我们提出了一个源自上颌骨的原发性骨内副神经节瘤（PGL）的独特病例。 PGL 是一种神经分泌性肿瘤，起源于被认为源自神经嵴的细胞。一名 30 岁女性因右面部疼痛、肿胀以及心悸就诊。计算机断层扫描 (CT) 成像显示上颌前部有一个 3.3 × 3.1 × 2.3 cm 的肿块，生化结果显示血浆多巴胺、3-甲氧基酪胺和嗜铬粒蛋白 A 水平升高。活检证实 PGL，突触素、嗜铬粒蛋白 A 和 GATA-3 阳性表达。全身正电子发射断层扫描/计算机断层扫描 (PET/CT) 扫描显示对 18F-氟多巴 (18F-FDOPA)、68Ga-DOTA(0)-Tyr(3)-octreotate (68Ga-DOTATATE) 和 18F 具有亲和力-氟脱氧葡萄糖（18F-FDG）。未发现其他病变（原发性或转移性）。由于潜在的并发症和患者的偏好，选择质子束治疗而不是手术。放疗后，她的症状有所缓解，多巴胺水平下降，嗜铬粒蛋白 A 正常化，并且在 11 个月的随访成像中病变保持稳定。该病例凸显了原发性骨 PGL 的稀有性，并强调了综合诊断方法结合体格检查、生化检测、功能成像和组织病理学分析正确指导个性化治疗策略的重要性。此外，质子束治疗成为头颈副神经节瘤 (HNPGL) 的一种非常合适的治疗选择，可有效控制肿瘤，同时将并发症降至最低。由牛津大学出版社代表内分泌学会出版，2024 年。

We present a unique case of primary intraosseous paraganglioma (PGL) originating from maxillary bone. PGL is a neurosecretory neoplasm that arises from cells believed to originate from the neural crest. A 30-year-old woman presented with right facial pain and swelling, along with palpitations. Computed tomography (CT) imaging revealed a 3.3 × 3.1 × 2.3 cm mass in the anterior maxilla, and biochemical results showed elevated plasma dopamine, 3-methoxytyramine, and chromogranin A levels. Biopsy confirmed a PGL, with positive expression of synaptophysin, chromogranin A, and GATA-3. Whole-body positron emission tomography/computed tomography (PET/CT) scans showed avidity on 18F-fluorodopa (18F-FDOPA), 68Ga-DOTA(0)-Tyr(3)-octreotate ​​ (68Ga-DOTATATE), and 18F-fluorodeoxyglucose (18F-FDG). No other lesions (primary or metastatic) were found. Proton beam therapy was chosen over surgery due to potential complications and patient's preference. Following radiotherapy, she experienced symptom relief, with dopamine levels decreasing and chromogranin A normalizing, with the lesion remaining stable on 11-month follow-up imaging. This case highlights the rarity of primary bone PGLs and underscores the importance of comprehensive diagnostic approaches combining physical examinations, biochemical testing, functional imaging, and histopathological analysis properly guiding personalized treatment strategies. Additionally, proton beam therapy emerges as a highly suitable treatment option for head and neck paragangliomas (HNPGLs), offering effective tumor control with minimal complications.Published by Oxford University Press on behalf of the Endocrine Society 2024.