Paragangliomas are rare neuroendocrine tumors originating from the paraganglia. They are mostly benign, slow-growing, and non-secretory. This report describes a rare case of a catecholamine-secreting paraganglioma located in the pterygopalatine fossa. A 45-year-old man presented with severe hypertension, occipital headache, and facial pain. Imaging revealed a large pterygopalatine mass, initially misdiagnosed as chondrosarcoma. Gamma knife stereotactic radiosurgery was performed, followed by surgical resection via a maxillary swing approach. Histological examination confirmed the zellballen pattern typical of paraganglioma. Post-surgery, the patient's blood pressure normalized immediately and subsequently. This case highlights the diagnostic challenges of head and neck paragangliomas in atypical locations and underscores the importance of surgical intervention for catecholamine-secreting tumors. Future studies should focus on refining diagnostic protocols and comparing the efficacy of radiosurgery versus surgical resection for these tumors.
Keywords: catecholamine-secreting paraganglioma; extra-adrenal paragangliomas; paraganglioma; pterygopalatine; zellballen pattern.