Mark-Paul F.M. Vrancken Peeters, Johanna M. Hendriks, Ellen V. Rouwet,

Marc R.H.M.van Sambeek, Hero van Urk, and Hence J.M. Verhagen

A 63-year old female was referred to our hospital because she had a mass on the right side of the neck. The swelling had slowly progressed in a couple of months. Besides problems with swallowing there were no other complaints. Her previous medical history was unremarkable and she could not remember any family members with similar lesions. Physical examination showed a non-tender mass with a diameter of around 6 cm located just anterior of the sternocleidomastoid muscle in the anterior triangle of the neck. The mass was mobile in a back-forward direction but could not be moved in a cranial-caudal direction. No signs of cranial nerve deficits were detected. An ultrasound examination showed a highly vascularized structure in the bifurcation between the internal and external carotid artery (Fig. 33.1).

Question 1

What is the most likely diagnosis that caused the swelling in the neck?

A.  Enlarged lymph mode

B.  Paraganglioma

C.  Aneurysm of the carotid artery

D.  Goitre of the right thyroid lobe

E.  Cystic neck lesion

H.J.M. Verhagen ( )

Department of Vascular Surgery, H-810, Erasmus University Medical Center, Rotterdam,

The Netherlands

Fig. 33.1 An ultrasound of a carotid body tumor

Question 2

Which of the following examinations is preferable to confirm the diagnosis? Rank in order of which is the best method.

A.Needle biopsy

B.Magnetic Resonance Imaging (MRI)

C.Contrast-enhanced angiography

D.Somatostatin receptor scintigraphy (SMS-scan)

E.Angiography

The diagnosis of carotid body tumor was confirmed by MRI (Fig. 33.2) and SMS-scan (Fig. 33.3). The lesion in the neck was measured 5.3 by 4.4 by 4.1 cm. The scans also revealed a similar vascularized mass near the aortic arch and one in the tympanic space of the middle ear. Because of the size of the tumor and the difficulty with swallowing, we decided to treat the patient.

Question 3

What are the possible complications due to the surgical excision of such a large mass in this area?

A.Horner’s syndrome

B.Vocal cord paralysis

C.Paresis of the mandibular branch of the trigeminal nerve

D.Ipsilateral tongue paresis

E.All of the above

Question 4

Which of the following statements is correct?

A.A Shamblin I tumor can always be removed without cranial nerve damage

B.A Shamblin III tumor can never be removed without cranial nerve damage

C.When the carotid body tumor is growing, encasement of the carotid arteries takes place

D.When a Shamblin III tumor is removed, the carotid bifurcation needs to be replaced

E.The chancesof cranial nerve damageis not dependent of the size of thecarotid body tumor

Question 5

What could be the best treatment option in this particular case?

A.Surgical excision

B.Selective embolization

C.Radiation therapy

D.Chemotherapy

E.Combination of these treatment modalities

The risk of complications with the surgical excision of such a large carotid body tumor is relatively high. Therefore, we first embolized the side branches of the external carotid artery and the thyrocervical trunk that feed the carotid body tumor, to let the tumor shrink in size (Fig. 33.4). After 1 year, the carotid body tumor had decreased in size to 4 by 3.5 by 3 cm. The smaller the size of the tumor, the more likely that resection of the tumor can be performed with minimal morbidity.

Fig. 33.4 Contrast angiography of the procedure, in which the feeding vessels to the carotid body tumor were coiled and a covered stent was placed in the common and internal carotid artery. In this way, the tumor was abolished from having access to the blood supply