Kakoki Katsura, Miyata Yasuyoshi, Shida Youhei, Hakariya Tomoaki, Takehara Kosuke, Izumida Seiya, Sekino Motohiro, Kinoshita Naoe, Igawa Tsukasa, Fukuoka Junya, Sakai Hideki
Department of Urology, Nagasaki University, Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, 852-8501, Japan.
BMC Res Notes. 2015 Dec 9;8:758. doi: 10.1186/s13104-015-1738-z.
Pheochromocytoma is a neuroendocrine tumor that predominantly presents with hypertension, palpitations, and tachycardia due to excessive catecholamine excretion. Although pheochromocytoma multisystem crisis (PMC) is relatively rare, urologists and clinicians should focus on early diagnosis as delay in initiating the appropriate treatment can lead to mortality
A 70-year-old man developed ileus after a few days of medication for hypertension. Computed tomography incidentally revealed a left adrenal mass. This finding together with his clinical course was compatible with pheochromocytoma. An α-blocker was administered immediately, and his blood pressure was well controlled. However, his general condition and laboratory data deteriorated rapidly, and the patient was diagnosed with PMC with lethal status. Thus, emergency adrenalectomy was performed without confirmation of catecholamine levels. From the resected specimen, his tumor was judged as pheochromocytoma. On immunohistochemical analysis, the proliferation index evaluated by Ki-67 staining was 9.7 %. This case report was approved by the Human Ethics Review Committee of the Nagasaki University Hospital.
The present case of PMC was successfully treated with emergency surgery. The benign pheochromocytoma also presented with high cell proliferation potential, which may be a cause of the extreme aggressiveness of PMC.
嗜铬细胞瘤是一种神经内分泌肿瘤,主要因儿茶酚胺分泌过多而表现为高血压、心悸和心动过速。尽管嗜铬细胞瘤多系统危象(PMC)相对罕见,但泌尿外科医生和临床医生应关注早期诊断,因为延迟启动适当治疗可能导致死亡。
一名70岁男性在接受高血压药物治疗几天后出现肠梗阻。计算机断层扫描偶然发现左肾上腺肿块。这一发现连同他的临床病程与嗜铬细胞瘤相符。立即给予α受体阻滞剂,其血压得到良好控制。然而,他的一般状况和实验室数据迅速恶化,患者被诊断为处于致死状态的PMC。因此,在未确认儿茶酚胺水平的情况下进行了急诊肾上腺切除术。从切除的标本来看,他的肿瘤被判定为嗜铬细胞瘤。免疫组织化学分析显示,通过Ki-67染色评估的增殖指数为9.7%。本病例报告经长崎大学医院人类伦理审查委员会批准。
本例PMC通过急诊手术成功治疗。良性嗜铬细胞瘤也表现出高细胞增殖潜能,这可能是PMC极端侵袭性的一个原因。