THE PHEOCHROMOCYTOMA - A BENIGN, INTRA-ADRENAL, HYPERTENSIVE, SPORADIC UNILATERAL TUMOR - DOES IT EXIST
THE PHEOCHROMOCYTOMA - A BENIGN, INTRA-ADRENAL, HYPERTENSIVE, SPORADIC UNILATERAL TUMOR - DOES IT EXIST
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DOI：
10.1007/bf00353738
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发表时间：
1994-07-01
影响因子：
2.6
通讯作者：
HILLER, W
中科院分区：
文献类型：
--
作者：
PROYE, CAG;VIX, M;HILLER, W
这项研究旨在检查嗜铬细胞瘤（PHEO）的频率，定义为“良性，肾上腺内，高血压，零星，单侧肿瘤。 ，分别对41年进行了审查。在共有310个Pheos的组合中，有48个（15.5％）为恶性（即转移性），而262（84.5％）是良性的。 42（13.5％）是异位（占恶性肿瘤的35％，而良性肿瘤的9.5％）； 230（74.2％）是高血压（占良性肿瘤的74％，占恶性肿瘤的73％）； 29（9.4％）是双边的，其中包括23名具有家族病史的患者； 41（13.2％）患有男性II型A或B综合征；和20个（6.5％）发生在幻影病中。一些患者有PHEO的非人类家族史（n = 5），看似零星的甲状旁腺功能亢进（n = 4）或其他相关的神经内分泌肿瘤（n = 9）。有时将其中几个功能组合在一起。最终，有125例（40.3％）的病例安装了对肿瘤的经典描述，在初次呈现时为47.0％，随访结束时40.3％。转移酶的迟到或家族性疾病的常规诊断使得终身随访是强制性的。在嗜铬细胞瘤患者中可以表明遗传研究。
This study aims to examine the frequency of the pheochromocytoma (pheo), defined as a ''benign, intra-adrenal, hypertensive, sporadic, unilateral tumor.'' Three large series amounting to 310 subphrenic chromaffin tumors operated over periods of 17, 23, and 41 years, respectively, have been reviewed. Among those combined 310 pheos, 48 (15.5%) were malignant (i.e., metastatic) and 262 (84.5%) were benign; 42 (13.5%) were ectopic (35% of malignant tumors versus 9.5% of benign tumors); 230 (74.2%) were hypertensive (74% of benign tumors versus 73% of malignant tumors); 29 (9.4%) were bilateral, including 23 patients with a family history; 41 (13.2%) of patients had MEN II type A or B syndrome; and 20 (6.5%) occurred in a phacomatosis setting. Some of the patients had a non-MEN family history of pheo (n = 5), seemingly sporadic hyperparathyroidism (n = 4), or other associated neuroendocrine tumor (n = 9). Sometimes several of these features were combined. Finally 125 (40.3%) cases fitted the classic description of the tumor, 47.0% at the time of initial presentation and 40.3% at the end of follow-up. Late occurrence of metastases or metachronous diagnosis of familial disease make lifelong follow-up mandatory. Genetic studies may be indicated in pheochromocytoma patients.