Clinical Review & Education
Clinical Problem Solving | RADIOLOGY
Carotid Space Mass Gavriel David Kohlberg, MD; Brian Jacob Stater, MD; David Ivan Kutler, MD; William Isadore Kuhel, MD; Marc Andrew Cohen, MD
A
B
C
D
Figure. A heterogeneous avascular lesion of the left side of the neck encircling carotid artery. A, T1-weighted magnetic resonance image (MRI), axial view. B, T2-weighted MRI, axial view. C, Diffusion-weighted MRI, axial view. D, Positron emission tomographic scan, coronal view.
A man in his 40s presented with a 3-month history of sore throat and neck discomfort. The patient was an active smoker from Georgia, the country fourth most affected by the Chernobyl nuclear accident in Ukraine. Findings from his physical examination were significant for a normal thyroid but with a vague feeling of fullness in the left side of the neck. The patient underwent neck ultrasonography, which revealed a heterogeneous avascular lesion of the left side of the neck that encircled the carotid artery. On magnetic resonance imaging (MRI) there was a homogeneous T1-weighted
jamaotolaryngology.com
hypointense, T2-weighted intermediate-intensity, uniformly enhancing mass that also demonstrated restricted diffusion (Figure, A-C). The mass, which encased the left common carotid artery, extended from the arch at the carotid origin superiorly to the level of C5. On positron emission tomography (PET), a mass corresponding to that seen on MRI was fluorodeoxyglucose F 18 (FDG)-avid (Figure, D). The patient underwent a fine-needle aspiration and incisional biopsy of the carotid space mass. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery December 2014 Volume 140, Number 12
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Karolinska Institutet University Library User on 06/01/2015
1237
Clinical Review & Education Clinical Problem Solving
Diagnosis Carotid space lymphoma
Discussion Histologic examination of fine-needle aspirate biopsy of the mass revealed an atypical proliferation of lymphocytes. The diagnosis of diffuse large B-cell non-Hodgkin lymphoma was confirmed by an incisional biopsy. Immunohistochemical staining was positive for antigens CD20, CD23, and CD10 and proteins PAX5, BCL6, MUM1, and BCL2, and was negative for protein BCL1 and terminal transferase. Test results for Epstein-Barr virus in situ hybridization were negative. Findings from a bone marrow biopsy were negative for malignant disease. The patient underwent R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) combination chemotherapy for stage II germinal center B-cell–like subtype of diffuse large B-cell non-Hodgkin lymphoma. In contrast with lymphoma presenting as nodal disease along the jugulodigastric chain, in this case the lymphoma presented in the extranodal carotid space. Primary extranodal lymphoma of the carotid space is a rare finding, to our knowledge not previously described in the literature. The carotid space runs from the carotid canal at the skull base superiorly to the thoracic inlet inferiorly. It is bounded by the carotid sheath, which contains the carotid artery, internal jugular vein, vagus nerve, ansa cervicalis, and cervical sympathetic plexus. In addition, the glossopharyngeal, spinal accessory and hypoglossal nerves all pierce the carotid sheath superiorly.1 Lesions arising in the carotid sheath include vascular lesions, infectious processes, neurogenic schwannomas and neurofibromas, chromaphil derived paragangliomas, and metastatic extracapsular spread of carcinoma and lymphoma. In addition, rare lesions of the carotid space include meningioma, cavernous hemangioma, solitary fibrous tumor, and lipoma.2-4 Computed tomography (CT), MRI, ARTICLE INFORMATION Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, Weill Cornell Medical College, New York, New York. Corresponding Author: Gavriel David Kohlberg, MD, Department of Otolaryngology–Head and Neck Surgery, Weill Cornell Medical College, 1305 York Ave, Fifth Floor, New York, NY 10021 (gkohlberg @gmail.com). Section Editor: C. Douglas Phillips, MD. Published Online: November 6, 2014. doi:10.1001/jamaoto.2014.2523. Conflict of Interest Disclosures: None reported. Previous Presentation: This study was a poster presentation at the Fifth World Congress of the International Federation of Head and Neck Oncologic Societies and the Annual Meeting of the
1238
angiography, ultrasonography, and PET can be used to radiographically evaluate carotid space masses. Hodgkin and non-Hodgkin lymphoma present as single or multiple enlarged nodes with variable enhancement on CT. On MRI, both Hodgkin and non-Hodgkin lymphoma classically appear as T1-weighted hypointense, intermediate to low signal intensity T2-weighted lesions that uniformly and intensely enhance with gadolinium while also demonstrating restricted diffusion on diffusion-weighted imaging. Lymphoma demonstrates FDG avidity on PET.5 Lymphoma is a commonly occurring malignant neoplasm in the head and neck and can occur in multiple locations with a myriad of presentations. Hodgkin lymphoma tends to afflict younger patients in their second and third decades of life, whereas nonHodgkin lymphoma has a mean age at occurrence in the sixth decade of life. Head and neck lymphoma usually involves the jugulodigastric lymph nodes. In contrast with Hodgkin lymphoma, non-Hodgkin lymphoma is more likely to involve noncontiguous lymph nodes, to have extranodal involvement, and to be necrotic.6 Four to five percent of patients with Hodgkin lymphoma present with extranodal involvement in the head and neck compared with 23% to 30% in those with non-Hodgkin lymphoma. Waldeyer ring is the most common site of extranodal involvement in the head and neck, accounting for about 50% of cases. Other sites of extranodal involvement include the orbit, parotid, brain, nasopharynx, hypopharynx, larynx, paranasal sinuses, and uvula.5,7 Lymphoma is diagnosed by evaluation of involved tissue by microscopy and immunohistochemical analysis. While a fine-needle aspiration biopsy or a core needle biopsy may suggest a diagnosis of lymphoma, an incisional or excisional biopsy is usually necessary to classify the lymphoma in preparation for type-specific chemotherapy or radiation therapy.8
American Head & Neck Society; July 26-30, 2014; New York, New York. REFERENCES 1. Kuwada C, Mannion K, Aulino JM, Kanekar SG. Imaging of the carotid space. Otolaryngol Clin North Am. 2012;45(6):1273-1292. 2. Antonopoulos C, Karagianni M, Zolindaki C, Anagnostou E, Vagianos C. Cavernous hemangioma of infrahyoid carotid sheath and review of the literature on carotid sheath tumors. Head Neck. 2009;31(10):1381-1386. 3. Parelkar S, Kapadnis S, Sanghvi B, et al. Carotid sheath lipoma: first case report in the English literature. Ann R Coll Surg Engl. 2013;95(5):e77-e79.
5. Aiken AH, Glastonbury C. Imaging Hodgkin and non-Hodgkin lymphoma in the head and neck. Radiol Clin North Am. 2008;46(2):363-378, ix-x. 6. Urquhart A, Berg R. Hodgkin’s and non-Hodgkin’s lymphoma of the head and neck. Laryngoscope. 2001;111(9):1565-1569. 7. Artese L, Di Alberti L, Lombardo M, Liberatore E, Piattelli A. Head and neck non-Hodgkin’s lymphomas. Eur J Cancer B Oral Oncol. 1995;31B(5): 299-300. 8. Ryu YJ, Cha W, Jeong WJ, Choi SI, Ahn SH. Diagnostic role of core needle biopsy in cervical lymphadenopathy [published online December 22, 2013]. Head Neck. doi:10.1002/hed.23580.
4. Gómez-Oliveira G, Alvarez-Flores M, Arribas-García I, Martínez-Gimeno C. Solitary fibrous tumor surrounding the carotid sheath. Med Oral Patol Oral Cir Bucal. 2010;15(2):e395-e397.
JAMA Otolaryngology–Head & Neck Surgery December 2014 Volume 140, Number 12
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Karolinska Institutet University Library User on 06/01/2015
jamaotolaryngology.com
Clinical Problem Solving | RADIOLOGY
Carotid Space Mass Gavriel David Kohlberg, MD; Brian Jacob Stater, MD; David Ivan Kutler, MD; William Isadore Kuhel, MD; Marc Andrew Cohen, MD
A
B
C
D
Figure. A heterogeneous avascular lesion of the left side of the neck encircling carotid artery. A, T1-weighted magnetic resonance image (MRI), axial view. B, T2-weighted MRI, axial view. C, Diffusion-weighted MRI, axial view. D, Positron emission tomographic scan, coronal view.
A man in his 40s presented with a 3-month history of sore throat and neck discomfort. The patient was an active smoker from Georgia, the country fourth most affected by the Chernobyl nuclear accident in Ukraine. Findings from his physical examination were significant for a normal thyroid but with a vague feeling of fullness in the left side of the neck. The patient underwent neck ultrasonography, which revealed a heterogeneous avascular lesion of the left side of the neck that encircled the carotid artery. On magnetic resonance imaging (MRI) there was a homogeneous T1-weighted
jamaotolaryngology.com
hypointense, T2-weighted intermediate-intensity, uniformly enhancing mass that also demonstrated restricted diffusion (Figure, A-C). The mass, which encased the left common carotid artery, extended from the arch at the carotid origin superiorly to the level of C5. On positron emission tomography (PET), a mass corresponding to that seen on MRI was fluorodeoxyglucose F 18 (FDG)-avid (Figure, D). The patient underwent a fine-needle aspiration and incisional biopsy of the carotid space mass. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery December 2014 Volume 140, Number 12
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Karolinska Institutet University Library User on 06/01/2015
1237
Clinical Review & Education Clinical Problem Solving
Diagnosis Carotid space lymphoma
Discussion Histologic examination of fine-needle aspirate biopsy of the mass revealed an atypical proliferation of lymphocytes. The diagnosis of diffuse large B-cell non-Hodgkin lymphoma was confirmed by an incisional biopsy. Immunohistochemical staining was positive for antigens CD20, CD23, and CD10 and proteins PAX5, BCL6, MUM1, and BCL2, and was negative for protein BCL1 and terminal transferase. Test results for Epstein-Barr virus in situ hybridization were negative. Findings from a bone marrow biopsy were negative for malignant disease. The patient underwent R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) combination chemotherapy for stage II germinal center B-cell–like subtype of diffuse large B-cell non-Hodgkin lymphoma. In contrast with lymphoma presenting as nodal disease along the jugulodigastric chain, in this case the lymphoma presented in the extranodal carotid space. Primary extranodal lymphoma of the carotid space is a rare finding, to our knowledge not previously described in the literature. The carotid space runs from the carotid canal at the skull base superiorly to the thoracic inlet inferiorly. It is bounded by the carotid sheath, which contains the carotid artery, internal jugular vein, vagus nerve, ansa cervicalis, and cervical sympathetic plexus. In addition, the glossopharyngeal, spinal accessory and hypoglossal nerves all pierce the carotid sheath superiorly.1 Lesions arising in the carotid sheath include vascular lesions, infectious processes, neurogenic schwannomas and neurofibromas, chromaphil derived paragangliomas, and metastatic extracapsular spread of carcinoma and lymphoma. In addition, rare lesions of the carotid space include meningioma, cavernous hemangioma, solitary fibrous tumor, and lipoma.2-4 Computed tomography (CT), MRI, ARTICLE INFORMATION Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, Weill Cornell Medical College, New York, New York. Corresponding Author: Gavriel David Kohlberg, MD, Department of Otolaryngology–Head and Neck Surgery, Weill Cornell Medical College, 1305 York Ave, Fifth Floor, New York, NY 10021 (gkohlberg @gmail.com). Section Editor: C. Douglas Phillips, MD. Published Online: November 6, 2014. doi:10.1001/jamaoto.2014.2523. Conflict of Interest Disclosures: None reported. Previous Presentation: This study was a poster presentation at the Fifth World Congress of the International Federation of Head and Neck Oncologic Societies and the Annual Meeting of the
1238
angiography, ultrasonography, and PET can be used to radiographically evaluate carotid space masses. Hodgkin and non-Hodgkin lymphoma present as single or multiple enlarged nodes with variable enhancement on CT. On MRI, both Hodgkin and non-Hodgkin lymphoma classically appear as T1-weighted hypointense, intermediate to low signal intensity T2-weighted lesions that uniformly and intensely enhance with gadolinium while also demonstrating restricted diffusion on diffusion-weighted imaging. Lymphoma demonstrates FDG avidity on PET.5 Lymphoma is a commonly occurring malignant neoplasm in the head and neck and can occur in multiple locations with a myriad of presentations. Hodgkin lymphoma tends to afflict younger patients in their second and third decades of life, whereas nonHodgkin lymphoma has a mean age at occurrence in the sixth decade of life. Head and neck lymphoma usually involves the jugulodigastric lymph nodes. In contrast with Hodgkin lymphoma, non-Hodgkin lymphoma is more likely to involve noncontiguous lymph nodes, to have extranodal involvement, and to be necrotic.6 Four to five percent of patients with Hodgkin lymphoma present with extranodal involvement in the head and neck compared with 23% to 30% in those with non-Hodgkin lymphoma. Waldeyer ring is the most common site of extranodal involvement in the head and neck, accounting for about 50% of cases. Other sites of extranodal involvement include the orbit, parotid, brain, nasopharynx, hypopharynx, larynx, paranasal sinuses, and uvula.5,7 Lymphoma is diagnosed by evaluation of involved tissue by microscopy and immunohistochemical analysis. While a fine-needle aspiration biopsy or a core needle biopsy may suggest a diagnosis of lymphoma, an incisional or excisional biopsy is usually necessary to classify the lymphoma in preparation for type-specific chemotherapy or radiation therapy.8
American Head & Neck Society; July 26-30, 2014; New York, New York. REFERENCES 1. Kuwada C, Mannion K, Aulino JM, Kanekar SG. Imaging of the carotid space. Otolaryngol Clin North Am. 2012;45(6):1273-1292. 2. Antonopoulos C, Karagianni M, Zolindaki C, Anagnostou E, Vagianos C. Cavernous hemangioma of infrahyoid carotid sheath and review of the literature on carotid sheath tumors. Head Neck. 2009;31(10):1381-1386. 3. Parelkar S, Kapadnis S, Sanghvi B, et al. Carotid sheath lipoma: first case report in the English literature. Ann R Coll Surg Engl. 2013;95(5):e77-e79.
5. Aiken AH, Glastonbury C. Imaging Hodgkin and non-Hodgkin lymphoma in the head and neck. Radiol Clin North Am. 2008;46(2):363-378, ix-x. 6. Urquhart A, Berg R. Hodgkin’s and non-Hodgkin’s lymphoma of the head and neck. Laryngoscope. 2001;111(9):1565-1569. 7. Artese L, Di Alberti L, Lombardo M, Liberatore E, Piattelli A. Head and neck non-Hodgkin’s lymphomas. Eur J Cancer B Oral Oncol. 1995;31B(5): 299-300. 8. Ryu YJ, Cha W, Jeong WJ, Choi SI, Ahn SH. Diagnostic role of core needle biopsy in cervical lymphadenopathy [published online December 22, 2013]. Head Neck. doi:10.1002/hed.23580.
4. Gómez-Oliveira G, Alvarez-Flores M, Arribas-García I, Martínez-Gimeno C. Solitary fibrous tumor surrounding the carotid sheath. Med Oral Patol Oral Cir Bucal. 2010;15(2):e395-e397.
JAMA Otolaryngology–Head & Neck Surgery December 2014 Volume 140, Number 12
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Karolinska Institutet University Library User on 06/01/2015
jamaotolaryngology.com
