Chew S L, Dacie J E, Reznek R H, Newbould E C, Sheaves R, Trainer P J, Lowe D G, Shand W S, Hungerford J, Besser G M
Department of Endocrinology, St. Bartholomew's Hospital, London.
Q J Med. 1994 Jan;87(1):49-54.
Three unrelated patients with von Hippel-Lindau disease had phaeochromocytomas diagnosed as a result of screening in their third decade. All had raised 24-h urinary noradrenaline levels and obvious unilateral adrenal tumours on ultrasound scanning and computed tomography. The contralateral adrenal appeared either normal or equivocally abnormal using the same imaging techniques. Radiolabelled meta-iodo-benzylguanidine uptake and enhanced T2-weighted magnetic resonance signals confirmed the ipsilateral lesion, but showed no abnormality in the contralateral adrenal. Nevertheless, venous sampling demonstrated abnormally elevated noradrenaline:adrenaline ratios (normal < 1) in blood draining both adrenals in all three patients. All underwent bilateral adrenalectomy: histological examination confirmed bilateral phaeochromocytomas in all three patients, with the smaller adrenal showing tumours between 3 and 11 mm in diameter. A fourth patient with von Hippel-Lindau disease was also evaluated because of a left 'suprarenal' mass discovered by ultrasound scanning. Urinary and plasma catecholamines were normal. Computed tomography showed a normal right adrenal, with a left para-aortic mass lying superior to the left adrenal. Magnetic resonance scanning showed an enhanced T2 signal from the mass, which also showed positive uptake with radiolabelled mIBG. At operation, a non-secretory left para-aortic paraganglioma was found. Venous sampling suggested that the left adrenal was normal, and this was confirmed on histology. Venous sampling for catecholamine assays is effective in locating small bilateral phaeochromocytomas and defining a normal adrenal gland, and can be useful in preventing unnecessary or repeat operations in patients with non Hippel-Lindau disease.
三名患有冯·希佩尔-林道病的非相关患者在三十多岁时通过筛查被诊断出患有嗜铬细胞瘤。他们的24小时尿去甲肾上腺素水平均升高，超声扫描和计算机断层扫描显示明显的单侧肾上腺肿瘤。使用相同的成像技术，对侧肾上腺看起来正常或有可疑异常。放射性标记的间碘苄胍摄取和增强的T2加权磁共振信号证实了同侧病变，但对侧肾上腺未显示异常。然而，静脉采样显示所有三名患者双侧肾上腺引流血液中的去甲肾上腺素：肾上腺素比值异常升高（正常<1）。所有患者均接受了双侧肾上腺切除术：组织学检查证实所有三名患者均为双侧嗜铬细胞瘤，较小的肾上腺中肿瘤直径在3至11毫米之间。第四名患有冯·希佩尔-林道病的患者也因超声扫描发现左侧“肾上腺上方”肿块而接受了评估。尿和血浆儿茶酚胺正常。计算机断层扫描显示右侧肾上腺正常，左侧主动脉旁有一个肿块位于左肾上腺上方。磁共振扫描显示肿块T2信号增强，放射性标记的间碘苄胍摄取也呈阳性。手术中发现一个无分泌功能的左侧主动脉旁副神经节瘤。静脉采样提示左肾上腺正常，组织学检查证实了这一点。用于儿茶酚胺测定的静脉采样对于定位双侧小嗜铬细胞瘤和确定正常肾上腺有效，并且可有助于防止非希佩尔-林道病患者进行不必要的或重复的手术。
