Chan F K, Choi K L, Tiu S C, Shek C C, Au Yong T K
Department of Medicine, Queen Elizabeth Hospital, 30 Gascoigne Road, Kowloon, Hong Kong.
Hong Kong Med J. 2000 Sep;6(3):325-8.
Malignant phaeochromocytoma is defined as the presence of tumour deposits at sites that are normally devoid of chromaffin cells. We report on a 63-year-old man who had a giant malignant phaeochromocytoma of the right adrenal gland that encased the inferior vena cava. The urinary excretion rates of catecholamines and their metabolites were normal, except for normetanephrine, which was excreted at a higher rate than normal. The tumour was surgically unresectable by laparotomy. Postoperatively, the patient was given a 4-month trial of subcutaneous octreotide and intravenous meta-iodobenzylguanidine I 131. Occult lung secondary tumours were first detected by meta-iodobenzylguanidine scintigraphy after 2 years, and the patient died of bone and lung metastases 1 year later. Because phaeochromocytoma is rare, local experience in managing this disease is limited. This report alerts physicians of the methods of diagnosing and managing surgically unresectable malignant phaeochromocytoma.
恶性嗜铬细胞瘤的定义为在通常没有嗜铬细胞的部位出现肿瘤沉积物。我们报告一名63岁男性,他患有右侧肾上腺巨大恶性嗜铬细胞瘤,该肿瘤包绕下腔静脉。儿茶酚胺及其代谢产物的尿排泄率正常,但去甲变肾上腺素的排泄率高于正常。经剖腹手术,该肿瘤无法切除。术后,患者接受了为期4个月的皮下注射奥曲肽和静脉注射间碘苄胍I 131试验。隐匿性肺转移瘤在2年后首次通过间碘苄胍闪烁显像检测到,患者1年后死于骨转移和肺转移。由于嗜铬细胞瘤罕见,当地对这种疾病的管理经验有限。本报告提醒医生注意诊断和管理无法手术切除的恶性嗜铬细胞瘤的方法。