Becquart J, Marchand X, Kacet S, Ythier H, Decoster H, Dujardin J J, Lekieffre J
Ann Cardiol Angeiol (Paris). 1987 Apr;36(4):191-6.
We are reporting a case of malignant pheochromocytoma surgically treated initially for an isolated left pararenal localization, and which recurred several years later accompanied with numerous metastases. Despite of a treatment with Iodine 131 MIBG, the evolution was rapidly fatal with a picture of cardiac failure. This cardiac involvement would be linked to a myocarditis directly secondary to the catecholamines and causing a marked increase of the free fatty acids concentration in the heart tissue. In reference to this case, all the data which may tend to suspect the malignant nature of a pheochromocytoma, present in 10 p. cent of the cases, are successively reviewed. There is no clinical specificity. The presence of a mixed secretion with marked urinary dopamine secretion, would not present, for all authors, the same criteria of specificity. Thoraco-abdominal scan and scintigraphy with iodine 131 MIBG are the two tests permitting to demonstrate, with a great sensitivity and specificity, an extra-adrenal localization, which is the best argument in favor of a malignancy since 30 to 40 p. cent of extra-adrenal pheochromocytomas are malignant, more especially as the metastases are located in areas where there are no embryonic remnants of tissues containing chromaffin cells. This permits to appreciate the difference between a non-malignant multicentric pheochromocytoma and a malignant pheochromocytoma. The ideal treatment of a malignant pheochromocytoma rests on surgery under the condition that there are ony one or two metastases. This procedure is preceded by a sodium nitroprusside preparation and followed with an alpha-blockers treatment. In case of multiple metastases, the therapeutic use of iodine 131 MIBG seems to be a tempting alternative.
我们报告了一例恶性嗜铬细胞瘤病例，该病例最初因孤立的左肾旁定位接受手术治疗，数年后复发并伴有多处转移。尽管采用了碘131 MIBG治疗，但病情迅速恶化，最终因心力衰竭死亡。这种心脏受累与儿茶酚胺直接导致的心肌炎有关，导致心脏组织中游离脂肪酸浓度显著升高。参照该病例，我们依次回顾了所有可能提示嗜铬细胞瘤恶性性质的数据，此类数据在10%的病例中存在。其并无临床特异性。对于所有作者而言，存在显著尿多巴胺分泌的混合分泌情况，其特异性标准并不相同。胸腹部扫描和碘131 MIBG闪烁扫描是两项能够以高灵敏度和特异性显示肾上腺外定位的检查，这是支持恶性肿瘤的最佳依据，因为30%至40%的肾上腺外嗜铬细胞瘤是恶性的，尤其是当转移灶位于不存在含嗜铬细胞组织胚胎残余的区域时。这有助于鉴别非恶性多中心嗜铬细胞瘤和恶性嗜铬细胞瘤。恶性嗜铬细胞瘤的理想治疗方法是在仅有一两个转移灶的情况下进行手术。此手术前需用硝普钠准备，术后需用α受体阻滞剂治疗。若有多个转移灶，碘131 MIBG的治疗应用似乎是一个诱人的选择。
