups according to operative difficulties by Shamblin et al. (4,8). Type I tumors are localized and easily resected. Type II tumors are partially adherent to surrounding vessels, and Type III tumors are paragangliomas intimately surround or encase the vessels. For our case, tumor was Shamblin Type II.

Currently, three treatment options are recognized: surgical resection, radiation therapy, and a wait-and-scan policy(9). Treatment must be individualized, taking into account the patient's age, tumor site and size, multicentricity, and pre-existing cranial nerve deficits. Surgical removal is the first intention treatment of these tumours. In the multiple forms, the therapeutic strategy must be adapted to each case: usually, the intervention begins by the carotid localization(1). The risk of sequential bilateral surgery and necessity of post-operative follow up are important for the patients(4).Cure rates of 89 to 100% have been reported(9,10). Advances in preoperative and operative techniques together with better postoperative care have reduced complication rates.