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Pictorial review of the imaging anatomy and common pathology of the parapharyngeal space. Poster No.: C-1092 Congress: ECR 2014 Type: Educational Exhibit Authors: B. S. Purohit , R. Hermans , K. Op de beeck ; SINGAPORE/SG, 1 2 2 1 2 Leuven/BE Keywords: Infection, Cysts, Cancer, Diagnostic procedure, MR, CT, Head and neck DOI: 10.1594/ecr2014/C-1092 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myESR.org Page 1 of 32 Learning objectives This educational exhibit aims to acquaint the reader with: 1. The imaging anatomy of the parapharyngeal space (PPS). 2. Approach to imaging issues of PPS lesions. 3. The displacement patterns of PPS fat that are essential for accurate diagnosis and appropriate treatment of pathology arising in this region. 3. Common differentials of true PPS lesions and their imaging features. Background • The PPS is a central fat-filled space in the deep face and suprahyoid neck extending from the skull base to the level of the hyoid bone. Since it largely contains fat, it is always well visualised on CT and MR imaging of the head and neck. • The PPS is bordered by the pharyngeal mucosal space (PMS) medially, masticator space (MS) laterally, parotid space (PS) posterolaterally, and the retropharyngeal space (RPS) posteromedially. The PPS is further subdivided into the prestyloid and retrostyloid compartments. • The presence of fat in the PPS allows the radiologist to identify displacement patterns of the PPS and to use a logical algorithm to identify the true space of origin of tumours in this region. • Although tumours of the PPS are rare, their diagnosis and treatment options may be challenging. Differentiation of a prestyloid lesion from a retrostyloid lesion is crucial for narrowing the differential diagnosis. • The most common lesion in the PPS are tumours arising from the deep parotid lobe, extending in the prestyloid compartment of the PPS. Neurogenic tumours and paragangliomas are the commonest primary lesions of the retrostyloid PPS. Primary prestyloid PPS lesions are rare, nd however, lipomas, atypical 2 branchial cleft cyst (BCC) and ectopic salivary gland tumours may be seen. Page 2 of 32 Findings and procedure details Anatomy of the PPS: The PPS is an inverted cone-shaped space that extends from the level of the skull base upto the hyoid bone on either side of the pharynx. The anatomic boundaries of the PPS are as follows: • Superior: The temporal bone lateral to the attachment of the pharyngobasilar fascia and medial to the foramen ovale and foramen spinosum. None of the skull base foramina open within the boundaries of the PPS (Fig. 1). • Inferior: The greater cornu of hyoid bone and the posterior belly of the digastric muscle. The PPS is continuous with the posterior aspect of the submandibular space (SMS) at this level (Fig. 1). • Medial: The buccopharyngeal fascia which covers the pharyngobasilar fascia and constrictor muscles. The PMS lies medial to the PPS and the RPS lies posteromedial to the PPS (Fig. 2). • Lateral: The fascia overlying the pterygoid muscles (MS) and the sphenomandibular ligament. The PPS communicates laterally with the PS through the stylomandibular tunnel (Fig. 1, 2). • Anterior: The pterygomandibular raphae which extends from the medial pterygoid plate to the mylohyoid line on the lingual surface of the mandible. • Posterior: The tensor-vascular-styloid fascia (TVS) overlying the tensor veli palatini (TVP) muscle extends from the medial pterygoid plate to the styloid process (Fig. 2). It subdivides the PPS into the prestyloid and retrostyloid compartments. Some authors describe the retrostyloid PPS separately as the carotid space [1-3]. Contents of the PPS: • Prestyloid PPS: Contains fat, the internal maxillary and ascending pharyngeal artery, pterygoid venous plexus and may contain ectopic salivary gland tissue. • Retrostyloid PPS: Contains the internal carotid artery (ICA), internal jugular vein (IJV), cranial nerves IX-X-XI-XII and the sympathetic plexus. Lymph Page 3 of 32 nodes of the deep cervical chain are found only below the level of the posterior belly of the digastric muscle [1-5]. Fig. 1 References: Bela Purohit Fig.1: Graphic representation of the coronal spatial anatomy and craniocaudal extent of the PPS. SB-Skull base, SZMS-Suprazygomatic masticator space, MS-Masticator space, Page 4 of 32 PMS-Pharyngeal mucosal space, PS-Parotid space, SMS-Submandibular space, SLSSublingual space, OC-Oral cavity. Fig. 2 References: Bela Purohit Fig. 2: Graphic representation of the axial spatial anatomy of the suprahyoid neck and the PPS. BS-Buccal space, RPS-Retropharyngeal space. Page 5 of 32 Radiological anatomy of the PPS: • The fat-filled PPS is well appreciated on both axial and coronal CT and MR images. The PPS appears hypodense with fat attenuation on CT images and hyperintense on T1W MR images. In normal circumstances, the PPS should look symmetrical on both sides (Fig. 3, 4) [1-3]. • MRI is often preferred over CT in the evaluation of PPS pathology because of its superior contrast resolution [1]. • In the axial plane, the prestyloid PPS is seen as a triangular fat-fillled space widest at the level of the soft palate (Fig. 3). The pharyngobasilar may be seen on MRI as a hypointense line outlining the pharyngeal mucosa [1]. • The TVS cannot be routinely identified on MRI. Its course can be traced by drawing an imaginary line from the TVP muscle upto the styloid process [1]. • The lateral fascial border of the PPS is not identifiable on MRI. Its cranial attachment lies just medial to the foramen ovale and the exiting mandibular nerve (Fig. 4) [1]. • The ICA and IJV appear as flow voids on T1W and T2W MR images, just medial to the syloid process (Fig. 3) [1]. Page 6 of 32 Fig. 3 References: Department of Radiology, University Hospitals, Leuven Fig. 3: Axial T1W MR image showing spatial anatomy of the PPS. The central fat-filled PPS (asterisk) is surrounded by the PMS medially (solid black arrow), MS anterolaterally (arrowhead) and the PS posterolaterally (thin black arrow). The ICA in the retrostyloid PPS is seen as a flow void (thin white arrow). Page 7 of 32 Fig. 4 References: Department of Radiology, University Hospitals, Leuven Fig. 4: Cor T1W MR image showing the craniocaudal extent of the PPS. Superiorly, the PPS (asterisk) extends to the skull base, just medial to the opening of the foramen ovale (white arrow). Inferiorly, the PPS merges with the SMS (thin black arrow). The PPS is related to the PMS medially (solid black arrow) and the MS laterally (arrowhead). Page 8 of 32 Clinical and diagnostic approach to PPS lesions: • Since the PPS has relatively few internal structures, primary lesions of the PPS are rare. The PPS is more commonly displaced or infiltrated by lesions arising in the surrounding spaces [1-4]. • Small tumours of the PPS may be incidental findings whereas large tumours may cause dysphagia, ear fullness, jaw pain and cranial nerve palsy. The deep location of these tumours may hinder their clinical evaluation and may lead to delay in diagnosis. Large lesions may cause internal bulging of the naso/oro-pharyngeal wall or swelling in the parotid region or in the submandibular region [1, 5]. • The clinican as well as the radiologist must be aware of the fact that the cranio-caudal extent of the PPS can make it function as an 'elevator shaft' through which infection or tumour from adjacent spaces can spread to the skull base [3]. • CT and MR are the cornerstones in the initial evaluation of PPS lesions [5]. The main goals of imaging are: 1. 2. To identify the true space of origin of a PPS lesion. To identify if a primary PPS lesion lies within the prestyloid or retrostyloid compartment. To find imaging clues leading to the likely differential diagnosis. To charcterise the lesion as aggressive or non-aggressive. If the lesion appears aggressive, to identify adjacent spaces of spread, intracranial extension and perineural spread. To assess for vascularity of lesions, especially, those which may need biopsy. To provide a road map for image-guided biopsy, if needed. 3. 4. 5. 6. 7. Displacement patterns of PPS fat: The PPS is surrounded by four key spaces (PMS, RPS, MS, PS) of the suprahyoid neck (SHN). The direction of displacement of the PPS by a mass lesion arising from a surrounding space can be a key finding in determining its space of origin (Fig. 5) [1-5]. PS mass lesion pushes PPS anteromedially. MS mass lesion pushes PPS posteromedially. PMS mass lesion pushes PPS laterally. Page 9 of 32 Lateral RPS mass pushes PPS anterolaterally. Retrostyloid PPS mass pushes prestyloid PPS anteriorly. Fig. 5 References: Bela Purohit Fig. 5: Graphic representation of the vectors of displacement of the PPS fat (asterisk) by surrounding space lesions. Some examples of displacement patterns of PPS fat: Page 10 of 32 Fig. 6 References: Department of Radiology, University Hospitals, Leuven Fig. 6: Axial T2W MR image in a patient with pleomorphic adenoma arising from the deep lobe of the left parotid gland. The black arrow indicates the vector of displacement of the left-sided PPS fat by the mass. Normal right PPS is shown for comparison (white arrow). Page 11 of 32 Fig. 7 References: Department of Radiology, University Hospitals, Leuven Fig. 7: Axial T2W MR image in a patient with sarcoma of the right MS (asterisk). The black arrow indicates the vector of displacement of the right-sided PPS fat by the mass. Normal left PPS is shown for comparison (white arrow). Page 12 of 32 Fig. 8 References: Diagnostic Imaging, National University Hospital, National University Hospital - SINGAPORE/SG Fig. 8: Axial contrast-enhanced T1W MR image in a patient with right-sided nasopharyngeal carcinoma. The black arrow indicates the vector of displacement of the right-sided PPS fat by the mass. Normal left PPS is shown for comparison (white arrow). Page 13 of 32 Fig. 9 References: Diagnostic Imaging, National University Hospital, National University Hospital - SINGAPORE/SG Fig. 9: Axial T2 fat-saturated MR image in a patient with right-sided oropharyngeal carcinoma (asterisk) and an enlarged metastatic right retropharyngeal node. The black arrow indicates the vector of displacement of the right-sided PPS fat by the retropharyngeal node. Normal left PPS is shown for comparison (white arrow). Page 14 of 32 Fig. 10 References: Department of Radiology, University Hospitals, Leuven Fig. 10: Axial T2W MR image in a patient with a right vagal schwannoma. The black arrow indicates the vector of displacement of the right-sided prestyloid PPS fat by the mass. The retrostyloid mass displaces the right styloid process anteriorly (arrowhead). The ICA is displaced anterolaterally. Normal left PPS is shown for comparison (white arrow). Imaging findings in PPS lesions: Page 15 of 32 We retrospectively reviewed PPS lesions detected on CT and MR at the University Hospitals, Leuven from 2002-2013. This pictorial review illustrates common examples of pathologies arising in the PPS. Prestyloid PPS lesions: 1. Salivary gland tumours: • These constitute the majority of neoplasms in the prestyloid PPS. These tumours arise from ectopic salivary gland rests in the prestyloid PPS. However, more commonly they extend from the deep lobe of the parotid and extend exophytically into the PPS (Fig. 6). • Pleomorphic adenoma/benign mixed tumour is the commonest salivary gland tumour. Malignant tumour like mucoepidermoid carcinoma and adenoid cystic carcinoma are uncommon. • A tumour is considered to be primary to the PPS if it is completely surrounded by PPS fat. A deep parotid lobe tumour often appears dumbellshaped. It is connected to the deep lobe of the parotid gland and is seen to widen the stylomandibular tunnel. It pushes the PPS fat anteromedially (Fig. 6). • Pleomorphic adenoma usually appears as a well-circumscribed, heterogeneous mass on CT. On MRI, it appears hypointense on T1W images with marked hyperintensity on T2W MR images. Heterogeneous post contrast enhancement may be seen (Fig. 6, 11, 12, 13). Haemorrhage, necrosis and calcifications may occur (Fig. 11). • Complete surgical removal is the treatment of choice for a pleomorphic adenoma in view of high tendency for recurrence and the less common but likely possibility of malignant conversion (malignant mixed tumour) [1-4, 6]. Page 16 of 32 Fig. 11 References: Department of Radiology, University Hospitals, Leuven Fig. 11: Axial T1W MR image in a patient with a pleomorphic adenoma arising from minor salivary glands in the right PPS. The tumour (asterisk) is isointense to muscle with a hyperintense focus within, likely due to haemorrhage (arrow). Page 17 of 32 Fig. 12 References: Department of Radiology, University Hospitals, Leuven Fig. 12: Axial T2W MR image in the same patient as in Fig. 11. The tumour (asterisk) shows heterogeneously hyperintense signal. There is no connection with the deep lobe of the right parotid gland (arrowhead). Page 18 of 32 Fig. 13 References: Department of Radiology, University Hospitals, Leuven Fig. 13: Axial contrast-enhanced T1W MR image in the same patient as in Fig. 11, 12. The tumour (asterisk) shows heterogeneous post-contrast enhancement. There is a well maintained fat-plane separating the tumour from the deep lobe of the right parotid gland (arrows). 2. Lipomas: Page 19 of 32 • Primary lipomas of the PPS are rare and liposarcomas rarer still. Some of lipomas may arise from the parotid space and extend into the PPS. • Lipomas usually appear as well-circumscribed lesions within the prestyloid PPS. Large lipomas may cause internal bulging of the lateral pharyngeal wall. They show fat attenuation on CT and hyperintense signal on TIW MR images (Fig. 14). Focal enhancement within a lipoma is suspicious for malignancy ie liposarcoma [1-3, 7]. Fig. 14 References: Department of Radiology, University Hospitals, Leuven Page 20 of 32 Fig. 14: Axial T1W MR image in a patient with a lipoblastoma in the left PPS. The lipoblastoma (asterisk) appears well-circumscribed and causes inward bulging of the left oropharyngeal wall. It appears typically uniformly hyperintense. 3. Branchial cleft cyst: • The prestyloid PPS is a likely but rare location for an atypical 2nd branchial cleft cyst (BCC). • An uncomplicated BCC usually appears well circumscribed with thin walls and fluid contents on CT and MRI (Fig. 15). Thickened irregular walls may be due to infection. • In the absence of infection, an atypical cystic lesion in the PPS may be suspicious for metastatic necrotic adenopathy from papillary carcinoma of the thyroid or squamous cell carcinoma of the pharyngeal mucosal space [1, 3, 4, 8]. Page 21 of 32 Fig. 15 References: Department of Radiology, University Hospitals, Leuven/ Reference 1. Fig. 15: Axial T2W MR image in a patient with an atypical 2nd BCC in the left PPS. The uncomplicated BCC (asterisk) is well circumscribed with thin walls and shows fluid intensity. It causes mild inward displacement of the left lateral oropharyngeal wall. Retrostyloid PPS lesions: 1. Neurogenic tumours: Page 22 of 32 • Neurogenic tumours (schwannomas and neurofibromas) are the most common primary tumours of the retrostyloid PPS. Retrostyloid tumours tyically displace the styloid process anteriorly, ICA anteromedially and the prestyloid PPS anteriorly. • Schwannomas commonly arise from the vagus nerve. CT shows a hypodense fusiform, well-circumscribed mass with mild homogeneous enhancement (Fig. 16). Schwannomas often appear hyperintense on T2W MR images (Fig. 10). Heterogeneous signal on T1W and T2W images may be due to cystic degeneration or intralesional haemorrhage. Schwannomas are relatively hypovascular lesions (which often helps to differentiate them from paragangliomas). Malignant transformation is rare [1-3, 5, 9]. • The imaging features of a solitary neurofibroma overlap those of a schwannoma. Neurofibromas tend to be more hypodense on CT and less enhanced as compared to schwannomas on post contrast CT and MR images. Neurofibromas may show the classical 'target-sign' on axial T2W images [5, 9]. Page 23 of 32 Fig. 16 References: Department of Radiology, University Hospitals, Leuven Fig. 16: Axial contrast-enhanced CT in a patient with left vagal schwannoma. The schwannoma (asterisk) appears homogeneously hypodense with poor contrast enhancement. The left prestyloid PPS is displaced anteriorly (black arrow), the left ICA is displaced anteromedially (arrowhead) and the left IJV (white arrow) is compressed and displaced posterolaterally. Page 24 of 32 2. Paragangliomas: • Paragangliomas/ glomus tumours in the head and neck arise from chemoreceptor cells located at three sites; at the nodose ganglion of the vagus at the skull base (vagal paraganglioma), at the carotid bifurcation (carotid paraganglioma/ carotid body tumour) and at the level of the jugular foramen (jugular paraganglioma). • Glomus tumours are typically hypervascular and show marked post contrast enhancement on both CT and MRI (Fig. 17). A 'salt-pepper' appearance is common on T1W MR images due to focal areas of haemorrhage and tortuous vessels causing flow voids. Carotid body tumours typically splay the internal and external carotid artery (ECA) away from each other (Fig. 18). • Conventional angiography helps to confirm the diagnosis and may be required for pre-operative embolisation [1-3, 5, 10]. Page 25 of 32 Fig. 17 References: Department of Radiology, University Hospitals, Leuven Fig. 17: Axial contrast-enhanced CT image in a patient with left carotid body tumour. The well-circumscribed mass (asterisk) is located at the left carotid bifurcation and marked, homogeneous post contrast enhancement. Page 26 of 32 Fig. 18 References: Department of Radiology, University Hospitals, Leuven Fig. 18: Coronal contrast-enhanced CT image in the same patient as in Fig. 17. The left carotid body tumour (asterisk) causes typical splaying of the left ICA (black arrow) and the left ECA (white arrow). Page 27 of 32 Secondary lesions of the PPS: • The PPS is often secondarily involved by tumour and infection arising in the PMS, MS and PS. Agressive masticator space neoplasms like sarcomas and PMS neoplasms like squamous cell carcinomas and lymphomas readily infiltrate the PPS [1-4]. • Secondary involvement of the PPS is often seen in benign muticompartmental lesions like lymphangioma and hemangioma [1, 3]. • Parapharyngeal abscess is rare in the modern era of effective antibiotics. The most common scenario is post acute tonsillitis with extension of a peritonsillar abscess through the buccopharyngeal fascia into the PPS (Fig. 19). The exact extension of the abscess into the adjacent spaces must be described so as to guide effective drainage [1, 3]. Page 28 of 32 Fig. 19 References: Department of Radiology, University Hospitals, Leuven Fig. 19: Axial contrast-enhanced CT in a child with acute left tonsillitis leading to left peri-tonsillar abscess. A rim-enhancing centrally hypodense/liquefied abscess is seen extending into the left PPS (black arrow). Normal right PPS is shown for comparison (white arrow). Page 29 of 32 Conclusion Take home points: 1. A clear understanding of the spatial anatomy of the PPS is crucial for an accurate diagnostic approach towards pathology arising in this region. 2. Lesions arising from adjacent spaces displace the PPS fat in a particular way; in combination with the imaging characteristics of the mass, this information helps to narrow down the list of differential diagnosis. 3. When tumour or infection is detected in the PPS, the entire PPS from the skull base to the hyoid is imaged to ascertain the point of origin. 4. The commonest lesions in the prestyloid PPS are deep parotid lobe tumours, nd ectopic salivary gland tumours, lipomas and atypical 2 BCCs. Neurogenic tumours and paragangliomas are the commonest lesions of the retrostyloid PPS. Personal information Dr. Bela Purohit, MD, DNB, FRCR. Singapore. E-mail: [email protected] Prof. Dr. Robert Hermans, MD, Ph.D. Leuven, Belgium. Email: [email protected] Dr. Katya Op de beeck, MD. Leuven, Belgium. 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