Mornex R
Service d'Endocrinologie-diabète-nutrition, Hôpital Edouard Herriot, Lyon, France.
Acta Chir Belg. 1991 Mar-Apr;91(2):102-6.
This study of pheochromocytoma addresses only the four aspects which changed dramatically. The diagnosis depends on the clinical picture, biological tests (plasma methoxamine) and the elimination of catecholamines and their metabolites. Abdominal CT scan localizes the tumor. M.I.B.G. scintigraphy is complementary. When the pheochromocytoma is part of a generalized endocrinopathy (MEN II) familial screening is essential. The incidence of malignant tumours is probably higher than the published 10%; these tumors are either of primary malignancy or malignant after a long latency period. Survival, even in primary malignant tumours is considerable. Multiple localizations are detected by M.I.B.G. scintigraphy and determine the treatment strategy. Surgical therapy with a near zero mortality is the factor which most favourably influenced the prognosis. The still high peroperative morbidity, not prevented by adrenolytic therapy, can be counteracted with appropriate pharmacological agents.
这项嗜铬细胞瘤研究仅涉及发生显著变化的四个方面。诊断取决于临床表现、生物学检测(血浆甲氧明)以及儿茶酚胺及其代谢产物的清除情况。腹部CT扫描可对肿瘤进行定位。间碘苄胍闪烁扫描具有辅助作用。当嗜铬细胞瘤是全身性内分泌病(MEN II)的一部分时,家族筛查至关重要。恶性肿瘤的发生率可能高于已公布的10%;这些肿瘤要么是原发性恶性肿瘤,要么是经过很长潜伏期后恶变的。即使是原发性恶性肿瘤,生存率也相当可观。间碘苄胍闪烁扫描可检测到多处肿瘤定位,并据此确定治疗策略。手术治疗死亡率近乎为零,是对预后最具积极影响的因素。术前发病率仍然较高,尽管使用肾上腺素能阻滞剂治疗也无法预防,但可通过适当的药物加以对抗。