Lázaro Bruno, Klemz Mônica, Flores Marlo Steiner, Landeiro José Alberto
Brazilian Air Force Hospital, Rio de Janeiro, RJ, Brazil.
Arq Neuropsiquiatr. 2003 Jun;61(2B):463-7. doi: 10.1590/s0004-282x2003000300026. Epub 2003 Jul 28.
A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. Magnetic resonance imaging (MRI), plain X-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I 131-MIBG, or chemotherapy, especially in malignant pheochromocitomas.
副神经节瘤是一种罕见肿瘤，由嗜铬细胞组成，这些细胞群与自主神经系统相关。当肿瘤发生在肾上腺时，称为嗜铬细胞瘤。恶性副神经节瘤非常少见，通过原发肿块全切术后局部复发或远处转移的发现来诊断。我们报告一例29岁有颈臂部疼痛的女性病例。1995年她接受了颈动脉体瘤切除术。磁共振成像（MRI）、普通X线和计算机断层扫描显示C5、T5和T12有多处病变。她接受了手术以纠正颈部病变。组织学和免疫组化检测显示为恶性副神经节瘤。她接受了辅助放疗，治疗后临床症状改善，一年后无症状。治疗方法以肿瘤全切为基础。远处转移的治疗可采用辅助措施，如传统放疗、I 131 - MIBG或化疗，特别是在恶性嗜铬细胞瘤中。
