Rakonjac Natasa, Marković Slobodan
Clinical Hospital Centre Zemun, Belgrade, Serbia.
Vojnosanit Pregl. 2012 Aug;69(8):721-4.
Collections of neuroendocrine cells dispersed throughout the body are known as paraganglia and the tumor arising from these paraganglia are known as paragangliomas. Paragangliomas located along side the aorta are associated with the pheochromocytoma and they secrete and store catecholamines.
We reported a 29-year-old woman with episodes of headache, palpitations, dizziness and sweats, associated with hypertension. Elevated urine catecholamines were consistent with pheochromocytoma. Nuclear magnetic resonance (NMR) was performed and the symptoms were caused by associated mediastinal and retroperitoneal paragangliomas. The tumors were surgically removed. In a 3-year follow-up period the patient had two recidivate lesions, one of them was surgically removed, and for the last one a 6-month follow-up was recommended, because urine cathecholamine level was not significantly elevated and blood pressure was normal.
Clinical and imaging data of patients with extra adrenal paragangliomas are not specific. Many of them may be asymptomatic even when the lesion is large, but if tumor is functional, diagnosis may be easier. Patients should be initially evaluated by determining catecholamine level, followed by computerized tomography (CT) or NMR to locate the primary lesion. Since there are no definite microscopic criteria for the distinction between benign and malignant tumors, radical excision and prolonged follow-up is necessary.
散布于全身的神经内分泌细胞集合称为副神经节，起源于这些副神经节的肿瘤称为副神经节瘤。位于主动脉旁的副神经节瘤与嗜铬细胞瘤相关，它们分泌和储存儿茶酚胺。
我们报告了一名29岁女性，有头痛、心悸、头晕和出汗发作，伴有高血压。尿儿茶酚胺升高与嗜铬细胞瘤相符。进行了核磁共振（NMR）检查，症状由相关的纵隔和腹膜后副神经节瘤引起。肿瘤被手术切除。在3年的随访期内，患者有两次复发病变，其中一次被手术切除，对于最后一次，建议进行6个月的随访，因为尿儿茶酚胺水平没有明显升高且血压正常。
肾上腺外副神经节瘤患者的临床和影像学数据不具有特异性。即使病变很大，许多患者可能无症状，但如果肿瘤有功能，诊断可能更容易。患者应首先通过测定儿茶酚胺水平进行评估，随后进行计算机断层扫描（CT）或NMR以定位原发病变。由于良性和恶性肿瘤之间没有明确的微观标准，根治性切除和长期随访是必要的。
