CLINICAL-FEATURES OF ADRENOCORTICAL CARCINOMA, PROGNOSTIC FACTORS, AND THE EFFECT OF MITOTANE THERAPY
CLINICAL-FEATURES OF ADRENOCORTICAL CARCINOMA, PROGNOSTIC FACTORS, AND THE EFFECT OF MITOTANE THERAPY
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DOI：
10.1056/nejm199004263221705
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发表时间：
1990-04-26
影响因子：
158.5
通讯作者：
BRICAIRE, H
中科院分区：
文献类型：
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作者：
LUTON, JP;CERDAS, S;BRICAIRE, H
肾上腺皮质癌是一种罕见的肿瘤，关于其自然史和治疗效果的信息有限。我们研究了 105 名肾上腺皮质癌患者（75 名女性和 30 名男性；平均年龄 46 岁），这些患者在 1963 年至 1987 年间转诊至我们。诊断前症状的平均持续时间为 8.7 个月。诊断时，68%的患者有内分泌症状，30%有远处转移。激素研究表明 79% 的肿瘤具有功能性。 80 名患者接受了手术，59 名患者还接受了肾上腺细胞毒剂米托坦治疗。术后中位无病间隔为 12.1 个月（范围：1 至 175）。 82% 的患者发生肿瘤播散，最常见的是肺、肝和邻近器官。中位生存时间为 14.5 个月（范围 < 1 至 175），五年生存率为 22%。年龄超过 40 岁和诊断时存在转移是唯一被认为表明预后不良的因素。米托坦控制了 75% 患者的荷尔蒙分泌。八名接受米托坦治疗的患者肿瘤部分消退，但该药物对生存没有显着影响。我们的结论是，肾上腺皮质癌的预后较差。米托坦治疗可能会带来短暂的好处，特别是在控制内分泌症状方面。
Adrenocortical carcinoma is a rare tumor, and only limited information is available about its natural history and the effects of therapy. We studied 105 patients (75 female and 30 male; mean age, 46 years) with adrenocortical carcinoma who were referred to us between 1963 and 1987. The average duration of symptoms before diagnosis was 8.7 months. At the time of diagnosis, 68 percent of the patients had endocrine symptoms, and 30 percent had distant metastases. Hormonal studies showed that 79 percent of the tumors were functional. Eighty patients underwent surgery, and 59 also received the adrenal cytotoxic agent mitotane. The median disease-free interval after surgery was 12.1 months (range, 1 to 175). Tumor dissemination occurred in 82 percent of the patients, most commonly to the lung, liver, and adjacent organs. The median survival time was 14.5 months (range, < 1 to 175), and the five-year survival was 22 percent. Age over 40 years and the presence of metastases at the time of diagnosis were the only factors recognized as indicating a poor prognosis. Mitotane controlled hormonal secretion in 75 percent of the patients. Eight mitotane-treated patients had partial tumor regression, but the drug did not have a significant effect on survival. We conclude that adrenocortical carcinoma carries a poor prognosis. Mitotane therapy may offer transient benefits, particularly in controlling endocrine symptoms.