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Rare localization of paraganglioma in head and neck
Auris, Nasus, Larynx 30 (2003) S149 /S152 www.elsevier.com/locate/anl
Rare localization of paraganglioma in head and neck Toshiharu Shintani a,*, Daisuke Oyake a, Ryoji Kanayama b, Toshifumi Takakuwa c, Izumi Koizuka a a
Department of Otolaryngology, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki 216-8511, Japan b Department of Otolaryngology, Mito Saiseikai General Hospital, Mito, Japan c Department of Pathology, St. Marianna University School of Medicine, Kawasaki 216-8511, Japan
Abstract In this paper, we describe the clinical course of a 61-year-old female patient with paraganglioma in the head and neck region. Computed tomographic scan (CT), magnetic resonance imaging (MRI), ultrasound scan (US) and arteriogram findings initially led us to suspect that this tumor originated in the vagal nerve. In particular, a color Doppler US enabled an easy diagnosis of hypervascular tumor. We removed this surgically, but the tumor was easy to peel from the vagal nerve and carotid bifurcation. The distal side of the tumor was under the digastric muscle and running into the hypoglossal nerve. The intraoperative findings were highly suggestive of localization at the hypoglossal nerve, although Xth and XIIth cranial nerve palsies have remained. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Paraganglioma; Doppler ultrasound scan; Hypoglossal nerve
1. Introduction Head and neck paragangliomas are rare tumors that originate in the glomus body located at the carotid body, jugular bulb and vagal or laryngeal paraganglia within the parasympathetic nervous system [1]. Ordinarily, diagnosis of their localizations is made from intraoperative observations and postoperative physical findings. In this case, we report a very rare localization of paraganglioma.
2. Case report A 61-year-old female presented with swelling in the left neck for the previous 18 months. The patient was referred to Mito Saiseikai General Hospital in December 1999. She complained of minimal discomfort in the left neck without tenderness. Her past and familial history were not remarkable. A mass was palpable in the left upper neck with minimal pulsation. It was 25 /25 mm in size, immo* Corresponding author. Tel.: /81-44-9778-111; fax: /81-44-9768748.
vable and with a smooth surface. There were no cranial nerve palsies. Blood and urine analyses were negative. CT showed a maximally enhancing mass in the left carotid space and the mass produced low- and highdensity signals in T1- and T2-weighted MRI, respectively. In particular, it had a characteristic ‘salt and pepper’ appearance on T1Gd contrast-enhanced images (Fig. 1A). A lateral subtraction arteriogram showed the hypervascular mass splaying the carotid bifurcation and the feeding vascular was seen from the external carotid artery (Fig. 1B). A color Doppler US revealed an iso- or hypoechoic solid tumor in front of the left internal carotid artery with hypervascularity (Fig. 2). A diagnosis of vagal paraganglioma was suspected. Surgery was performed in February 2000. An incision was made at the anterior border of the sternocleidomastoid muscle. During the procedure, a tumor was identified on the carotid bifurcation. The tumor was excised without the adherent carotid artery and Xth cranial nerve (Fig. 3A). The distal side of the tumor made way under the digastric muscle (Fig. 3B) and it was encased by a nerve (probably the XIIth). After removal of the tumor, we could not identify the hypoglossal nerve. Blood loss of 220 ml was recorded. The size of the excised specimen was 43 /19 /17 mm (Fig. 4A). A histopathological examination of the tumor
0385-8146/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 3 8 5 - 8 1 4 6 ( 0 2 ) 0 0 1 3 2 - 3
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paraganglioma localized to the hypoglossal nerve was made. The patient made an uneventful post-operative recovery, except for the Xth and XIIth cranial nerve palsies. Two years post-operatively, the XIIth cranial palsies remained, but she did not have swallowing disorders, the Xth palsy was improving and hoarseness was recovered completely.
3. Discussion
Fig. 1. (A) Axial view of T1Gd contrast-enhanced MRI, showing a tumor. (B) Arteriogram demonstrates the vascular tumor with anterior displacement of the internal carotid artery (lateral side view).
revealed nests of chief cells that had round or oval nuclei surrounded by fibrovascular stroma. It was designated as a ‘Zellbaren pattern’. No inflammation or malignancy was seen (Fig. 4B) and a definitive diagnosis of
Paraganglioma originates in the chief cells of the paraganglia (glomus body), which is distributed along the parasympathetic nerve and is associated with vascular and neural structures in the head and neck region [1]. There have been reports of paraganglioma located in the carotid body, jugulotympanic, vagal and laryngeal paraganglia [2,3]. There have also been very rare cases originating in the face [4], cheek [5], trachea [6], external ear canal [7] and frontal skull base [8]. Wilson [9] reported that paraganglioma is viable in the hypoglossal nerve, but it was not described in detail. An extensive search of the literature showed that there has only been one case of paraganglioma in the hypoglossal nerve that was metastasized from carotid body paraganglioma [10]. It is unusual to find paraganglioma originating in the hypoglossal nerve because it does not have a glomus body. Therefore, based on our CT, MRI, US and arteriogram investigations, we suspected that it originated in the carotid body or vagal nerve. Recent developments in image analysis have made the diagnoses of head and neck tumors easier, especially color Doppler US [11,12]. In the present case, we used US to measure the speed of blood flow in the tumor and peripheral blood vessels. The flow speeds in the arteries and veins in the tumor were similar to the peripheral ones*/this large blood flow led to our diagnosis of a tumor. The most appropriate treatment for head and neck paraganglioma is surgical removal because malignant change occurs in :/12% of sporadic cases [2]. An arteriogram would be helpful in deciding the surgical approach because it shows the blood supply, feeder and extent of the tumor [13,14]. 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 [15,16]. On the other hand, some reports have indicated that this procedure represents the risk of cerebral arterial occlusion [2,13]. In our case, we performed total extirpation because it was easy to confirm the feeder of the tumor and the tumor was small. This case illustrates some problems in diagnosing paraganglioma originating from the hypoglossal nerve. Firstly, this nerve does not contain the chief cells of
T. Shintani et al. / Auris, Nasus, Larynx 30 (2003) S149 /S152
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Fig. 2. Color Doppler US shows a smoothly demarcated, iso- or hypoechoic and highly vascularized solid tumor located between the internal and external carotid artery.
Fig. 3. Intraoperative photograph showing the tumor (arrowheads). (A) Tumor peeled from the vagal nerve (arrow) and carotid bifurcation (asterisk). (B) Distal side of tumor is under the digastric muscle and the tumor is running into the hypoglossal nerve (arrow).
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4. Conclusion We have presented a rare case of paraganglioma that arose from hypoglossal nerve. The diagnosis of this case depended on intraoperative observation.
Acknowledgements The authors wish to thank Dr O. Kato for his advice on the color Doppler US.
References
Fig. 4. (A) Photomacrograph shows excised specimen encased by nerve fiber. (B) Low power photomicrograph from tumor showing rounded nests of cells in a fibrovascular stroma with ‘Zellbaren pattern’ (H&E; /).
paraganglia, but there are some reports of very rare locations that do not involve a glomus body, such as the cheek, external ear canal and frontal skull base [5,7,8]. Most of those authors investigated whether paraganglion cells strayed into other regions or they did not provide sufficient detail. Secondly, our patient had both Xth and XIIth cranial nerve disorder after the surgery. The Xth nerve might have been damaged when the tumor was peeled from the vagus nerve, even though it was not adherent (Fig. 3B). Moreover, we could not find the hypoglossal nerve after the tumor removal. These findings lead to our diagnosis that this tumor originated in the hypoglossal nerve.
[1] Lack EE. Paraganglia of the head and neck region. In: Atlas of tumor pathology. Tumors of the adrenal gland and extra-adrenal paraganglioma. Washington, DC: Armed Forces Institute of Pathology, 1997:303 /8. [2] Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872 /7. [3] McCaffrey TV, Meyer FB, Michels VV, Piepgras DG, Marion MS. Familial paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1994;120:1211 /6. [4] Milroy EJG. Chemodectoma (non-chromaffin paraganglioma) of the face. Br J Surg 1969;7:510 /2. [5] DeLozier HL. Chemodectoma of the cheek. Ann Otol Rhinol Laryngol 1983;92:109 /12. [6] Horre´e WA. An unusual primary tumor of the trachea (chemodectoma). Pract Oto-Rhino-Laryngol 1963;25:125 /6. [7] Skinner LA, Curran AJ, Barnes C, Timon CI. Paraganglioma of the external auditory canal: an unusual case. J Laryngol Otol 2000;114:370 /2. [8] Yamaguchi T, Kubota M, Saeki N, Aihara N, Iwadate Y, Yamaura A. Paraganglioma in the frontal skull base. Neurol Med Chir 1999;39:308 /12. [9] Wilson H. Carotid body tumor. Surgery 1966;59:483 /93. [10] Marchesi M, Biffoni M, Jaus MO, Benedetti RN, Tromba L, Berni A, Campana FP. Surgical treatment of paraganglioma of the carotid body and other rare localizations. J Cardiovasc Surg 1999;40:691 /4. [11] Jansen JC, Baatenburg de Jong RJ, Schipper J, van der Mey GL, van Gils APG. Color Doppler imaging of paragangliomas in the neck. J Clin Ultrasound 1997;25:481 /5. [12] Steinke W, Hennerici M, Aulich A. Doppler color flow imaging of carotid body tumors. Stroke 1989;20:1574 /7. [13] Urquhart AC, Johnson JT, Myers EN, Schechter GL. Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope 1994;104:440 /5. [14] Moore G, Yarington CT, Mangham C. Vagal body tumors: diagnosis and treatment. Laryngoscope 1986;96:533 /6. [15] Higo R, Asai M, Sugasawa M, Takeuchi N, Nemoto S. Preoperative embolization for paraganglioma. Auris Nasus Larynx 1994;21:122 /5. [16] 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.
Rare localization of paraganglioma in head and neck Toshiharu Shintani a,*, Daisuke Oyake a, Ryoji Kanayama b, Toshifumi Takakuwa c, Izumi Koizuka a a
Department of Otolaryngology, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki 216-8511, Japan b Department of Otolaryngology, Mito Saiseikai General Hospital, Mito, Japan c Department of Pathology, St. Marianna University School of Medicine, Kawasaki 216-8511, Japan
Abstract In this paper, we describe the clinical course of a 61-year-old female patient with paraganglioma in the head and neck region. Computed tomographic scan (CT), magnetic resonance imaging (MRI), ultrasound scan (US) and arteriogram findings initially led us to suspect that this tumor originated in the vagal nerve. In particular, a color Doppler US enabled an easy diagnosis of hypervascular tumor. We removed this surgically, but the tumor was easy to peel from the vagal nerve and carotid bifurcation. The distal side of the tumor was under the digastric muscle and running into the hypoglossal nerve. The intraoperative findings were highly suggestive of localization at the hypoglossal nerve, although Xth and XIIth cranial nerve palsies have remained. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Paraganglioma; Doppler ultrasound scan; Hypoglossal nerve
1. Introduction Head and neck paragangliomas are rare tumors that originate in the glomus body located at the carotid body, jugular bulb and vagal or laryngeal paraganglia within the parasympathetic nervous system [1]. Ordinarily, diagnosis of their localizations is made from intraoperative observations and postoperative physical findings. In this case, we report a very rare localization of paraganglioma.
2. Case report A 61-year-old female presented with swelling in the left neck for the previous 18 months. The patient was referred to Mito Saiseikai General Hospital in December 1999. She complained of minimal discomfort in the left neck without tenderness. Her past and familial history were not remarkable. A mass was palpable in the left upper neck with minimal pulsation. It was 25 /25 mm in size, immo* Corresponding author. Tel.: /81-44-9778-111; fax: /81-44-9768748.
vable and with a smooth surface. There were no cranial nerve palsies. Blood and urine analyses were negative. CT showed a maximally enhancing mass in the left carotid space and the mass produced low- and highdensity signals in T1- and T2-weighted MRI, respectively. In particular, it had a characteristic ‘salt and pepper’ appearance on T1Gd contrast-enhanced images (Fig. 1A). A lateral subtraction arteriogram showed the hypervascular mass splaying the carotid bifurcation and the feeding vascular was seen from the external carotid artery (Fig. 1B). A color Doppler US revealed an iso- or hypoechoic solid tumor in front of the left internal carotid artery with hypervascularity (Fig. 2). A diagnosis of vagal paraganglioma was suspected. Surgery was performed in February 2000. An incision was made at the anterior border of the sternocleidomastoid muscle. During the procedure, a tumor was identified on the carotid bifurcation. The tumor was excised without the adherent carotid artery and Xth cranial nerve (Fig. 3A). The distal side of the tumor made way under the digastric muscle (Fig. 3B) and it was encased by a nerve (probably the XIIth). After removal of the tumor, we could not identify the hypoglossal nerve. Blood loss of 220 ml was recorded. The size of the excised specimen was 43 /19 /17 mm (Fig. 4A). A histopathological examination of the tumor
0385-8146/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 3 8 5 - 8 1 4 6 ( 0 2 ) 0 0 1 3 2 - 3
S150
T. Shintani et al. / Auris, Nasus, Larynx 30 (2003) S149 /S152
paraganglioma localized to the hypoglossal nerve was made. The patient made an uneventful post-operative recovery, except for the Xth and XIIth cranial nerve palsies. Two years post-operatively, the XIIth cranial palsies remained, but she did not have swallowing disorders, the Xth palsy was improving and hoarseness was recovered completely.
3. Discussion
Fig. 1. (A) Axial view of T1Gd contrast-enhanced MRI, showing a tumor. (B) Arteriogram demonstrates the vascular tumor with anterior displacement of the internal carotid artery (lateral side view).
revealed nests of chief cells that had round or oval nuclei surrounded by fibrovascular stroma. It was designated as a ‘Zellbaren pattern’. No inflammation or malignancy was seen (Fig. 4B) and a definitive diagnosis of
Paraganglioma originates in the chief cells of the paraganglia (glomus body), which is distributed along the parasympathetic nerve and is associated with vascular and neural structures in the head and neck region [1]. There have been reports of paraganglioma located in the carotid body, jugulotympanic, vagal and laryngeal paraganglia [2,3]. There have also been very rare cases originating in the face [4], cheek [5], trachea [6], external ear canal [7] and frontal skull base [8]. Wilson [9] reported that paraganglioma is viable in the hypoglossal nerve, but it was not described in detail. An extensive search of the literature showed that there has only been one case of paraganglioma in the hypoglossal nerve that was metastasized from carotid body paraganglioma [10]. It is unusual to find paraganglioma originating in the hypoglossal nerve because it does not have a glomus body. Therefore, based on our CT, MRI, US and arteriogram investigations, we suspected that it originated in the carotid body or vagal nerve. Recent developments in image analysis have made the diagnoses of head and neck tumors easier, especially color Doppler US [11,12]. In the present case, we used US to measure the speed of blood flow in the tumor and peripheral blood vessels. The flow speeds in the arteries and veins in the tumor were similar to the peripheral ones*/this large blood flow led to our diagnosis of a tumor. The most appropriate treatment for head and neck paraganglioma is surgical removal because malignant change occurs in :/12% of sporadic cases [2]. An arteriogram would be helpful in deciding the surgical approach because it shows the blood supply, feeder and extent of the tumor [13,14]. 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 [15,16]. On the other hand, some reports have indicated that this procedure represents the risk of cerebral arterial occlusion [2,13]. In our case, we performed total extirpation because it was easy to confirm the feeder of the tumor and the tumor was small. This case illustrates some problems in diagnosing paraganglioma originating from the hypoglossal nerve. Firstly, this nerve does not contain the chief cells of
T. Shintani et al. / Auris, Nasus, Larynx 30 (2003) S149 /S152
S151
Fig. 2. Color Doppler US shows a smoothly demarcated, iso- or hypoechoic and highly vascularized solid tumor located between the internal and external carotid artery.
Fig. 3. Intraoperative photograph showing the tumor (arrowheads). (A) Tumor peeled from the vagal nerve (arrow) and carotid bifurcation (asterisk). (B) Distal side of tumor is under the digastric muscle and the tumor is running into the hypoglossal nerve (arrow).
S152
T. Shintani et al. / Auris, Nasus, Larynx 30 (2003) S149 /S152
4. Conclusion We have presented a rare case of paraganglioma that arose from hypoglossal nerve. The diagnosis of this case depended on intraoperative observation.
Acknowledgements The authors wish to thank Dr O. Kato for his advice on the color Doppler US.
References
Fig. 4. (A) Photomacrograph shows excised specimen encased by nerve fiber. (B) Low power photomicrograph from tumor showing rounded nests of cells in a fibrovascular stroma with ‘Zellbaren pattern’ (H&E; /).
paraganglia, but there are some reports of very rare locations that do not involve a glomus body, such as the cheek, external ear canal and frontal skull base [5,7,8]. Most of those authors investigated whether paraganglion cells strayed into other regions or they did not provide sufficient detail. Secondly, our patient had both Xth and XIIth cranial nerve disorder after the surgery. The Xth nerve might have been damaged when the tumor was peeled from the vagus nerve, even though it was not adherent (Fig. 3B). Moreover, we could not find the hypoglossal nerve after the tumor removal. These findings lead to our diagnosis that this tumor originated in the hypoglossal nerve.
[1] Lack EE. Paraganglia of the head and neck region. In: Atlas of tumor pathology. Tumors of the adrenal gland and extra-adrenal paraganglioma. Washington, DC: Armed Forces Institute of Pathology, 1997:303 /8. [2] Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872 /7. [3] McCaffrey TV, Meyer FB, Michels VV, Piepgras DG, Marion MS. Familial paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1994;120:1211 /6. [4] Milroy EJG. Chemodectoma (non-chromaffin paraganglioma) of the face. Br J Surg 1969;7:510 /2. [5] DeLozier HL. Chemodectoma of the cheek. Ann Otol Rhinol Laryngol 1983;92:109 /12. [6] Horre´e WA. An unusual primary tumor of the trachea (chemodectoma). Pract Oto-Rhino-Laryngol 1963;25:125 /6. [7] Skinner LA, Curran AJ, Barnes C, Timon CI. Paraganglioma of the external auditory canal: an unusual case. J Laryngol Otol 2000;114:370 /2. [8] Yamaguchi T, Kubota M, Saeki N, Aihara N, Iwadate Y, Yamaura A. Paraganglioma in the frontal skull base. Neurol Med Chir 1999;39:308 /12. [9] Wilson H. Carotid body tumor. Surgery 1966;59:483 /93. [10] Marchesi M, Biffoni M, Jaus MO, Benedetti RN, Tromba L, Berni A, Campana FP. Surgical treatment of paraganglioma of the carotid body and other rare localizations. J Cardiovasc Surg 1999;40:691 /4. [11] Jansen JC, Baatenburg de Jong RJ, Schipper J, van der Mey GL, van Gils APG. Color Doppler imaging of paragangliomas in the neck. J Clin Ultrasound 1997;25:481 /5. [12] Steinke W, Hennerici M, Aulich A. Doppler color flow imaging of carotid body tumors. Stroke 1989;20:1574 /7. [13] Urquhart AC, Johnson JT, Myers EN, Schechter GL. Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope 1994;104:440 /5. [14] Moore G, Yarington CT, Mangham C. Vagal body tumors: diagnosis and treatment. Laryngoscope 1986;96:533 /6. [15] Higo R, Asai M, Sugasawa M, Takeuchi N, Nemoto S. Preoperative embolization for paraganglioma. Auris Nasus Larynx 1994;21:122 /5. [16] 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.