Presentation
Left neck swelling with hoarseness of voice and aspiration when taking liquids.
Patient Data
Well-defined lobulated heterogeneously enhancing mass is seen at the left cervical region within the post-styloid parapharyngeal space and carotid space. It displaces the left parapharyngeal fat anteriorly. It encases the common, internal, and external carotids with splaying of the internal and external carotid arteries. The left internal jugular vein is compressed. There is no clear plane with the left prevertebral muscles. No bony erosion.
The left vallecula was effaced. The left aryepiglottic fold thickened.
Multiple bilateral subcentimeter cervical nodes.
Large well-defined lobulated mass (isointense to muscle on T1W and hyperintense to muscle on T2W) within the left post-styloid parapharyngeal space and carotid spaces, from the level of the jugular foramen superiorly to the level of C5/C6 inferiorly as seen on CT. It is heterogeneously enhanced with the central non-enhancing cystic component within, likely to represent central necrosis. No T1W hyperintensities or flow voids within suggest a “salt and pepper” appearance. No blooming artifacts were seen on gradient echo images to suggest intratumoral hemorrhage. It is causing a mass effect on the adjacent structures. It abuts the left prevertebral muscles; however, no abnormal signal or enhancement is seen within. This mass splays the internal carotid (ICA) and external carotid arteries (ECA) as well as displaces them anterolaterally. There is a complete (360-degree) encasement of the left common carotid (CCA), ICA, and ECA; however, these vessels still remain patent. On MRA, no significantly enlarged feeding vessels were demonstrated. The left internal jugular vein (IJV) is compressed. No intracranial extension.
Mild hyperintense signal is seen involving the left side of the tongue on T2W and STIR, with a dorsal bulge causing effacement of the left vallecula. The left aryepiglottic fold is mildly thickened with medial deviation.
Multiple subcentimeter-enhancing cervical nodes bilaterally, largest on the right.
The visualized brain parenchyma is unremarkable.
Case Discussion
Due to the location of the mass and the presence of vascular encasement with splaying of the ICA and ECA, this mass was initially thought to represent a large cervical paraganglioma, either arising from the carotid body and/or vagus nerve. However, in view of atypical features (no intratumoral hemorrhage and lack of flow voids), a schwannoma could not be totally excluded.
She also had imaging features in keeping with left vocal cord palsy (confirmed on scope) and left hypoglossal nerve palsy, which correlates with her presenting symptoms.
The patient went on to have debulking surgery, and histopathology results showed Ewing sarcoma.
Extraskeletal Ewing sarcoma is rare compared to Ewing sarcoma of the bone. They tend to be aggressive, with a high rate of local recurrence and distant metastasis. In this patient, a post-operative PET scan showed no evidence of distant metastases.
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