J Rhinol.  2020 May;27(1):41-45. 10.18787/jr.2019.00307.

Endoscopic Resection of Functional Paraganglioma of the Pterygopalatine Fossa: A Case Report

• Choi JH
• Lee E
• Hong SD

• KMID: 2502793
• DOI: http://doi.org/10.18787/jr.2019.00307

Abstract

Functional paraganglioma is a rare tumor that secretes the catecholamine which is able to cause secondary hypertension. Surgical resection is the only curative treatment modality for this tumor, which can correct the secondary hypertension. The pterygopalatine fossa is a small retromaxillary space which contains a neurovascular bundle that crosses the middle cranial fossa to the nasal cavity, nasal septum, and oral cavity. To our knowledge, two cases have been reported for functional paraganglioma involving the pterygopalatine fossa removed by surgery. We present a patient who had a catecholamine-secreting paraganglioma in the pterygopalatine fossa, which is the first case successfully resected with an endoscopic approach.

Keyword

Functional paraganglioma; Pterygopalatine fossa; Endoscopic surgery

Figure

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  Fig. 1. A: Coronal view of contrast CT scan represents about 2-cm sized round enhancing mass in the right pterygopalatine fossa. The tumor causes widening of adjacent bony structure with extension to inferior orbital fissure (white arrow). B: Axial view of contrast CT represents that the tumor was adjacent to the cavernous sinus superiorly (black arrow) and the sphenoid lateral recess posteriorly. C, D: Immediate post-operative CT scan demonstrated no definite residual mass in pterygopalatine fossa.

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  Fig. 2. A: In contrast-enhanced T1-weighted MRI scan, tumor (T) showed high signal intensity comparing to muscle. B: In T2-weighted MRI scan, the tumor (T) showed heterogeneous signal intensity and there are flow voids (white arrow heads) within the tumor caused by rapid vascular flow, which is a typical radiological finding in paraganglioma called “salt-and-pepper pattern.” C: Axial contrast-enhanced T1-weighted MRI scan. Though cavernous sinus invasion was suspected in CT scan, no definite cavernous sinus invasion was seen in MRI scan.

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  Fig. 3. Histopathologic examinations. The tumor shows typical Zellballen cluster formation (star) with prominent vascular network (black arrows) separating clusters of endocrine cells (H & E, ×100). In Zellballen cluster, the endocrine cells with round nuclei containing abundant granular eosinophilic cytoplasm are observed.

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