Figure 1
A 30-year-old male patient presented complaining of throat pain and difficulty in swallowing for 1 month. On examination a mucosal bulge was noted on the right pharyngeal wall.
Carotid body paraganglioma
Vagal paraganglioma
Pathological lymph node
Nerve sheath tumour
Contrast-enhanced CT evaluation revealed a well-defined, ovoid, homogeneously enhancing soft tissue density mass involving the right carotid space (Fig. 2, 3, 4). The lesion caused splaying of right internal and external carotid artery (Fig. 2c; 4c, d; 5). The location of the lesion was confirmed by lateral displacement of the styloid process (Fig. 2a - red arrow), anterior displacement of para-pharyngeal fat (Fig. 2a - blue arrow) and medial displacement of the right pharyngeal wall (Fig. 2a - yellow arrow).
Non-contrast MRI evaluation revealed a well-defined lesion, isointense on T1-weighted imaging and hyperintense on T2-weighted imaging, involving the right carotid space (Fig. 3, 4). T2-weighted imaging showed typical 'salt and pepper' appearance (Fig. 3a, b).
Non-contrast MRI evaluation revealed a well-defined lesion, isointense on T1-weighted imaging and hyperintense on T2-weighted imaging, involving the right carotid space (Fig. 3, 4). T2-weighted imaging showed typical 'salt and pepper' appearance (Fig. 3a, b).
Paraganglioma are tumours arising from paraganglia (non-neuronal cells derived from neural crest cells) anywhere in the body [1, 2]. Common locations include the carotid body, vagus nerve in the head and neck region, jugular foramen, middle ear, aorto-pulmonary window and organ of Zuckerkandl. They comprise 0.6 % of all head and neck neoplasms [3]. Carotid body and jugular foramen paragangliomas account for 80% of paragangliomas in head and neck [4].
They can present as sporadic and familial forms. The sporadic form is commonly seen in the 45-50 year age group with no gender predilection [5, 6]. Its aetiology is unknown. Lesions present as a slow growing, painless lateral neck mass. Associated symptoms include cranial nerve palsies, change in voice and hearing loss [5, 7, 10]. The familial form, usually presenting bilaterally, accounts for 10% of cases and is inherited as a component of multiple endocrine neoplasia, tuberous sclerosis, neurofibromatosis and von Hippel-Lindau syndrome.
Paragangliomas in general show homogeneous and intense enhancement after IV administration of contrast material because of their hyper-vascularity. T2- weighted MR imaging shows the presence of a hyperintense mass with internal hypointense areas giving a 'salt-and-pepper' appearance. 'Salt' denotes hyperintense areas of haemorrhage whereas 'pepper' denotes hypointense vascular flow voids [11]. Splaying of the internal and external carotid arteries demonstrated on contrast-enhanced CT and angiography is known as the lyre sign, consistent with a carotid body tumour [12]. Angiographic confirmation of hypervascularity is noted as intense tumour blush. Thus, a salt-and-pepper appearance on MRI, splaying of internal and external carotid vessel and tumoral hyper-vascularity clinch the diagnosis of carotid body paraganglioma [17, 18].
The differential diagnosis includes vagal paraganglioma and nerve sheath tumour. Vagal paraganglioma tends to displace both the external and internal carotid arteries antero-medially [13, 14]. Nerve sheath tumours also displace both arteries antero-medially but lack the hypervascularity of a paraganglioma.
Surgical excision is the treatment of choice. Preoperative angiography is useful for surgical planning and preoperative embolisation [11, 15]. Embolisation promotes tumour shrinkage and reduces blood loss during surgery. Radiotherapy may be considered where surgery is not an option or indicated.
Teaching point: Carotid body paraganglioma can be readily diagnosed based on its CT, MRI and angiographic appearances.
They can present as sporadic and familial forms. The sporadic form is commonly seen in the 45-50 year age group with no gender predilection [5, 6]. Its aetiology is unknown. Lesions present as a slow growing, painless lateral neck mass. Associated symptoms include cranial nerve palsies, change in voice and hearing loss [5, 7, 10]. The familial form, usually presenting bilaterally, accounts for 10% of cases and is inherited as a component of multiple endocrine neoplasia, tuberous sclerosis, neurofibromatosis and von Hippel-Lindau syndrome.
Paragangliomas in general show homogeneous and intense enhancement after IV administration of contrast material because of their hyper-vascularity. T2- weighted MR imaging shows the presence of a hyperintense mass with internal hypointense areas giving a 'salt-and-pepper' appearance. 'Salt' denotes hyperintense areas of haemorrhage whereas 'pepper' denotes hypointense vascular flow voids [11]. Splaying of the internal and external carotid arteries demonstrated on contrast-enhanced CT and angiography is known as the lyre sign, consistent with a carotid body tumour [12]. Angiographic confirmation of hypervascularity is noted as intense tumour blush. Thus, a salt-and-pepper appearance on MRI, splaying of internal and external carotid vessel and tumoral hyper-vascularity clinch the diagnosis of carotid body paraganglioma [17, 18].
The differential diagnosis includes vagal paraganglioma and nerve sheath tumour. Vagal paraganglioma tends to displace both the external and internal carotid arteries antero-medially [13, 14]. Nerve sheath tumours also displace both arteries antero-medially but lack the hypervascularity of a paraganglioma.
Surgical excision is the treatment of choice. Preoperative angiography is useful for surgical planning and preoperative embolisation [11, 15]. Embolisation promotes tumour shrinkage and reduces blood loss during surgery. Radiotherapy may be considered where surgery is not an option or indicated.
Teaching point: Carotid body paraganglioma can be readily diagnosed based on its CT, MRI and angiographic appearances.
References
[1] Lattes R (1950) Nonchromaffin paraganglioma of ganglion nodosum, carotid body, and aortic-arch bodies. Cancer 3:667-694 (PMID: 15427070)
[2] Kliewer KE, Cochran AJ (1989) A review of the histology, ultrastructure, immunohistology, and molecular biology of extra-adrenal paragangliomas. Arch Pathol Lab Med 113:1209–1218 (PMID: 2684087)
[3] Borba LA, Al-Mefty O (1996) Intravagal paragangliomas: report of four cases. Neurosurgery 38:569–575 (PMID: 8837811)
[4] Kliewer KE, Wen D-R, Cancilla PA, Cochran AJ (1989) Paragangliomas: assessment of prognosis by histologic, immunohistochemical, and ultrastructural techniques. Hum Pathol 20:29–39 (PMID: 2912871)
[5] Gad Amr, Sayed Ahmed (2014) Carotid body tumors: a review of 25 years experience in diagnosis and management of 56 tumors. Ann Vasc Dis 7(3):292–299 (PMID: 25298832)
[6] Kay JM, Laidler P (1977) Hypoxia and the carotid body. J Clin Pathol Suppl (R Coll Pathol) 11:30–44 (PMID: 198435)
[7] Wienek Jacqueline A, Smith Alice (2009) Paraganglioma: carotid body tumor. Head Neck Pathol 3(4):303–306 (PMID: 20016787)
[8] Hensen Erik F, Bayley Jean-Pierre (2011) Recent advances in the genetics of SDH-related paraganglioma and pheochromocytoma. Fam Cancer 10(2):355–363 (PMID: 21082267)
[9] Baez Juan C, Jagannathan Jyoyhi P (2012) Pheochromocytoma and paraganglioma: imaging characteristics. Cancer Imaging 12 (1):153–162 (PMID: 22571874)
[10] Boedeker Carsten Christol (2011) Paragangliomas and Paraganglioma Syndromes. GMS Curr Top Otorhinolaryngol Head Neck Surg 10 : Doc 03 (PMID: 22558053)
[11] Olsen WL, Dillon WP, Kelly WM, Norman D, Brant-Zawadzki M, Newton TH (1987) MR imaging of paragangliomas. AJR 148:201–204 (PMID: 3024473)
[12] Som PM, Sacher M, Stollman AL, Biller HF, Lawson W (1988) Common tumors of the para-pharyngeal space: refined imaging diagnosis. Radiology 169:81–85 (PMID: 2843942)
[13] Rao AB, Koeller KK, Adair CF (1999) From the archives of the AFIP; Paragangliomas of the head and neck: radiologic–pathologic correlation. Radiographics 19:1605–1632 (PMID: 10555678)
[14] Som PM, Biller HF, Lawson W, Sacher M, Lanzieri CF (1984) Para-pharyngeal space masses: an updated protocol based upon 104 cases. Radiology 153:149–156 (PMID: 6089262)
[15] Duncan AW, Lack EE, Deck MF (1979) Radiological evaluation of paragangliomas of the head and neck. Radiology 132:99–105 (PMID: 451228)
[16] Elisabetta Zanoletti, Antonio Mazzoni. (2006) Vagal Paraganglioma. Skull Base 16:161-168 (PMID: 1586175)
[17] A.B Rao, Kelly K. Koeller, Carol F. Adair (1999) From the Archives of the AFIP. Paragangliomas of the Head and Neck: radiologic-pathologic correlation. Radiographics 19:1605-1632 (PMID: 10555678)
[18] Grotemeyer D1, Loghmanieh SM, Pourhassan S, Sagban TA, Iskandar F, Reinecke P, Sandmann W. (2009) Dignity of carotid body tumors. Review of the literature and clinical experiences. Chirurg 80(9):854-63 (PMID: 19458915)
Case information
| URL: | https://www.eurorad.org/case/15320 |
| DOI: | 10.1594/EURORAD/CASE.15320 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 1
Department of Radio-diagnosis, Shri M.P Shah Medical College, G.G Hospital, Jamnagar, Gujarat, India.
Department of Radio-diagnosis, Shri M.P Shah Medical College, G.G Hospital, Jamnagar, Gujarat, India.
Department of Radio-diagnosis, Shri M.P Shah Medical College, G.G Hospital, Jamnagar, Gujarat, India.
Department of Radio-diagnosis, Shri M.P Shah Medical College, G.G Hospital, Jamnagar, Gujarat, India.
Figure 2
Rosai J,ed. Atlas of tumor pathology, Ser 3 fasc 19.Washington DC: Armed Forces Institute of Pathology.
Figure 3
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Figure 4
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
Department of Radiodiagnosis, Shri M P Shah medical college, G G Hospital, Jamnagar, Gujarat, India.
