Azar Adel, Alkheder Ahmad, Sukkar Ghina, Dmirieh Ahmad, Alassaf Arige
Department of Otorhinolaryngology, Al Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria.
Faculty of Medicine, Syrian Private University, Damascus, Syria.
Ear Nose Throat J. 2024 Nov 30:1455613241304902. doi: 10.1177/01455613241304902.
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.
副神经节瘤是起源于副神经节的罕见神经内分泌肿瘤。它们大多为良性，生长缓慢，且无分泌功能。本报告描述了一例罕见的位于翼腭窝的分泌儿茶酚胺的副神经节瘤。一名45岁男性出现严重高血压、枕部头痛和面部疼痛。影像学检查发现翼腭窝有一个大肿块，最初被误诊为软骨肉瘤。进行了伽玛刀立体定向放射外科治疗，随后通过上颌骨摆动入路进行手术切除。组织学检查证实了副神经节瘤典型的巢状结构。术后，患者的血压立即并随后恢复正常。该病例凸显了非典型部位头颈部副神经节瘤的诊断挑战，并强调了对分泌儿茶酚胺肿瘤进行手术干预的重要性。未来的研究应集中于完善诊断方案，并比较这些肿瘤放射外科与手术切除的疗效。
