Edwards G A, Smythe G A, Graham P E, Lazarus L
Department of Chemical Pathology, St Vincent's Hospital, Darlinghurst, NSW.
Med J Aust. 1992 Feb 3;156(3):153-7. doi: 10.5694/j.1326-5377.1992.tb139697.x.
To examine the impact of recent advances in diagnostic technology on the spectrum of clinical and biochemical features of patients presenting with a new diagnosis of phaeochromocytoma.
A retrospective review of the clinical and biochemical features of patients diagnosed by our laboratory as having phaeochromocytoma within a 27-month period up to December, 1990. Noradrenaline, adrenaline and dihydroxyphenylglycol were assayed in 24-hour urine specimens (19 patients) or plasma (1 anuric patient) by gas chromatography/mass spectrometry.
A tertiary level chemical pathology department.
Twenty patients with a new diagnosis of phaeochromocytoma.
The classic, episodic adrenergic symptoms traditionally associated with phaeochromocytoma were absent in 9 of the 20 patients (45%). "Atypical" phaeochromocytoma presented as a mass on computed tomography imaging (6 patients, 30%), "phaeochromocytoma crisis" (4 patients, 20%) or family screening (1 patient, 5%). Excessive adrenaline production was found in 11 patients (55%) and six (30%) had predominantly adrenaline-secreting tumours. The urinary noradrenaline:dihydroxyphenylglycol ratio was raised in all nine patients with predominantly noradrenaline-secreting tumours but was not raised in nine out of ten patients with adrenaline-secreting phaeochromocytoma. Adrenaline excretion was significantly correlated with tumour size (r = 0.8; P less than 0.05).
Advances in diagnostic technology, particularly specific adrenaline assays and computed tomography, have made possible the early diagnosis of patients with phaeochromocytoma presenting in ways previously thought to be uncommon. All patients with adrenal masses noted incidentally on CT scan should be investigated for phaeochromocytoma. Adrenaline-secreting tumours are common and both noradrenaline and adrenaline should be assayed in all patients investigated for phaeochromocytoma.
探讨诊断技术的最新进展对初诊嗜铬细胞瘤患者临床及生化特征谱的影响。
回顾性分析在截至1990年12月的27个月内,经本实验室诊断为嗜铬细胞瘤的患者的临床及生化特征。采用气相色谱/质谱法对24小时尿液标本(19例患者)或血浆(1例无尿患者)中的去甲肾上腺素、肾上腺素和二羟苯乙二醇进行检测。
三级化学病理科。
20例初诊嗜铬细胞瘤患者。
20例患者中有9例(45%)无传统上与嗜铬细胞瘤相关的典型发作性肾上腺素能症状。“非典型”嗜铬细胞瘤在计算机断层扫描成像上表现为肿块(6例患者,30%)、“嗜铬细胞瘤危象”(4例患者,20%)或家族筛查(1例患者,5%)。11例患者(55%)存在肾上腺素分泌过多,6例(30%)主要为分泌肾上腺素的肿瘤。9例主要分泌去甲肾上腺素的肿瘤患者的尿去甲肾上腺素:二羟苯乙二醇比值升高,但10例分泌肾上腺素的嗜铬细胞瘤患者中有9例该比值未升高。肾上腺素排泄与肿瘤大小显著相关(r = 0.8;P < 0.05)。
诊断技术的进展,尤其是特异性肾上腺素检测和计算机断层扫描,使得以先前认为不常见的方式出现的嗜铬细胞瘤患者得以早期诊断。所有在CT扫描中偶然发现肾上腺肿块的患者均应进行嗜铬细胞瘤检查。分泌肾上腺素的肿瘤很常见,所有接受嗜铬细胞瘤检查的患者都应检测去甲肾上腺素和肾上腺素。