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Synchronous bilateral carotid body tumor and vagal paraganglioma: A case report and review of literature
Auris Nasus Larynx 35 (2008) 171–175 www.elsevier.com/locate/anl
Synchronous bilateral carotid body tumor and vagal paraganglioma: A case report and review of literature Erkan Karatas a,*, Akif Sirikci b, Tekin Baglam a, Semih Mumbuc a, Cengiz Durucu a, Ediz Tutar c, Muzaffer Kanlikama a a
Gaziantep University, School of Medicine, Department of Otorhinolaryngology, Head and Neck Surgery, Gaziantep, Turkey b Gaziantep University, School of Medicine, Department of Radiology, Gaziantep, Turkey c Gaziantep University, School of Medicine, Department of Pathology, Gaziantep, Turkey Received 28 October 2006; accepted 22 May 2007 Available online 11 September 2007
Abstract We present a case of synchronous bilateral carotid body tumor and vagal paraganglioma in a 49-year-old man who presented with 3 months history of tenderness and palpable neck masses bilaterally. An encapsulated mass which was thought to be a carotid body tumor and an incidental 3 cm 2 cm, 5 cm 1 cm, 5 cm mass which seemed to be originated from vagus nerve were dissected from the left side. Pathology revealed carotid body tumor and vagal paraganglioma. Six months after the first operation, the carotid body tumor on the right side was totally excised. A discussion of this case is followed by a review of the literature surrounding this rare clinic and pathological entity. # 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Neck; Synchronous; Carotid body tumor; Vagal paraganglioma; Surgery
1. Introduction Paragangliomas of the head and neck are distributed widely around vascular structures, along the autonomic nervous system as jugulotympanic, vagal and carotid bodies. Paragangliomas, previously named as chemodectomas, are highly vascularized tumors localized in blood vessel walls and along specific neurons [1]. In the head and neck, these lesions are most commonly located in four primary sites, the carotid bifurcation, jugular foramen, middle ear cavity, and along the course of the vagus nerve. Besides these sites orbit, larynx, and nasopharynx are the other locations. These tumors arise from extra adrenal paragangliar chief cells. Paragangliomas are encapsulated, brown and hard tumors which are formed by epithelial cell islands separated by a vascular fibrous stoma. Paragangliomas which do not show
catecholamine by chromaffin stainings are named as nonchromaffin paragangliomas [2,3]. Carotid body tumors usually present as slowly growing, painless neck masses. They are most commonly encountered in the fourth or fifth decades of life equally in both sexes [2–4]. 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. Bilateral paragangliomas are rarely encountered in nonfamilial cases. We have described non-familial bilateral carotid body tumors together with a left sided vagal paraganglioma in this study.
2. Case report * Corresponding author at: Gaziantep University, Medical Faculty, Department of Otorhinolaryngology, Head and Neck Surgery (KBB), Kilis ¨ zeri, 27310 Gaziantep, Turkey. Tel.: +90 342 3606060; Yolu U fax: +90 342 3603928. E-mail address: [email protected] (E. Karatas).
A 49-year-old male patient was presented with bilateral neck masses for the last 3 years and a recent tenderness around the masses. Otolaryngologic examination was negative except 5 cm 4 cm left and 4 cm 4 cm right
0385-8146/$ – see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2007.05.007
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E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
Fig. 1. A hypointense, homogenously opaque, highly vascularized mass (m) on the right carotid bifurcation and a mass (m) on the left carotid bifurcation were seen in the post contrast T1 weighted MRI of patient.
Fig. 2. T2 weighted images showed bilateral enhancing neck masses (m) extending from the carotid artery bifurcation to the skull base with mass effect on the hypopharynx.
neck masses. These masses were pulsating with limited vertical mobility. Cranial nerve examinations were intact. Past medical history and family history were unremarkable. The magnetic resonance imaging (MRI) studies with contrast and the color Doppler ultrasonography (USG) revealed the hypointense, homogenously opaque, highly vascularized 3 cm 3 cm 2 cm mass on the right carotid bifurcation and a 4.5 cm 3 cm 5 cm mass on the left carotid bifurcation. Post contrast T1 weighted and T2 MR images showed bilateral enhancing neck masses extending from the carotid artery bifurcation to the skull base with mass effect on the hypopharynx in Figs. 1 and 2. Abdominal USG, 24 h vanilmandeleic acid (VMA) levels and endocrinology consultation were found to be normal. Preoperative angiography and embolization were performed in this case. After the selective catheterization of the feeding artery with microcatheter (Boston Scientific, SL 10, MA, USA), the hypervascular tumor was located and total embolization of the paraganglioma was approved (Fig. 3). Under fluoroscopic control, embolization was performed using two vials of Contour polyvinyl alcohol particles (355– 500 lm) (Boston Scientific, Watertown, MA, USA) until complete flow stagnation was achieved (Fig. 3). During the
embolization procedure 100 ml of Ultravist 370 mg I/ml (Schering, Germany) was used. The satellite lesion after the embolization of the masses on the left side was seen in Fig. 3. The left side was operated first. In the operation 4 cm 3 cm, 5 cm 3 cm, encapsulated mass which was thought to be a carotid body tumor was dissected from carotid bifurcation (Fig. 4). An incidental 3 cm 2.5 cm 1.5 cm mass which seemed to be originated from vagus nerve, located posterior superior to the former mass was found and dissected too. Vagus nerve was sacrificed in order to totally excise the mass. Pathology revealed carotid body tumor and vagal paraganglioma (Fig. 5). Six months after the first operation, the mass on the right side was totally excised in the second operation. Pathologic diagnosis was again carotid body tumor (Fig. 5). A medialization thyroplasty was applied to the patient 4 months after the first operation. The patient is still in follow up and had no complaints.
3. Discussion Head and neck paragangliomas are found in two major locations; cervical (carotid, vagal) and skull base (jugular
E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
173
Fig. 4. An encapsulated mass thought to be as carotid body tumor was seen in the carotid bifurcation.
Fig. 3. Angiography showed highly vascularized carotid body masses with characteristic tumor blush and broadening of the carotid bifurcation. Note the satellite lesion after the embolization of the masses on the left side.
and tympanic) [2]. Carotid body tumors arise from neural crest cells at the level of carotid bifurcation. These highly vascularized tumors are rare benign tumors which grow slowly approximately 1 mm per year [2]. While there is only a neck mass at the beginning, symptoms such as pain, difficulty in swallowing and hoarseness will develop as the tumor gets bigger. Our patient had bilateral neck masses for the last 3 years and a recent tenderness around the masses.
Vagal paragangliomas are tumors originating from the nodose ganglion at the skull base. Parapharyngeal tumors are generally silent unless vagal or other nerves are compressed. Twenty-two percent of paragangliomas are reported to be nonfamilial multicentric [3]. Multiple tumors are more commonly seen in the familial variant. Familial paragangliomas are inherited as autusomal dominant with variable penetrance. The most common tumor is carotid body tumor. If there is a second tumor, this is also most likely the other carotid tumor. Multiple tumors are seen in 78–87% of familial paragangliomas [4]. Bilateral carotid body tumors are encountered in 31, 8% of familial cases and 4, 4% of nonfamilial cases [4]. Peripheral chemoreceptor function loss as a result of bilateral carotid body tumor excision can lead to arterial bioreflex fluctuations [5]. Carotid body tumors are more commonly seen in people living in high altitudes while bilateral cases are more common in people living in low altitudes [6]. Although multiple tumors are
Fig. 5. Nests of eosinophilic cells seperated by fibrous septa. The eosinophilic cells are bordered by spindle-shaped cells. (a) Carotid body (H&E, original magnification 200). (b) Vagal Paraganglioma (H&E, original magnification 400).
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E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
especially common in the familial variant, non-familial bilateral carotid body tumors has been found in our case. This disease may have a familial characteristic. Nonfamilial multicentric paragangliomas are reported from 4 to 22% and show familial, autosomal and variable penetrance. Familial carotid body tumors show many mutations, of which, the most common is SDHT mutation [7]. Malignant paragangliomas have been defined. However, malignancy is not primarily defined by tumor histology, instead according to the presence of lymph nodes and/or distant metastasis. Malignancy is reported in 5–13, 5% of paragangliomas [8]. Malignancy is more common in nonfamilial, sporadic cases [8]. Five-year survival in patient with lymph node metastasis and no systemic metastasis is 72% after postoperative radiotherapy [8]. CT and MRI are useful imaging procedures in case of suspicious paraganglioma. CT can reveal the skull base invasion and intracranial entension [9]. Salt cellar image in the MRI indicates vascular structures. Internal and external carotid arteries can be seen to incline from the bifurcation, and internal carotid artery can be compressed to posterior [9]. Intramural tumor can be shown with gadolinium studies. Vagal paragangliomas can compress carotid vessels. Digital substraction angiography is the best imaging technique before carotid body tumor operations [9]. Preoperative angiography and selective embolization are possible methods of management. Localization of the tumor and compression of the neighboring structures can lead to complications during resection [10]. Femoral angiography both gives opportunity for embolization and provides important clues to the surgeon [4,11]. Super selective angiography can show the artery supplying the tumor and venous structure in the late phase of arteriogram [10]. Multiple tumors in familial cases can be detected by angiography [3,10]. Although some authors prefer to use embolization in only larger tumors, there are many authors supporting the idea that embolization should be applied routinely as it decreases blood loss, facilitates easier dissection [3,10–12]. Tumor mass may reduce after embolization up to 25% due to loss of blood supply. Moreover, to define the cerebral tolerance during transient or permanent ligation of the internal carotid artery, techniques such as Xenon CT, observation of mental functions during 20 min balloon occlusion, detection of contra lateral cerebral blood flow by angiography have been advanced. Carotid body tumors are classified into three groups according to operative difficulties by Shamblin et al. [13]. 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. In our case, tumors were Shamblin Type II bilaterally. The risk of sequential bilateral surgery and necessity of post-operative follow up are important for the patients. Cranial nerves 9–12 may be affected by head and neck paragangliomas either primarily or as a result of
compression. Although carotid body tumors can totally excised by careful sub adventitial dissection, there are possibilities of massive bleeding and damage to neighboring structures [14]. Selective ligation of external carotid artery is applied by some authors while some avoid it [14]. Postoperative neurological deficits are seen as a result of nerve damage. During operation retraction or even scarification of the vagus nerve may be necessary. Superior vagal nerve lesions result in vocal cord paralysis, laryngeal anesthesia and palatal weakness. Dissection of vagal paragangliomas almost always necessitate scarification of vagus nerve at the level of nodose ganglion [4]. Rarely, if there is no skull base extension a careful dissection can protect function [10]. Jugular paraganglioma dissection can necessitate scarification of 9–12 nerves and postoperative voice, swallowing, and shoulder weakness can be anticipated. Medialization laryngoplasty may be applied in case of cord paralysis. Radiotherapy is suitable in patients with skull base and intracranial extensions, patients with multiple or bilateral tumors, or patients who cannot tolerate surgery and sometimes in combination with surgery [3,4,14]. Radiotherapy shrinks the tumor and in massive tumors this is an advantage to control tumor without significant morbidity. However, the risk of malignant degeneration after radiotherapy was reported [8].
References [1] Hallett Jr JW, Nora JD, Hollier LH, Cherry Jr KJ, Pairolero PC. Trends in neurovascular complications of surgical management for carotid body and cervical paragangliomas: a 50 year experience with 153 tumors. J Vasc Surg 1988;7:284–91. [2] Jansen JC, van den Berg R, Kuiper A, van der Mey AG, Zwinderman AH, Cornelisse CJ. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 2000;88:2811–6. [3] Gardner P, Dalsing M, Weisberger E, Sawchuk A, Miyamoto R. Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Am J Surg 1996;172:196–9. [4] Netterville JL, Reilly KM, Robertson D, Reiber ME, Armstrong WB, Childs P. Carotid body tumors: a review of 30 patients with 46 tumors. Laryngoscope 1995;105:115–26. [5] Timmers HJ, Karemaker JM, Wieling W. Baroreflex and chemoreflex function after bilateral carotid body tumor resection. J Hypertens 2003;21:591–9. [6] Rodriguez-Cuevas S, Lopez-Garza J, Labastida-Almendaro S. Carotid body tumors in inhabitants of altitudes higher than 2000 meters above sea level. Head Neck 1998;20:374–8. [7] Baysal BE, Willett-Brozick JE, Lawrence EC, Drovdlic CM, Savul SA, McLeod DR, et al. Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas. J Med Genet 2002;39:178–83. [8] Manolidis S, Shohet JA, Jackson CG, Glasscock ME. Malignant glomus tumors. Laryngoscope 1999;109:30–4. [9] van den Berg R, Wasser MN, van Gils AP, van der Mey AG, Hermans J, van Buchem MA. Vascularization of head and neck paragangliomas: comparison of three MR angiographic techniques with digital subtraction angiography. AJNR Am J Neuroradiol 2000;21: 162–70.
E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175 [10] Persky MS, Setton A, Niimi Y, Hartman J, Frank D, Berenstein A. Combined endovascular and surgical treatment head and neck pragangliomas—A team approach. Head Neck 2002;5: 423–31. [11] Anand VK, Alemar GO, Sanders TS. Management of the internal carotid artery during carotid body tumor surgery. Laryngoscope 1995;105:231–5.
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[12] Litle VR, Reilly LM, Ramos TK. Preoperative embolization of carotid body tumors: when is it appropriate? Ann Vasc Surg 1996;10:464–8. [13] Shamblin WR, ReMine WH, Sheps SG, Harrison Jr EG. Carotid body tumor (chemodectoma). Clinicopathologicanalysis of ninety cases. Am J Surg 1971;122:732–9. [14] Maier W, Marangos N, Laszig R. Paraganglioma as asystemic syndrome: pitfalls and strategies. J Laryngol Otol 1999;113:978–82.
Synchronous bilateral carotid body tumor and vagal paraganglioma: A case report and review of literature Erkan Karatas a,*, Akif Sirikci b, Tekin Baglam a, Semih Mumbuc a, Cengiz Durucu a, Ediz Tutar c, Muzaffer Kanlikama a a
Gaziantep University, School of Medicine, Department of Otorhinolaryngology, Head and Neck Surgery, Gaziantep, Turkey b Gaziantep University, School of Medicine, Department of Radiology, Gaziantep, Turkey c Gaziantep University, School of Medicine, Department of Pathology, Gaziantep, Turkey Received 28 October 2006; accepted 22 May 2007 Available online 11 September 2007
Abstract We present a case of synchronous bilateral carotid body tumor and vagal paraganglioma in a 49-year-old man who presented with 3 months history of tenderness and palpable neck masses bilaterally. An encapsulated mass which was thought to be a carotid body tumor and an incidental 3 cm 2 cm, 5 cm 1 cm, 5 cm mass which seemed to be originated from vagus nerve were dissected from the left side. Pathology revealed carotid body tumor and vagal paraganglioma. Six months after the first operation, the carotid body tumor on the right side was totally excised. A discussion of this case is followed by a review of the literature surrounding this rare clinic and pathological entity. # 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Neck; Synchronous; Carotid body tumor; Vagal paraganglioma; Surgery
1. Introduction Paragangliomas of the head and neck are distributed widely around vascular structures, along the autonomic nervous system as jugulotympanic, vagal and carotid bodies. Paragangliomas, previously named as chemodectomas, are highly vascularized tumors localized in blood vessel walls and along specific neurons [1]. In the head and neck, these lesions are most commonly located in four primary sites, the carotid bifurcation, jugular foramen, middle ear cavity, and along the course of the vagus nerve. Besides these sites orbit, larynx, and nasopharynx are the other locations. These tumors arise from extra adrenal paragangliar chief cells. Paragangliomas are encapsulated, brown and hard tumors which are formed by epithelial cell islands separated by a vascular fibrous stoma. Paragangliomas which do not show
catecholamine by chromaffin stainings are named as nonchromaffin paragangliomas [2,3]. Carotid body tumors usually present as slowly growing, painless neck masses. They are most commonly encountered in the fourth or fifth decades of life equally in both sexes [2–4]. 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. Bilateral paragangliomas are rarely encountered in nonfamilial cases. We have described non-familial bilateral carotid body tumors together with a left sided vagal paraganglioma in this study.
2. Case report * Corresponding author at: Gaziantep University, Medical Faculty, Department of Otorhinolaryngology, Head and Neck Surgery (KBB), Kilis ¨ zeri, 27310 Gaziantep, Turkey. Tel.: +90 342 3606060; Yolu U fax: +90 342 3603928. E-mail address: [email protected] (E. Karatas).
A 49-year-old male patient was presented with bilateral neck masses for the last 3 years and a recent tenderness around the masses. Otolaryngologic examination was negative except 5 cm 4 cm left and 4 cm 4 cm right
0385-8146/$ – see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2007.05.007
172
E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
Fig. 1. A hypointense, homogenously opaque, highly vascularized mass (m) on the right carotid bifurcation and a mass (m) on the left carotid bifurcation were seen in the post contrast T1 weighted MRI of patient.
Fig. 2. T2 weighted images showed bilateral enhancing neck masses (m) extending from the carotid artery bifurcation to the skull base with mass effect on the hypopharynx.
neck masses. These masses were pulsating with limited vertical mobility. Cranial nerve examinations were intact. Past medical history and family history were unremarkable. The magnetic resonance imaging (MRI) studies with contrast and the color Doppler ultrasonography (USG) revealed the hypointense, homogenously opaque, highly vascularized 3 cm 3 cm 2 cm mass on the right carotid bifurcation and a 4.5 cm 3 cm 5 cm mass on the left carotid bifurcation. Post contrast T1 weighted and T2 MR images showed bilateral enhancing neck masses extending from the carotid artery bifurcation to the skull base with mass effect on the hypopharynx in Figs. 1 and 2. Abdominal USG, 24 h vanilmandeleic acid (VMA) levels and endocrinology consultation were found to be normal. Preoperative angiography and embolization were performed in this case. After the selective catheterization of the feeding artery with microcatheter (Boston Scientific, SL 10, MA, USA), the hypervascular tumor was located and total embolization of the paraganglioma was approved (Fig. 3). Under fluoroscopic control, embolization was performed using two vials of Contour polyvinyl alcohol particles (355– 500 lm) (Boston Scientific, Watertown, MA, USA) until complete flow stagnation was achieved (Fig. 3). During the
embolization procedure 100 ml of Ultravist 370 mg I/ml (Schering, Germany) was used. The satellite lesion after the embolization of the masses on the left side was seen in Fig. 3. The left side was operated first. In the operation 4 cm 3 cm, 5 cm 3 cm, encapsulated mass which was thought to be a carotid body tumor was dissected from carotid bifurcation (Fig. 4). An incidental 3 cm 2.5 cm 1.5 cm mass which seemed to be originated from vagus nerve, located posterior superior to the former mass was found and dissected too. Vagus nerve was sacrificed in order to totally excise the mass. Pathology revealed carotid body tumor and vagal paraganglioma (Fig. 5). Six months after the first operation, the mass on the right side was totally excised in the second operation. Pathologic diagnosis was again carotid body tumor (Fig. 5). A medialization thyroplasty was applied to the patient 4 months after the first operation. The patient is still in follow up and had no complaints.
3. Discussion Head and neck paragangliomas are found in two major locations; cervical (carotid, vagal) and skull base (jugular
E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
173
Fig. 4. An encapsulated mass thought to be as carotid body tumor was seen in the carotid bifurcation.
Fig. 3. Angiography showed highly vascularized carotid body masses with characteristic tumor blush and broadening of the carotid bifurcation. Note the satellite lesion after the embolization of the masses on the left side.
and tympanic) [2]. Carotid body tumors arise from neural crest cells at the level of carotid bifurcation. These highly vascularized tumors are rare benign tumors which grow slowly approximately 1 mm per year [2]. While there is only a neck mass at the beginning, symptoms such as pain, difficulty in swallowing and hoarseness will develop as the tumor gets bigger. Our patient had bilateral neck masses for the last 3 years and a recent tenderness around the masses.
Vagal paragangliomas are tumors originating from the nodose ganglion at the skull base. Parapharyngeal tumors are generally silent unless vagal or other nerves are compressed. Twenty-two percent of paragangliomas are reported to be nonfamilial multicentric [3]. Multiple tumors are more commonly seen in the familial variant. Familial paragangliomas are inherited as autusomal dominant with variable penetrance. The most common tumor is carotid body tumor. If there is a second tumor, this is also most likely the other carotid tumor. Multiple tumors are seen in 78–87% of familial paragangliomas [4]. Bilateral carotid body tumors are encountered in 31, 8% of familial cases and 4, 4% of nonfamilial cases [4]. Peripheral chemoreceptor function loss as a result of bilateral carotid body tumor excision can lead to arterial bioreflex fluctuations [5]. Carotid body tumors are more commonly seen in people living in high altitudes while bilateral cases are more common in people living in low altitudes [6]. Although multiple tumors are
Fig. 5. Nests of eosinophilic cells seperated by fibrous septa. The eosinophilic cells are bordered by spindle-shaped cells. (a) Carotid body (H&E, original magnification 200). (b) Vagal Paraganglioma (H&E, original magnification 400).
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E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175
especially common in the familial variant, non-familial bilateral carotid body tumors has been found in our case. This disease may have a familial characteristic. Nonfamilial multicentric paragangliomas are reported from 4 to 22% and show familial, autosomal and variable penetrance. Familial carotid body tumors show many mutations, of which, the most common is SDHT mutation [7]. Malignant paragangliomas have been defined. However, malignancy is not primarily defined by tumor histology, instead according to the presence of lymph nodes and/or distant metastasis. Malignancy is reported in 5–13, 5% of paragangliomas [8]. Malignancy is more common in nonfamilial, sporadic cases [8]. Five-year survival in patient with lymph node metastasis and no systemic metastasis is 72% after postoperative radiotherapy [8]. CT and MRI are useful imaging procedures in case of suspicious paraganglioma. CT can reveal the skull base invasion and intracranial entension [9]. Salt cellar image in the MRI indicates vascular structures. Internal and external carotid arteries can be seen to incline from the bifurcation, and internal carotid artery can be compressed to posterior [9]. Intramural tumor can be shown with gadolinium studies. Vagal paragangliomas can compress carotid vessels. Digital substraction angiography is the best imaging technique before carotid body tumor operations [9]. Preoperative angiography and selective embolization are possible methods of management. Localization of the tumor and compression of the neighboring structures can lead to complications during resection [10]. Femoral angiography both gives opportunity for embolization and provides important clues to the surgeon [4,11]. Super selective angiography can show the artery supplying the tumor and venous structure in the late phase of arteriogram [10]. Multiple tumors in familial cases can be detected by angiography [3,10]. Although some authors prefer to use embolization in only larger tumors, there are many authors supporting the idea that embolization should be applied routinely as it decreases blood loss, facilitates easier dissection [3,10–12]. Tumor mass may reduce after embolization up to 25% due to loss of blood supply. Moreover, to define the cerebral tolerance during transient or permanent ligation of the internal carotid artery, techniques such as Xenon CT, observation of mental functions during 20 min balloon occlusion, detection of contra lateral cerebral blood flow by angiography have been advanced. Carotid body tumors are classified into three groups according to operative difficulties by Shamblin et al. [13]. 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. In our case, tumors were Shamblin Type II bilaterally. The risk of sequential bilateral surgery and necessity of post-operative follow up are important for the patients. Cranial nerves 9–12 may be affected by head and neck paragangliomas either primarily or as a result of
compression. Although carotid body tumors can totally excised by careful sub adventitial dissection, there are possibilities of massive bleeding and damage to neighboring structures [14]. Selective ligation of external carotid artery is applied by some authors while some avoid it [14]. Postoperative neurological deficits are seen as a result of nerve damage. During operation retraction or even scarification of the vagus nerve may be necessary. Superior vagal nerve lesions result in vocal cord paralysis, laryngeal anesthesia and palatal weakness. Dissection of vagal paragangliomas almost always necessitate scarification of vagus nerve at the level of nodose ganglion [4]. Rarely, if there is no skull base extension a careful dissection can protect function [10]. Jugular paraganglioma dissection can necessitate scarification of 9–12 nerves and postoperative voice, swallowing, and shoulder weakness can be anticipated. Medialization laryngoplasty may be applied in case of cord paralysis. Radiotherapy is suitable in patients with skull base and intracranial extensions, patients with multiple or bilateral tumors, or patients who cannot tolerate surgery and sometimes in combination with surgery [3,4,14]. Radiotherapy shrinks the tumor and in massive tumors this is an advantage to control tumor without significant morbidity. However, the risk of malignant degeneration after radiotherapy was reported [8].
References [1] Hallett Jr JW, Nora JD, Hollier LH, Cherry Jr KJ, Pairolero PC. Trends in neurovascular complications of surgical management for carotid body and cervical paragangliomas: a 50 year experience with 153 tumors. J Vasc Surg 1988;7:284–91. [2] Jansen JC, van den Berg R, Kuiper A, van der Mey AG, Zwinderman AH, Cornelisse CJ. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 2000;88:2811–6. [3] Gardner P, Dalsing M, Weisberger E, Sawchuk A, Miyamoto R. Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Am J Surg 1996;172:196–9. [4] Netterville JL, Reilly KM, Robertson D, Reiber ME, Armstrong WB, Childs P. Carotid body tumors: a review of 30 patients with 46 tumors. Laryngoscope 1995;105:115–26. [5] Timmers HJ, Karemaker JM, Wieling W. Baroreflex and chemoreflex function after bilateral carotid body tumor resection. J Hypertens 2003;21:591–9. [6] Rodriguez-Cuevas S, Lopez-Garza J, Labastida-Almendaro S. Carotid body tumors in inhabitants of altitudes higher than 2000 meters above sea level. Head Neck 1998;20:374–8. [7] Baysal BE, Willett-Brozick JE, Lawrence EC, Drovdlic CM, Savul SA, McLeod DR, et al. Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas. J Med Genet 2002;39:178–83. [8] Manolidis S, Shohet JA, Jackson CG, Glasscock ME. Malignant glomus tumors. Laryngoscope 1999;109:30–4. [9] van den Berg R, Wasser MN, van Gils AP, van der Mey AG, Hermans J, van Buchem MA. Vascularization of head and neck paragangliomas: comparison of three MR angiographic techniques with digital subtraction angiography. AJNR Am J Neuroradiol 2000;21: 162–70.
E. Karatas et al. / Auris Nasus Larynx 35 (2008) 171–175 [10] Persky MS, Setton A, Niimi Y, Hartman J, Frank D, Berenstein A. Combined endovascular and surgical treatment head and neck pragangliomas—A team approach. Head Neck 2002;5: 423–31. [11] Anand VK, Alemar GO, Sanders TS. Management of the internal carotid artery during carotid body tumor surgery. Laryngoscope 1995;105:231–5.
175
[12] Litle VR, Reilly LM, Ramos TK. Preoperative embolization of carotid body tumors: when is it appropriate? Ann Vasc Surg 1996;10:464–8. [13] Shamblin WR, ReMine WH, Sheps SG, Harrison Jr EG. Carotid body tumor (chemodectoma). Clinicopathologicanalysis of ninety cases. Am J Surg 1971;122:732–9. [14] Maier W, Marangos N, Laszig R. Paraganglioma as asystemic syndrome: pitfalls and strategies. J Laryngol Otol 1999;113:978–82.