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Molly Kantor, 2010 Gillian Lieberman, MD August 2010 The Radiologic Exploration of a Carotid Body Tumor Molly Kantor, Harvard Medical School Year III Gillian Lieberman, MD Molly Kantor, 2010 Gillian Lieberman, MD Agenda • • • • • • • Patient History and Physical Exam Findings Differential Diagnosis of a Neck Mass Anatomy Review Patient Imaging and Refining the Differential Overview of Carotid Body Tumors Menu of Tests to image Carotid Body Tumors Our Patient’s Interventional Radiology Images 2 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s History and Physical exam Molly Kantor, 2010 Gillian Lieberman, MD Our Patient: History • 29 year old nurse • Incidental finding on neck MRI at OSH for whiplash s/p motor vehicle accident • Aware of a lump in her left neck since age 17, believed to be a lymph node but not evaluated • Asymptomatic – Denies pain, dysphagia, odynophagia, hoarseness, change in voice • PMH & PSH: Cesarean section, Hashimoto thyroiditis, nasal septoplasty • SHx: denies smoking or drinking 4 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient: Physical Exam • Exam: – Firm, nontender soft‐tissue mass in the left neck • Moves side‐to‐side but not up and down • Close in proximity to carotid pulsation – No palpable lymphadenopathy – No masses on the right – No bruit auscultated – No thyroid nodules palpated – Normal laryngoscopic examination 5 Molly Kantor, 2010 Gillian Lieberman, MD Differential Diagnosis Molly Kantor, 2010 Gillian Lieberman, MD Differential Diagnosis of Neck Mass • Congenital – Usually present at birth but may present at any age • Inflammatory – Typically viral and last < 6 weeks • Neoplastic – Both benign and malignant – In an adult, suspect malignancy first 7 Molly Kantor, 2010 Gillian Lieberman, MD DDx: Congenital • Branchial cleft cyst – First, second, or third • • • • Thyroglossal duct cyst Dermoid cyst Thymic cyst Vascular anomalies – hemangiomas, malformations, lymphatic malformations • Ranula • Teratoma 8 Molly Kantor, 2010 Gillian Lieberman, MD DDx: Inflammatory • Reactive lymphadenopathy (infectious): – Viral – most common – Bacterial – including unusual bacteria such as tularemia, brucellosis, toxoplasmosis – HIV‐associated • Non‐infectious lymphadenopathy: – Including sarcoidosis, Castleman disease, Rosai‐ Dorfman disease, Kawasaki disease (children < 5) 9 Molly Kantor, 2010 Gillian Lieberman, MD DDx: Neoplastic • Malignant: – – – – Carcinomas of the tonsil, tongue base, thyroid Lymphoma Sarcoma Metastatic disease: squamous cell carcinoma of the aerodigestive tract, cutaneous malignancies • Usually benign – Salivary gland neoplasm – Paraganglioma • Benign – – – – Schwannoma Lipoma Benign thyroid masses – cysts, nodules, adenomas Benign skin cysts, e.g. epidermoid inclusion cyst 10 Molly Kantor, 2010 Gillian Lieberman, MD Neck Anatomy Review Molly Kantor, 2010 Gillian Lieberman, MD Anatomy: Triangles of the Neck UpToDate: Anatomical regions of the neck http://www.uptodate.com/online/content/image.do?imageKey=PC%2F21883 12 Molly Kantor, 2010 Gillian Lieberman, MD Anatomy: Vasculature of the Neck Adapted from: http://accweb.itr.maryville.edu/myu/Bio301Summer/301on9.html 13 Molly Kantor, 2010 Gillian Lieberman, MD Anatomy: Carotid Bifurcation Netter’s Clinical Anatomy, 2nd ed. Figure 8‐58 Major Arteries of the Head and Neck 14 Molly Kantor, 2010 Gillian Lieberman, MD Anatomy: External Carotid Artery Netter’s Clinical Anatomy, 2nd ed. Figure 8‐48 External Carotid Artery and Its Branches 15 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Images Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI Mass PACS, BIDMC Coronal MRI, T1, post‐contrast, subtraction image 17 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Close Up Mass Large (2.5cm x 2.0 cm x 3.6 cm), heterogeneous, enhancing mass at the level of the carotid bifurcation PACS, BIDMC Coronal MRI, T1, post‐contrast, subtraction image 18 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Arterial Structures R and L internal carotid artery R external carotid artery Mass R common carotid artery PACS, BIDMC L common carotid artery Coronal MRI, T1, post‐contrast, subtraction image 19 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Venous Structures Mass R internal jugular vein L internal jugular vein R external jugular vein L external jugular vein R subclavian vein PACS, BIDMC L subclavian vein Coronal MRI, T1, post‐contrast, subtraction image 20 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Glandular Structures R parotid gland L parotid gland Mass Thyroid gland PACS, BIDMC Coronal MRI, T1, post‐contrast, subtraction image 21 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Splaying Carotids Mass The mass splays the external and internal carotid arteries PACS, BIDMC Axial MRI, T1, without contrast 22 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Splaying Carotids (arteries labeled) R external carotid artery L external carotid artery R internal carotid artery Mass L internal carotid artery R vertebral artery L vertebral artery Comparison of the right and left shows the mass splaying the left external and internal carotids PACS, BIDMC Axial MRI, T1, without contrast 23 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Splaying Carotids (labeled) L submandibular gland R submandibular gland Mass Sternocleidomastoid muscle Vertebral body Spinal cord PACS, BIDMC Axial MRI, T1, without contrast 24 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Common Carotid level Mass PACS, BIDMC Axial MRI, T1, without contrast 25 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Common Carotid level (labeled) Pharynx L submandibular gland L common carotid artery R submandibular gland Mass R common carotid artery Sternocleidomastoid muscle Vertebral body Spinal cord PACS, BIDMC Axial MRI, T1, without contrast 26 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on MRI: Comparison of T1 and T2 The mass is brighter on T2 than T1, and enhances, consistent with a tumor PACS, BIDMC Axial MRI, T1 and T2 images, without contrast 27 Molly Kantor, 2010 Gillian Lieberman, MD Refining the Differential based on the patient’s imaging and H&P Molly Kantor, 2010 Gillian Lieberman, MD DDx for Our Patient’s tumor based on MRI • Soft tissue mass, not cystic or made of fat – Rule out branchial cleft cyst, thyroglossal duct cyst, dermoid cyst, thymic cyst, thyroid cyst, teratoma, lipoma, skin lesions • The mass enhanced – Indicates hypervascular – Rules out cysts (above), ranula, teratoma, lymphadenopathy • Located at carotid bifurcation and splays the carotids – Rule out salivary gland tumor, benign thyroid mass, Schwannoma, tonsilar tumor, aerodigestive tract tumor, tongue tumor, skin lesions – Great location for a paraganglioma 29 Molly Kantor, 2010 Gillian Lieberman, MD … combined with the H & P • Mass has been present for at least 12 years – Not aggressive, so unlikely to be metastatic disease (SCC of aerodigestive tract, skin cancer, thyroid) or lymphoma • Moves side to side but not up and down This is a Paraganglioma! 30 Molly Kantor, 2010 Gillian Lieberman, MD Overview of Carotid Body Tumors Molly Kantor, 2010 Gillian Lieberman, MD Carotid Body Tumors • Paragangliomas are rare tumors of neural crest cells associated with extraadrenal autonomic ganglia – Carotid body tumors are the most common type • Tumor characteristics – – – – Rarely (1‐3%) functioning (secrete catecholamines) Grow indolently, ~1mm/year Rarely metastatic 10%‐50% have a family history • When hereditary, often present earlier (< 35) and in multiple • Diagnosis: H&P + imaging! – Often pulsatile, often a bruit can be heard, and classically mobile in a side to side but not vertical direction • Treatment options: – Surgical excision (often with pre‐op embolization of blood supply) – Radiation 32 Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: Carotid Body Tumor • • • • • CT MRI Doppler Ultrasound Angiography Nuclear medicine: Octreotide Scintigraphy Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: CT • CT – Homogenous mass with intense enhancement with IV contrast – Typically displaces the carotid bifurcation • • • • MRI Doppler Ultrasound Angiography Nuclear medicine: Octreotide Scintigraphy Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: MRI • CT • MRI – Often is intermediate signal of T2 and classically “salt and pepper” on T2 – Smooth contours and located at the carotid bifurcation – Assess size and extent of tumor, course of vessels, and relationship to surrounding structures (better than CT) • Doppler Ultrasound • Angiography • Nuclear medicine: Octreotide Scintigraphy Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: Doppler Ultrasound • CT • MRI • Doppler Ultrasound – Used to visualize the tumor and the mass effect on the surrounding structures – Often used in conjunction with CT or MRI • Angiography • Nuclear medicine: Octreotide Scintigraphy Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: Angiography • • • • CT MRI Doppler Ultrasound Angiography – Direct visualization of the vessels and tumor blood supply – Can be both therapeutic and help in surgical planning (e.g. adequacy of intracranial circulation if sacrifice of the internal carotid is necessary) • Nuclear medicine: Octreotide Scintigraphy Molly Kantor, 2010 Gillian Lieberman, MD Menu of Tests: Nuclear Medicine • • • • • CT MRI Doppler Ultrasound Angiography Nuclear medicine: Octreotide Scintigraphy – Neuroendocrine tumors have a high density of somatostatin type 2 receptors – Can be used to preoperatively identify unexpected sites of disease Molly Kantor, 2010 Gillian Lieberman, MD Carotid Body Tumor Menu of Tests: Summary • CT – Homogenous mass with intense enhancement with IV contrast – Typically displaces the carotid bifurcation • MRI – Often is intermediate signal of T2 and classically “salt and pepper” on T2 – Smooth contours and located at the carotid bifurcation – Assess size and extent of tumor, course of vessels, and relationship to surrounding structures (better than CT) • Doppler Ultrasound – Used to visualize the tumor and the mass effect on the surrounding structures – Often used in conjunction with CT or MRI • Angiography – Direct visualization of the vessels and tumor blood supply – Can be both therapeutic and help in surgical planning (e.g. adequacy of intracranial circulation if sacrifice of the internal carotid is necessary) • Nuclear medicine: Octreotide Scintigraphy – Neuroendocrine tumors have a high density of somatostatin type 2 receptors – Can be used to preoperatively identify unexpected sites of disease Molly Kantor, 2010 Gillian Lieberman, MD Comparison Patient 1: Carotid Body Tumor on CT L external carotid artery R external carotid artery Mass R internal carotid artery L internal carotid artery R vertebral artery A mass at the carotid bifurcation on the left PACS, BIDMC Axial CT with contrast 41 Molly Kantor, 2010 Gillian Lieberman, MD Comparison Patient 1: Carotid Body Tumor on MRI L external carotid artery Mass L internal carotid artery As with our previous patient, the mass is bright on T2 and splays the carotids PACS, BIDMC Axial MRI, T1 and T2 (fat sat), without contrast 42 Molly Kantor, 2010 Gillian Lieberman, MD Comparison Patient 2: Carotid Body Tumor on Ultrasound Mass Densely echogenic mass in the left neck with some areas of altered echogenecity Ultrasound, without contrast Chiu, G. A., A. I. Edwards, S. Akhtar, J. C. Hill, and I. M. Hanson. Carotid body paraganglioma manifesting as a malignant solitary mass on imaging: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 109 (4):e54‐8. 43 Molly Kantor, 2010 Gillian Lieberman, MD Comparison Patient 3: Carotid Body Tumor on Nuclear Medicine Lesion 111‐indium labeled octreotide scintigraphy Milardovic, R., E. P. Corssmit, and M. Stokkel. Value of 123I‐MIBG Scintigraphy in Paraganglioma. Neuroendocrinology 91 (1):94‐100. 44 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Interventional Radiology Images Molly Kantor, 2010 Gillian Lieberman, MD The Plan for H.K.’s Tumor • Embolization followed by surgical excision the next day – Embolization (by interventional radiology) to cut off the tumor’s blood supply – Surgery (by an ENT and a vascular surgeon) because there is a low chance (3%) of being malignant, the tumor is likely to grow over time and easier to remove when smaller 46 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Carotid Body Tumor on Angiogram Vascular blush seen at the carotid bifurcation, and displacement of the internal and external carotid arteries is also evident PACS, BIDMC Angiogram, left common carotid artery 47 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Angiogram: Anatomy (posterior view) Ascending pharyngeal artery Internal carotid artery External carotid artery Superior thyroid artery Common carotid artery PACS, BIDMC Angiogram, left common carotid artery 48 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Angiogram: Anatomy (lateral view) Superficial temporal artery Maxillary artery Occipital artery Ascending pharyngeal artery Facial artery Lingual artery Superior thyroid artery PACS, BIDMC Angiogram, left external carotid artery 49 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Angiogram: Inserting Microcatheter Injecting contrast through a microcatheter in a branch of the ascending pharyngeal artery causes the tumor to blush PACS, BIDMC Angiogram, left ascending pharyngeal artery 50 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient‘s Angiogram: Embolization Two of three feeding branches of the ascending pharyngeal artery were embolized with Embospheres and one coil PACS, BIDMC Angiogram, left ascending pharyngeal artery 51 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient’s Angiogram: Pre‐ and Post‐ Embolization Pre‐Embolization Post‐Embolization Tumor blushes only from inferior branches, not from ascending pharyngeal artery PACS, BIDMC Angiogram, left common carotid artery 52 Molly Kantor, 2010 Gillian Lieberman, MD Our Patient: Further Follow‐Up • H.K. had an successful surgery with preservation of both the internal and external carotid arteries and a post‐operative course complicated only by minor nerve weakness 53 Molly Kantor, 2010 Gillian Lieberman, MD Acknowledgements • • • • • • Gillian Lieberman, MD Suresh Reddy, MD Sachin Pandey, MD Diane DiNoble, NP Graham Frankel Larry Barbaras 54 Molly Kantor, 2010 Gillian Lieberman, MD References • • • • • • • Chiu, G. A., A. I. Edwards, S. Akhtar, J. C. Hill, and I. M. Hanson. Carotid body paraganglioma manifesting as a malignant solitary mass on imaging: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 109 (4):e54‐8. Emerick, Kevin, and Derrick Lin. Differential diagnosis of a neck mass May 2010 [cited August 18, 2010]. Available from http://www.uptodate.com/online/content/topic.do?topicKey=prim_ent/8791&selectedTitle= 2~6&source=search_result#H1. Hansen, John T. 2010. Netter’s Clinical Anatomy. 2nd ed. Philadelphia: Saunders Elsevier. Lin, Derrick, and Daniel G Deschler. UpToDate: Anatomical regions of the neck. 2010 [cited August 18, 2010]. Available from http://www.uptodate.com/online/content/image.do?imageKey=PC%2F21883. Milardovic, R., E. P. Corssmit, and M. Stokkel. Value of 123I‐MIBG Scintigraphy in Paraganglioma. Neuroendocrinology 91 (1):94‐100. Thyroid Gland: Anterior View [cited August 18, 2010]. Available from http://accweb.itr.maryville.edu/myu/Bio301Summer/301on9.html. van den Berg, R. 2005. Imaging and management of head and neck paragangliomas. Eur Radiol 15 (7):1310‐8. 55
