Murat Enoz, Department of Otolaryngology, Head&Neck Surgery, Istanbul University, School of Medicine, Turkey.
22 March 2005
Dear Editor
The carotid body is derived from both mesodermal elements of the third branchial arch and neural elements originating from the neural crest ectoderm [1]. It is a small ovoid or irregular mass bilaterally situated on the bifurcation of the common carotid artery, and functions as a chemoreceptor sensitive to changes in arterial pO2, pCO2 and pH, which induces reflex changes in vasomotor activity and respiration [2]. Carotid body tumour (paraganglioma) is both unusual and highly vascular, arising from the paraganglion cells of the carotid body.
The most frequent symptom of this tumor is a palpable neck mass located below the angle of the mandible, which is not mobile vertically but laterally because of its adventitial attachments.
Complete intra-arterial (transfemoral) bilateral cerebral angiography is the mainstay of the diagnosis, with 100% reported accuracy [3]. The presence of a lyre-like image (an enhancing high vascular oval mass widening the angle of the bifurcation with displacement of the internal and external carotid arteries) is essentially pathognomonic of a carotid body tumour . The blood supply is primarily from the bifurcation and external carotid artery, but contribution from the internal carotid artery, vertebral artery, and thyrocervical trunk can occur. Knowledge of the sources of blood supply aids in exposure and hemostasis.
Preoperative investigation for any excess catecholamine production by measuring urinary metanephrines and valimandelic acid is justified in these patients, since this is of paramount importance for the intraoperative anesthetic management [4]. In the differential diagnosis are included lymphomas, cervical cysts (branchial cleft cyst, or thyroid cysts), aberrant thyroid tissue, parotid gland or thyroid tumor, aneurysms, nerve sheath tumors, and swollen lymph nodes (from inflammatory or metastatic process); all have to be excluded.
The first successful dissection of tumor in the English language literature was in 1903 by Scudder [3]. In 1940, Gordon-Taylor [5] demonstrated a safe subadvential plane of tumor dissection. Before the advent of current vascular surgical techniques, large carotid body tumors frequently necessitated ligation of the carotid artery for resection. Mortality approached 50% and those patients who survived had a 15–30% incidence of stroke [6]. In 1953, Linder [7], then professor of surgery at the University of Berlin, classified the tumors of the carotid body, stating the following: type I, Dissection is easily achieved with simple peeling; type II, dissection of the tumor is achieved without resection of the common and internal carotid, sometimes dissecting the adventitia and the external carotid; type III, in which the condition is completely different because, in this case, dissection is achieved by resection of the carotid bifurcation with all the known consequences of a carotid ligation.
About 20 years later (1971), Shamblin [8] repeated the same classification by stating: "26% of the tumors could be easily dissected from the adjacent vessels without significant trauma to the vessel or to the tumor capsule and they were classified as group 1. In a second group the tumor seemed to surround the vessel partially and to be adherent to vessel adventitia dissection of this tumor became much more difficult; 46.5% per cent of the tumor fell into this group 2. Group 3 tumors, however, had such an intimate adherent relationship to the entire circumference of the carotid bifurcation that surgical dissection would be impossible (27.6%).”
The most appropriate treatment for head and neck paraganglioma is surgical removal because malignant change occurs in about 12% of sporadic cases [9]. An arteriogram would be helpful in deciding the surgical approach because it shows the blood supply, feeder and extent of the tumor [10,11]. There are some reports on preoperative embolization for neck paraganglioma. By shutting off the blood flow, it is effective in tumor-bed obliteration and reduces operative bleeding [12,13]. On the other hand, some reports have indicated that this procedure represents the risk of cerebral arterial occlusion [14,10].
Surgical techniques
Surgical excision requires good exposure with identification of the carotid artery both proximal and distal to the tumor. Every effort should be made to identify and preserve the vagus, hypoglossal, and spinal accessory nerves because compromising these cranial nerves contributes significantly to postoperative morbiditiy. Tumor removal requires subadventitial dissection.
The primary step before attempting to excise the tumor is proximal and distal control of the blood flow of the carotid by using slings or tapes, given that the blood supply to the tumor rises from the common and external carotid arteries. The proximal common carotid artery is exposed by dissection of the deep fascia anterior to the the tumor and dissecting superolaterally exposes the tumor body interface and this is subsequently dissected in the periadventitial layer close to the arteries, thus avoiding damage to the nerves. In 30% of our material and in a tumor of >5 cm in diameter, ligation of the external carotid artery had to be performed; this decreases tumor vascularity and local bleeding, thus facilitating complete removal and tumor dissection away from the internal carotid artery. Sufficient superior tumor exposure requires identification of the facial nerve and its marginal mandibular branch, some parotid gland elevation, dissection of digastric and stylohyoid muscles, and occasionally, submandibular gland resection. Mandibular traction is an additional maneuver, which facilitates high carotid exposure.
The preparation of this area is considered the most difficult part of the procedure in cases of large tumors with distal extension. A large complicated tumor is a case for the interdisciplinary team, but in every case a vascular surgeon is invaluable for the reconstitution of the anatomic relationships, while the assistance of an experienced head and neck surgeon occasionally proves to be very significant for the safe and radical excision of large tumors [15]. Owing to the multiple perforating arteries arising from the vasa vasorum, bipolarcauterization, by coagulating these vessels, provides a bloodless dissection along the length of the surface of the tumor, significantly diminishing the danger of damage to the adjacent arteries and nerves [16].
The intraoperative management of the carotid body tumors in patients at high risk for injury to the internal carotid artery should be based on the results of cerebral blood flow studies. The use of a shunt aims at ensuring continued cerebral blood flow during cross-clamping of the carotid artery. It is usually indicated in type III tumors in which the arterial wall involvement or stenosis exists and the common or internal carotid is injured or sacrificed to secure radical excision of the tumor [17,18].
Sincerely
Dr. Murat Enoz
References
1- Pryse-Davies J, Dawson IMP, Westbury G. Some morphologic, histochemical, and chemical observations on chemodectomas and the normal carotid body, including a study of the chromaffin reaction and possible ganglion cell elements. Cancer 1964;17:185–202.
2- Roistacher SL. Carotid body tumor with concurrent masticatory pain dysfunction. Oral Surg Oral Med Oral Pathol 1997;83:10–13.
3- Kaman L, Singh R, Aggarwal R, Kumar R, Behera A, Katariya RN (1999) Diagnostic and therapeutic approaches to carotid body tumours: report of three cases and review of the literature. Aust N Z J Surg 69:852–855
4- Meyer FB, Sundt TM Jr, Pearson BW (1986) Carotid body tumors: a subject review and suggested surgical approach. J Neurosurg Mar 64:377–385
5- Gordon-Taylor G (1940) On carotid tumours. Br J Surg 28: 163–172
6- McIlrath DC, ReMine WH (1963) Carotid-body tumors. Surg Clin North Am 43:1135–1144
7- Byrne JJ (1958) Carotid body and allied tumors. Am J Surg 95:371–384
8- Shamblin WR, ReMine WH, Sheps SG (1971) Carotid body tumor – clinicopathologic analysis of 90 cases. Am J Surg 122: 732–739
9- Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-7.
10- Urquhart AC, Johnson JT, Myers EN, Schechter GL. Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope 1994;104:440-5.
11- Moore G, Yarington CT, Mangham C. Vagal body tumors: diagnosis and treatment. Laryngoscope 1986;96:533-6.
12- Higo R, Asai M, Sugasawa M, Takeuchi N, Nemoto S. Preoperative embolization for paraganglioma. Auris Nasus Larynx 1994;21:122-5.
13- Tikkakoski T, Luotonen J, Leinonen S, Siniluoto T, Heikkila¨ O, Pa¨iva¨nsalo M, Hyrynkangas K. Preoperative embolization in the malignant of neck paragangliomas. Laryngoscope 1997;107:821-6.
14- Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-/7.
15- Robinson JG, Shagets FW, Beckett WC, Spies JB (1989) A multidisciplinary approach to reducing morbidity and operative blood loss during resection of carotid body tumor. Surg Gynecol Obstet 168:166–170.
16- Hallett JW (1994) Carotid body and cervical paragangliomas. In: CW Jamieson, JST Yao (eds) Operative surgery. Chapman and Hall Medical, London, 5th edn, pp 95–104.
17- Dean RH (1997) Carotid body tumors. In: Sabiston textbook of surgery, 15th edn. Saunders, London pp 1661–1662.
18- Sanghvi VD, Chandawarkar RY (1993) Carotid body tumors. J Surg Oncol 54:190–192.
Surgical techniques for carotid body tumors
22 March 2005
Dear Editor
The carotid body is derived from both mesodermal elements of the third branchial arch and neural elements originating from the neural crest ectoderm [1]. It is a small ovoid or irregular mass bilaterally situated on the bifurcation of the common carotid artery, and functions as a chemoreceptor sensitive to changes in arterial pO2, pCO2 and pH, which induces reflex changes in vasomotor activity and respiration [2]. Carotid body tumour (paraganglioma) is both unusual and highly vascular, arising from the paraganglion cells of the carotid body.
The most frequent symptom of this tumor is a palpable neck mass located below the angle of the mandible, which is not mobile vertically but laterally because of its adventitial attachments.
Complete intra-arterial (transfemoral) bilateral cerebral angiography is the mainstay of the diagnosis, with 100% reported accuracy [3]. The presence of a lyre-like image (an enhancing high vascular oval mass widening the angle of the bifurcation with displacement of the internal and external carotid arteries) is essentially pathognomonic of a carotid body tumour . The blood supply is primarily from the bifurcation and external carotid artery, but contribution from the internal carotid artery, vertebral artery, and thyrocervical trunk can occur. Knowledge of the sources of blood supply aids in exposure and hemostasis.
Preoperative investigation for any excess catecholamine production by measuring urinary metanephrines and valimandelic acid is justified in these patients, since this is of paramount importance for the intraoperative anesthetic management [4]. In the differential diagnosis are included lymphomas, cervical cysts (branchial cleft cyst, or thyroid cysts), aberrant thyroid tissue, parotid gland or thyroid tumor, aneurysms, nerve sheath tumors, and swollen lymph nodes (from inflammatory or metastatic process); all have to be excluded.
The first successful dissection of tumor in the English language literature was in 1903 by Scudder [3]. In 1940, Gordon-Taylor [5] demonstrated a safe subadvential plane of tumor dissection. Before the advent of current vascular surgical techniques, large carotid body tumors frequently necessitated ligation of the carotid artery for resection. Mortality approached 50% and those patients who survived had a 15–30% incidence of stroke [6]. In 1953, Linder [7], then professor of surgery at the University of Berlin, classified the tumors of the carotid body, stating the following: type I, Dissection is easily achieved with simple peeling; type II, dissection of the tumor is achieved without resection of the common and internal carotid, sometimes dissecting the adventitia and the external carotid; type III, in which the condition is completely different because, in this case, dissection is achieved by resection of the carotid bifurcation with all the known consequences of a carotid ligation.
About 20 years later (1971), Shamblin [8] repeated the same classification by stating: "26% of the tumors could be easily dissected from the adjacent vessels without significant trauma to the vessel or to the tumor capsule and they were classified as group 1. In a second group the tumor seemed to surround the vessel partially and to be adherent to vessel adventitia dissection of this tumor became much more difficult; 46.5% per cent of the tumor fell into this group 2. Group 3 tumors, however, had such an intimate adherent relationship to the entire circumference of the carotid bifurcation that surgical dissection would be impossible (27.6%).”
The most appropriate treatment for head and neck paraganglioma is surgical removal because malignant change occurs in about 12% of sporadic cases [9]. An arteriogram would be helpful in deciding the surgical approach because it shows the blood supply, feeder and extent of the tumor [10,11]. There are some reports on preoperative embolization for neck paraganglioma. By shutting off the blood flow, it is effective in tumor-bed obliteration and reduces operative bleeding [12,13]. On the other hand, some reports have indicated that this procedure represents the risk of cerebral arterial occlusion [14,10].
Surgical techniques
Surgical excision requires good exposure with identification of the carotid artery both proximal and distal to the tumor. Every effort should be made to identify and preserve the vagus, hypoglossal, and spinal accessory nerves because compromising these cranial nerves contributes significantly to postoperative morbiditiy. Tumor removal requires subadventitial dissection.
The primary step before attempting to excise the tumor is proximal and distal control of the blood flow of the carotid by using slings or tapes, given that the blood supply to the tumor rises from the common and external carotid arteries. The proximal common carotid artery is exposed by dissection of the deep fascia anterior to the the tumor and dissecting superolaterally exposes the tumor body interface and this is subsequently dissected in the periadventitial layer close to the arteries, thus avoiding damage to the nerves. In 30% of our material and in a tumor of >5 cm in diameter, ligation of the external carotid artery had to be performed; this decreases tumor vascularity and local bleeding, thus facilitating complete removal and tumor dissection away from the internal carotid artery. Sufficient superior tumor exposure requires identification of the facial nerve and its marginal mandibular branch, some parotid gland elevation, dissection of digastric and stylohyoid muscles, and occasionally, submandibular gland resection. Mandibular traction is an additional maneuver, which facilitates high carotid exposure.
The preparation of this area is considered the most difficult part of the procedure in cases of large tumors with distal extension. A large complicated tumor is a case for the interdisciplinary team, but in every case a vascular surgeon is invaluable for the reconstitution of the anatomic relationships, while the assistance of an experienced head and neck surgeon occasionally proves to be very significant for the safe and radical excision of large tumors [15]. Owing to the multiple perforating arteries arising from the vasa vasorum, bipolarcauterization, by coagulating these vessels, provides a bloodless dissection along the length of the surface of the tumor, significantly diminishing the danger of damage to the adjacent arteries and nerves [16].
The intraoperative management of the carotid body tumors in patients at high risk for injury to the internal carotid artery should be based on the results of cerebral blood flow studies. The use of a shunt aims at ensuring continued cerebral blood flow during cross-clamping of the carotid artery. It is usually indicated in type III tumors in which the arterial wall involvement or stenosis exists and the common or internal carotid is injured or sacrificed to secure radical excision of the tumor [17,18].
Sincerely
Dr. Murat Enoz
References
1- Pryse-Davies J, Dawson IMP, Westbury G. Some morphologic, histochemical, and chemical observations on chemodectomas and the normal carotid body, including a study of the chromaffin reaction and possible ganglion cell elements. Cancer 1964;17:185–202.
2- Roistacher SL. Carotid body tumor with concurrent masticatory pain dysfunction. Oral Surg Oral Med Oral Pathol 1997;83:10–13.
3- Kaman L, Singh R, Aggarwal R, Kumar R, Behera A, Katariya RN (1999) Diagnostic and therapeutic approaches to carotid body tumours: report of three cases and review of the literature. Aust N Z J Surg 69:852–855
4- Meyer FB, Sundt TM Jr, Pearson BW (1986) Carotid body tumors: a subject review and suggested surgical approach. J Neurosurg Mar 64:377–385
5- Gordon-Taylor G (1940) On carotid tumours. Br J Surg 28: 163–172
6- McIlrath DC, ReMine WH (1963) Carotid-body tumors. Surg Clin North Am 43:1135–1144
7- Byrne JJ (1958) Carotid body and allied tumors. Am J Surg 95:371–384
8- Shamblin WR, ReMine WH, Sheps SG (1971) Carotid body tumor – clinicopathologic analysis of 90 cases. Am J Surg 122: 732–739
9- Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-7.
10- Urquhart AC, Johnson JT, Myers EN, Schechter GL. Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope 1994;104:440-5.
11- Moore G, Yarington CT, Mangham C. Vagal body tumors: diagnosis and treatment. Laryngoscope 1986;96:533-6.
12- Higo R, Asai M, Sugasawa M, Takeuchi N, Nemoto S. Preoperative embolization for paraganglioma. Auris Nasus Larynx 1994;21:122-5.
13- Tikkakoski T, Luotonen J, Leinonen S, Siniluoto T, Heikkila¨ O, Pa¨iva¨nsalo M, Hyrynkangas K. Preoperative embolization in the malignant of neck paragangliomas. Laryngoscope 1997;107:821-6.
14- Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-/7.
15- Robinson JG, Shagets FW, Beckett WC, Spies JB (1989) A multidisciplinary approach to reducing morbidity and operative blood loss during resection of carotid body tumor. Surg Gynecol Obstet 168:166–170.
16- Hallett JW (1994) Carotid body and cervical paragangliomas. In: CW Jamieson, JST Yao (eds) Operative surgery. Chapman and Hall Medical, London, 5th edn, pp 95–104.
17- Dean RH (1997) Carotid body tumors. In: Sabiston textbook of surgery, 15th edn. Saunders, London pp 1661–1662.
18- Sanghvi VD, Chandawarkar RY (1993) Carotid body tumors. J Surg Oncol 54:190–192.
Competing interests
None declared