Malik Azhar Ali, Afandi Bachar, Jamil Gohar, Akhter Syed M J
Division of Endocrinology, Tawam Hospital, Al-ain, Abu Dhabi, UAE.
BMJ Case Rep. 2013 Aug 5;2013:bcr2013010063. doi: 10.1136/bcr-2013-010063.
Paraganglioma of the urinary bladder is extremely rare. In this report of a young man, hypertensive crisis and ventricular arrhythmia was provoked during cystoscopic evaluation of a bladder mass. A diagnosis of pheochromocytoma was considered following detection of high serum and urinary catecholamine levels. A preoperative meta-iodobenzylguanidine scan was, however, negative. The bladder mass was surgically removed following initiation of antihypertensive therapy. Pathological confirmation of extraadrenal pheochromocytoma was established. During a serial follow-up, serum and urine catecholamine levels were persistently elevated. This was explained by abnormalities on fluorodeoxyglucose positron emission tomography scan, which were considered to represent a metastatic malignant neuroendocrine tumour. The patient is on palliative chemotherapy for malignant paraganglioma. This case highlights variable presentation of pheochromocytoma, importance of having a high index of clinical suspicion for early recognition and prompt management and serious adverse consequence of a delayed diagnosis.
膀胱副神经节瘤极为罕见。在本病例报告中，一名年轻男性在膀胱肿物的膀胱镜检查过程中引发了高血压危象和室性心律失常。在检测到血清和尿儿茶酚胺水平升高后，考虑诊断为嗜铬细胞瘤。然而，术前间碘苄胍扫描结果为阴性。在开始抗高血压治疗后，手术切除了膀胱肿物。病理证实为肾上腺外嗜铬细胞瘤。在系列随访期间，血清和尿儿茶酚胺水平持续升高。这通过氟脱氧葡萄糖正电子发射断层扫描的异常表现得以解释，这些异常被认为代表转移性恶性神经内分泌肿瘤。该患者正在接受恶性副神经节瘤的姑息化疗。本病例突出了嗜铬细胞瘤的多样表现、对早期识别和及时处理保持高度临床怀疑指数的重要性以及延迟诊断的严重不良后果。
