Department of Endocrinology and General Medicine, Mater Dei Hospital, Msida, Malta
Department of Endocrinology and General Medicine, Mater Dei Hospital, Msida, Malta.
BMJ Case Rep. 2023 Sep 12;16(9):e256052. doi: 10.1136/bcr-2023-256052.
We report a rare case of a functional bladder paraganglioma diagnosed in a young man who presented with acute compressive thoracic myelopathy secondary to vertebral metastasis. A histological diagnosis of a metastatic paraganglioma was made following biopsy of a rib lesion. CT revealed a lesion in the inferior wall of the bladder, which demonstrated avid uptake on 68Ga-DOTATATE PET/CT. Serum metanephrine levels were more than 40 times the upper limit of normal. The patient was hypertensive and treatment with doxazosin was initiated. In view of neurological deterioration, he required urgent spinal decompression to preserve neurological function and prevent permanent paraplegia. Despite inadequate alpha-blockade, surgery was successful, and the perioperative course was uneventful. Alpha-blockade was subsequently optimised. Treatment with cyclophosphamide, vincristine and dacarbazine was started but, in view of disease progression, treatment was subsequently changed to sunitinib.
我们报告了一例罕见的功能性膀胱副神经节瘤病例，该病例发生于一名年轻男性，他因椎体转移导致的急性压迫性胸髓病就诊。肋骨病变活检后作出转移性副神经节瘤的组织学诊断。CT 显示膀胱下壁有一处病变，68Ga-DOTATATE PET/CT 显示其摄取明显增加。血清间甲肾上腺素水平超过正常值上限的 40 倍。患者患有高血压，开始用多沙唑嗪治疗。鉴于神经功能恶化，他需要紧急进行脊柱减压以保护神经功能，防止永久性截瘫。尽管α受体阻滞剂的作用不足，但手术成功，围手术期过程顺利。随后对α受体阻滞剂进行了优化。开始使用环磷酰胺、长春新碱和达卡巴嗪进行治疗，但鉴于疾病进展，随后改为舒尼替尼进行治疗。
