Background: Carotid body tumour (CBT) is a rare neoplasm, yet it is the commonest head and neck paraganglioma. In Iraq, relevant literature is sparse. Herein, we present our second case series. Methodology: Patients with CBTs that were operated upon in the Department of Thoracic and Vascular Surgery, Baghdad Medical City from 2010 to 2014 were enrolled. History and examination were followed by a workup of duplex ultrasonography, CT scan, magnetic resonance imaging, CT or conventional carotid angiography. Surgical exploration via a standard anterolateral cervical incision and subadventitial dissection was used to resect the tumours with preservation of carotid arteries. Intra-luminal carotid shunts and vein grafts were prepared to be used if necessary. Results: There were 5 males and 2 females aging 17 - 46 with a mean of 32.9 ± 9.8 year. All patients had slowly growing painless pulsatile swelling below mandiblular angle for long durations (1 - 25 years) and a positive Fontaine’s sign. All tumours were benign, unilateral (right n = 4, left n = 3) and ranging in size from 3 × 3 cm to 6.4 × 3.2 cm. Beside US neck exam, carotid angiography was done in 5 patients. According to Shamblin classification, 4 were class II, 2 class I and 1 class III. All tumours were successfully resected with preservation of ICA. However, the ECA was safely ligated twice due to severe involvement. Tongue deviation occurred once (14.3%) but no patient died and none had stroke or recurrence. Conclusions: Our results of surgery for CBT compare very well with the international standards.
The carotid body (CB), described for the first time by Albrecht Von Haller in 1743 [
In Iraq, little has been published about this uncommon disease; one paper [
Patients with CBTs that were operated upon in the Department of Thoracic and Vascular Surgery, Baghdad Medical City Teaching Hospital over a 4-year period (2010-2014) were enrolled in this prospective study. Detailed history taking and thorough physical examination particularly of the region of the neck were performed. Any cranial nerve involvement was recorded when present. Relevant investigations were ordered such as Duplex US scanning, MRI, MRA, and carotid arteriography (conventional or CT angiography). Preoperative preparation included a thorough medical checkup, fitness for general anaesthesia, blood preparation and written high risk consent was signed by each patient after explaining the possible morbidity and mortality. Due to the non- availability of indwelling Javid carotid shunt, an alternative shunt (Scribner’s shunt of hemodialysis) was pre- pared.
Surgical Technique“The patient is positioned supine with the neck rotated to the opposite side. Since the need for carotid resection cannot always be predicted, one leg is prepared in case the saphenous vein should be required. The carotid artery is exposed through a standard anterolateral cervical incision along the anterior border of the sternocleidomastoid muscle. Control of the common, internal and external carotid arteries is obtained and hypoglossal and vagus nerves are identified. Using the bipolar diathermy, a capsular-adventitial or sub-adventitial (white line) dissection plane is established at the inferior margin of the tumour at bifurcation and extended cephalad onto the internal and external carotid arteries. Branches of external carotid artery may require division to facilitate the dissection (
artery and replacement with a saphenous interposition vein graft” [
Seven patients (5 males and 2 females). The male to female ratio was 2.5:1. The youngest was a girl of 17 and the oldest was a man of 46. The mean age was 32.9 ± 9.8 year.
The patients lived in the middle and south of Iraq except one patient from Kirkuk in the north. The 2 ladies were a housewife and a student whereas men were (workers n = 3, soldier n = 1 and a civil servant n = 1). The demographic and clinical features are shown in
There were 5 males and 2 females. All patients had slowly growing painless pulsatile swelling below the angle of the mandible present for long durations (1 to 25 years) and a positive Fontaine̓ s sign. All tumours were unilateral (4 on the right side and 3 on the left side). There was one patient with hypertension, one with palpable cervical lymph nodes and one with prior history of incisional biopsy (
Age (year) | Male, n (%) | Female, n (%) | Total, n (%) |
---|---|---|---|
17 - 20 | 0 | 1 | 1 |
21 - 30 | 1 | 0 | 1 |
31 - 40 | 3 | 1 | 4 (57.1) |
41 - 50 | 1 | 0 | 1 |
Total | 5 (71.4) | 2 (28.6) | 7 (100) |
ID | Gender, age | Swelling* | Fountain Sign# | Side | Past history of incisional biopsy | Cx LN@ | HT! | Workup | Size of CBT | Shamblin Class | Resection with preserved ICA | ECA ligation | Postop. ND^ |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | M, 24 | +, 4 yr | + | R | - | + | - | US, CT, conventional arteriography | 5 × 3 cm | I | + | - | Nil |
2 | M, 46 | +, 25 yr | + | R | - | - | - | US, CT angiography | 3 × 3 cm | I | + | - | Nil |
3 | F, 17 | +, 3 yr | + | L | - | - | - | US, CT angiography | 3 × 3 cm | II | + | - | Nil |
4 | F, 35 | +, 1 yr | + | R | - | - | - | US, CT | ? | II | + | - | XII n$ |
5 | M, 37 | +, 2 yr | + | L | - | - | - | US, CT angiography | 5 × 3 cm | II | + | - | Nil |
6 | M, 31 | +, 10 yr | + | L | + | - | - | US, CT, Conventional angiography | 6.2 × 5.3 cm | III | + | + | Nil |
7 | M, 40 | +, ? | + | R | - | - | + | US, CT | 6.4 × 3.2 cm | II | + | + | Nil |
*Slowly growing painless pulsatile swelling below the angle of the mandible for some duration; #A swelling moveable horizontally but not vertically; @Cervical lymphadenopathy; !Hypertension; ^Postoperative neurological deficits; $Ipsilateral tongue deviation.
Shamblin class | Patients n | ENDs n | P value |
---|---|---|---|
I and II | 6 | 0 | The result is significant at p < 0.05 |
III | 1 | 1 | |
Total | 7 | 1 |
Details of workup are shown in
Head and neck paragangliomas are rare neoplasms comprising about 0.03% of all human tumors. The annual incidence is around 0.001% [
The peak age of the patients was in the 4th decade. This finding is similar to other reports “Most patients become symptomatic between their 30th and 60th birthday” [
In this study, male to female ratio was 2.5:1. However, other studies state that females are more frequently involved [
The results of this study coincide with the published literature about the most frequent symptom of CBT that is a slowly growing painless mass below the angle of the mandible discovered by the patient or an examiner incidentally [
Technique | Patients, n (%) | Comments |
---|---|---|
US and Doppler neck exam | 7 (100) | It was accurate in (n = 6, 85.7%) of cases. One case of CBT was falsely diagnosed as an enlarged lymph node. |
CT scan of the neck with iv contrast ( | 2 (28.6) | It provided more detailed description of the tumour extent, encasement of carotid arteries and local invasion. |
MRI of the neck | 0 (0) | It was the least used imaging method despite its well-known advantages. |
Carotid arteriography | 5 (71.4) | It used to be the gold standard test for CBT. It has merits and demerits. |
Conventional angiography | 3 (42.6) | |
CT angiography ( | 2 (28.6) | |
FNAC | 0 (0) | |
Incisional biopsy | 1 (14.3) | It was risky. |
Serum or urinary catecholamine assay | 0 (0) | It was not done, although it was indicated particularly in the hypertensive patient. |
Routine blood tests | 7 (100) | CBC, FBS, BU, S. Creatinine, bleeding and clotting times, and virology studies. |
Mayo Clinic, USA [ | 153 cases/50 yr: 3.06 |
---|---|
Hussen W. M., Iraq [ | 8 cases/12 yr: 0.66 |
Salehian et al., Iran [ | 97 cases/16 yr: 6.06 |
O’Neill et al., Ireland [ | 29 cases/22 yr: 1.3 |
Wang et al., USA [ | 29 cases/25 yr: 1.2 |
Tayyab et al., Pakistan [ | 8 cases/11 yr: 0.7 |
Patetsios et al., USA [ | 29 cases/30 yr: 0.97 |
The present study, Iraq | 7 cases/4 yr: 1.75 |
The swelling may be very small producing just neck asymmetry or very huge. The presence of a bruit over the mass is uncommonly noted but when present suggests significant compression of the artery [
All patients in this series exhibited a unilateral neck swelling of variable sizes with positive Fontaine sign but none had a neurological deficit preoperatively. Most of the sporadic CBTs (95%) are unilateral [
One patient was hypertensive complaining of headache (14.3%). Unfortunately, he was not investigated by serum or urinary catecholamine measurement to exclude a functioning CBT. Well, studies mention that functional CBTs can cause paroxysmal hypertension which should disappear after surgical resection of the tumour; otherwise, if hypertension persists following surgery, then it might be due to other causes [
When a carotid body tumor is suspected, an imaging study is the next diagnostic step. Bilateral carotid angiography is the most sensitive imaging technique [
“Vascular mass displacing the internal and external carotid arteries at the bifurcation is essentially diagnostic of a carotid paraganglioma [
In the current case series, all patients had US examination of the neck together with colour Doppler imaging which was very useful. “On sonography, paraganglioma present as a well-defined, solid hypoechoic mass and on color Doppler imaging, hypervascularity with a low-resistance flow pattern is seen” [
Noninvasive imaging studies such as CT, MRI, and magnetic resonance angiography are also excellent methods for evaluation [
An open biopsy should not be attempted when a carotid body tumor is suspected because of the highly vascular nature of these tumors, and it is generally not necessary for diagnosis [
The head and neck surgeon should have a high index of suspicion when facing a unilateral neck swelling in the region of carotid bifurcation. Carotid paraganglioma should be the top differential diagnosis in such a situation [
Controversy is faced everywhere in the literature related to CBT. Treatment options are just an example of this varied opinion. On one hand, surgery is considered by many authors as the standard therapy as it provides an immediate and complete removal of the tumour [
In our series, all patients were managed surgically because all were symptomatic and have accepted the explained risks of surgery.
The routine use of preoperative embolization is controversial because of the potential neurologic complication associated with the accidental reflux of particulate matter into the ophthalmic or cerebral circulation. Some authors advocate its use before the resection of large tumours because it may decrease the vascularity of the tumour, reducing intraoperative blood loss and transfusion requirements. The apparent benefit of embolization should be weighed against the risk of stroke [
In our study, this technique hasn’t been used simply because we do not have the necessary expertise in this technique. Kafie et al. (2001) from USA published a nice report of 2 patients with CBTs greater than 4 cm successfully embolized with gel the day before surgery; an intervention that made resection smoother, safer and with minimum blood loss [
We utilized the technique of sub-adventitial dissection for tumour excision in our cases that is adapted by many authors [
The ECA was ligated in 2 of our cases as it was densely involved by the tumour. This is considered by many authors an important maneuver to minimize the blood loss during resection of CBTS which receive their blood supply from this artery [
Ferreira et al. (2013) from Portugal reported 4 cases of CBTs resected using an ultrasound dissector [
Carotid BTs grow in a longitudinal direction and hence tend to involve the cranial nerves in proximity to them [
Modified intra-luminal carotid shunts were prepared in some of our cases but were not used. The use of such shunts during resection of CBTs is controversial. Patetsios et al. believe that familiarity with the use of intra- luminal vascular shunts is critical since the vessel wall is easily damaged during sub-adventitial dissection [
In our series, the tumours were classified according to Shamblin and the intra-operative findings into 3 groups. Suarez et al. states that Shamblin classification is the most widely used staging system for CBTs [
For the sake of better understanding the outcome of surgery we divided our patients into 2 subgroups:
1) Resection of CBT with preservation of carotid arteries (n = 5).
Suarez et al. in a retrospective review of 2175 CBT resections reported 483 (22%) cases of new cranial nerve permanent deficits [
2) Resection of CBT with ligation of ECA only (n = 2). In both cases, the tumours were benign class II and III with encasement of carotid bifurcation. The ECA was ligated as it was heavily involved by the tumours. The outcome was uneventful. Safety of ligation of ECA is widely addressed in the literature [
This study clearly shows that Shamblin class III significantly influenced the occurrence of early neurological deficits (p ˂ 0.05). According to Makeieff et al., the rate of serious complications, i.e., permanent nerve palsy, and vascular complications was 2.3% for Shamblin Class I/II tumors and 35.7% for Shamblin Class III tumors (p ˂ 0.001) [
Our study revealed a noticeable increase in the hospital-based incidence of CBT in Iraq since 2008. The diagnosis of CBT can be made easily if a high index of suspicion is coupled with the necessary investigations. Our results of surgery for CBT compare very well with the international standards.
We would like to thank Dr. Sabah N Jaber FIBMS (CTVS) for his permission to report his patient (the 4th in this series).
None is declared.
A: Study design, B: Data collection, C: Statistical analysis, D: Data interpretation, E: Manuscript preparation, F: Literature search. Waleed M. Hussen: A, B/Abdulsalam Y. Taha: A, B, C, D, E, F/Diar S. Hama-Kareem: A, B.