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Multimodality Imaging of Paragangliomas of the Head and Neck Jarett Thelen1,2* and Alok A

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Multimodality Imaging of Paragangliomas of the Head and Neck Jarett Thelen1,2* and Alok A Thelen and Bhatt Insights into Imaging (2019) 10:29 https://doi.org/10.1186/s13244-019-0701-2 Insights into Imaging PICTORIALREVIEW Open Access Multimodality imaging of paragangliomas of the head and neck Jarett Thelen1,2* and Alok A. Bhatt1 Abstract Paragangliomas arise from paraganglion cells which serve varied regulatory tasks in the body. When these cells demonstrate neoplasia within the head and neck, they typically present in characteristic locations including the carotid space, the jugular foramen, the middle ear, and along the course of the vagus nerve. The goal of this article is to review the relevant anatomy related to head and neck paragangliomas, as well as their typical imaging characteristics on cross- sectional imaging including CT, MR, ultrasound, and nuclear medicine studies. Additionally, differential considerations, as well as relevant involvement of adjacent structures which should be conveyed to the clinician, will be discussed. Keywords: Paraganglioma, Extra-adrenal,Glomustumor,Neoplasms,Head,Neck Key points tasks in the body, including chemoreceptor functions, which allow the body to respond to stresses such as hyp- Paragangliomas arise from paraganglion cells. These oxia, hypercapnia, and hypoglycemia. When these cells cells serve varied regulatory tasks in the body, demonstrate neoplasia within the head and neck, they typ- including chemoreceptor functions, which allow the ically present in characteristic locations, and with common body to respond to stresses such as hypoxia, symptomatology; thus, understanding these key features in hypercapnia, and hypoglycemia. conjunction with their imaging appearance can lead to ac- The imaging evaluation of head and neck curate and prompt diagnosis. The imaging evaluation of paragangliomas utilizes multiple cross-sectional im- head and neck paragangliomas utilizes multiple cross-sec- aging modalities including CT, MR, ultrasound, and tional imaging modalities to evaluate for extent of disease various nuclear medicine techniques. and to help guide surgical management by evaluating for Head and neck paragangliomas most common osseous and neurovascular involvement. In this article, we anatomic locations include the carotid space, jugular will review the expected anatomic locations of paraganglio- foramen, middle ear, and along the course of the mas and their general imaging appearance on computed vagus nerve. tomography (CT) and magnetic resonance (MR), as well as Paragangliomas are vascular tumors and CT and MR angiography. Additionally, we will discuss demonstrate avid enhancement on both CT and other differential considerations for lesions found in these MR. They also often exhibit a characteristic “salt locations. By understanding these fundamental concepts, and pepper” appearance on MR. the radiologist will be able to accurately differentiate a generally benign paraganglioma from a potentially more Introduction aggressive head and neck neoplasm. Paragangliomas, also known as glomus tumors, arise from Their most common anatomic locations include the paraganglion cells which form the basis of the extra-adrenal carotid space, jugular foramen, middle ear, and along the neuroendocrine system. These cells serve varied regulatory course of the vagus nerve (Fig. 1). The majority of head and neck paragangliomas are benign and only locally inva- sive, with only approximately 2–13% of paragangliomas demonstrating malignancy [1]. Malignancy is defined by * Correspondence: [email protected] 1University of Rochester Medical Center, Rochester, USA the anatomic presence of metastasis, as there are no 2Department of Imaging Sciences, University of Rochester Medical Center, current histopathologic diagnostic criteria which can 601 Elmwood Avenue, Rochester, NY 14607, USA © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. Thelen and Bhatt Insights into Imaging (2019) 10:29 Page 2 of 9 Fig. 1 Anatomic regions of head and neck paragangliomas accurately define malignant paragangliomas from their be- Imaging characteristics nign counterparts [2]. Malignant paragangliomas of the Ultrasound is typically utilized early in the diagnostic head and neck most commonly demonstrate regional process, often for initial evaluation of a palpable neck mass. nodal metastasis, and distant metastasis is extremely un- Sonographic evaluation of paragangliomas demonstrates a common [3]. well-defined, heterogeneously hypoechoic mass, with While the majority of cases are sporadic, approxi- marked internal vascularity on color Doppler (Fig. 2). Care- mately 40% are thought to be hereditary [4]. These fa- ful assessment of the displacement pattern of the internal milial tumors tend to occur earlier than their sporadic and external carotid arteries can raise the suspicion for ca- counterparts, with a peak prevalence of 30–35 years of rotid body and vagal paragangliomas and prompt cross-sec- age [5]. Additionally, familial paragangliomas are more tional imaging evaluation. commonly multicentric than sporadic tumors. Paragan- Magnetic resonance (MR) imaging is the most sensitive gliomas of the head and neck are more common in fe- imaging modality for evaluation of paragangliomas given its males, with this predilection most common in jugular superior soft tissue resolution, and it can readily characterize (3:1) and tympanic subtypes (6:1) [6]. Paragangliomas these tumors from other head and neck neoplasms. Com- are associated with multiple syndromes and genes and pared to the adjacent soft tissues, paragangliomas typically are commonly seen in patients with von Hippel-Lindau demonstrate hypointense signal on T1-weighted sequences (VHL), neurofibromatosis type I (NF I), and multiple and isointense to hyperintense signal on T2-weighted se- endocrine neoplasia type II (MEN II) [7, 8]. quences. Given the vascular nature of most paragangliomas, Thelen and Bhatt Insights into Imaging (2019) 10:29 Page 3 of 9 Various nuclear medicine imaging techniques may be used to evaluate for multicentric or metastatic disease, in- cluding I-131 and I-123 metaiodobenzylguanidine (MIBG), In 111 octreotide, and F-18 PET/CT [13, 14], demonstrat- ing focally increased uptake within the lesion (Fig. 3. Carotid body paragangliomas Carotid body paragangliomas are the most common para- gangliomas within the head and neck, accounting for approximately 60% of all cases. The majority of cases occur in older patients, ranging from 40 to 70 years of age with a median age of 57 [15]. As their name suggests, they arise from the carotid body, a grouping of chemoreceptors which reside between the internal and external carotid arteries at the bifurcation of the common carotid artery. The carotid Fig. 2 Color Doppler ultrasound image of the right cervical neck body provides regulatory function for both oxygenation, demonstrates a relatively hypoechoic, well-circumscribed mass with carbon dioxide, and pH within the blood. Carotid body tu- internal vascular flow (white arrows) in the right level II region. The mass mors classically present as slow-growing, painless swelling splays the external carotid artery superficially (arrowheads) in the lateral neck [16], often laterally mobile but fixed vertically (known as Fontaine’ssign).Asthetumorgrows internal flow voids are commonly seen, particularly on within the carotid space, it can compress the adjacent T2-weighted sequences. More rarely, areas of hyperintense nerves (most commonly the vagus nerve), resulting in intertumoral hemorrhage can be seen on both T1- and symptoms such as dysphagia, hoarseness, or Horner’ssyn- T2-weighted sequences. Interspersing of these, hypointense drome [17]. The majority of carotid body paragangliomas flow voids and hyperintense areas of hemorrhage results in are unilateral, though bilateral lesions are seen in approxi- acharacteristic“salt and pepper” appearance which is most mately 18% of patients [6]. Increased incidence (up to 10% apparent in tumors greater than 1 cm. Paragangliomas of patients) of sporadic carotid body paragangliomas is seen most commonly demonstrate avid, homogenous enhance- in patients living at high altitude, or in the setting of ment after administration of intravenous gadolinium chronic obstructive lung disease [18]. contrast agents. Both CT and MR are useful for initial evaluation of po- On computed tomography (CT), paragangliomas present tential carotid body paragangliomas and demonstrate an asawell-definedsofttissueattenuationmass.Theymost enhancing soft tissue attenuation mass situated within the commonly demonstrate homogenous, avid enhancement carotid space centered at the carotid bifurcation. Associ- after administration of intravenous contrast, though hetero- ated mass effect classically results in splaying of the in- geneity can occur in lesions with intratumoral thrombosis ternal and external carotid arteries, resulting in the or hemorrhage. Additionally, paragangliomas may cause characteristic “lyre sign” [16], rather than displacing them local erosion of adjacent bony structures, and CT imaging is together (Figs. 4, 5, 6,and7). Differential considerations excellent for evaluation of osseous involvement. In the case when encountering a mass
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