Malignant Vagal Paraganglioma

Abstract

Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.

Keywords: Carotid body tumor; Malignant paraganglioma; Paraganglioma.

Fig. 1

Left image coronal post-contrast CT demonstrating an avidly enhancing mass within the carotid space extending from slightly posterior to the carotid bifurcation superiorly to the skull base without intracranial extension. Top right image axial post-contrast CT showing left post-styloid parapharyngeal mass. Note the splaying of the internal and external carotid arteries and apparent obliteration of the internal jugular vein. Bottom right image T1 post-gadolinium MRI showing isointense, left parapharyngeal space mass with apparent flow voids

Fig. 2

Broad bands of dense connective tissue separate individual and groups of tumor cells which involved Vagus and Hypoglossal nerves

Fig. 3

A metastatic focus of tumor within a cervical lymph node that demonstrated the characteristic “Zellballen” histologic pattern separated by thin septae. Note the delicate vascularity

Fig. 4

Tumor involving a resected cervical lymph node appearing in a clustered, nesting pattern

Fig. 5

Previously inconspicuous sustentacular cells outlining the clustered “zellballen” are highlighted with s100 immunostain (left) and neurosecretory granules within the cytoplasm of chief cells are highlighted by a synaptophysin immunostain (right) within the main body of tumor