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囊性嗜铬细胞瘤是一个具有特殊临床、影像学和组织学特征的独特亚组,可能会误导诊断。

Cystic phaeochromocytoma is a distinctive subgroup with special clinical, imaging and histological features that might mislead the diagnosis.

作者信息

Andreoni Cassio, Krebs Rodrigo K, Bruna Paulo C, Goldman Suzan M, Kater Claudio E, Alves Maria T S, Ortiz Valdemar

机构信息

Division of Urology, Federal University of Sao Paulo, Brazil.

出版信息

BJU Int. 2008 Feb;101(3):345-50. doi: 10.1111/j.1464-410X.2007.07370.x. Epub 2007 Dec 7.

DOI:10.1111/j.1464-410X.2007.07370.x
PMID:18070168
Abstract

OBJECTIVE

To report and analyse cases of cystic phaeochromocytoma at our institution and in previous publications, as adrenal cystic masses are usually associated with nonfunctional lesions, but they can be phaeochromocytoma.

PATIENTS AND METHODS

The prospective adrenalectomy database at our institution was reviewed to identify patients with phaeochromocytoma and cystic lesions. The clinical, radiological and histological features of cystic phaeochromocytoma were evaluated. Other previously published data were identified and compared with the present series.

RESULTS

In all, there were 107 adrenalectomies at our institution from November 2000 to March 2007; 31 of these patients had a confirmed diagnosis of phaeochromocytoma and six (19%) were cystic phaeochromocytomas. Three of the six cases were asymptomatic and had a negative biochemical evaluation (one male and five females, mean tumour size 6.6 cm); the imaging diagnosis was mainly based on computed tomography and magnetic resonance imaging, and the foremost feature was the enhancement of the tumour rim associated with a central cystic mass. Nine other previously published cases were reviewed.

CONCLUSIONS

From the present large series and previous anecdotally reported cases, patients with cystic phaeochromocytomas are more likely to be asymptomatic, to complain of chronic abdominal pain, and to have a negative biochemical evaluation, which might deter physicians from conducting a full evaluation for phaeochromocytoma and mislead the final diagnosis. As such, there could be an even higher prevalence of this entity that is yet to be determined.

摘要

目的

报告并分析我院及既往文献中囊性嗜铬细胞瘤的病例,因为肾上腺囊性肿块通常与无功能病变相关,但也可能是嗜铬细胞瘤。

患者与方法

回顾我院前瞻性肾上腺切除术数据库,以确定患有嗜铬细胞瘤和囊性病变的患者。对囊性嗜铬细胞瘤的临床、影像学和组织学特征进行评估。查找其他既往发表的数据并与本系列进行比较。

结果

2000年11月至2007年3月,我院共进行了107例肾上腺切除术;其中31例患者确诊为嗜铬细胞瘤,6例(19%)为囊性嗜铬细胞瘤。6例中有3例无症状,生化检查结果为阴性(1例男性和5例女性,肿瘤平均大小6.6 cm);影像学诊断主要基于计算机断层扫描和磁共振成像,最主要的特征是肿瘤边缘强化伴中央囊性肿块。回顾了其他9例既往发表的病例。

结论

从目前的大样本系列及既往零星报道的病例来看,囊性嗜铬细胞瘤患者更可能无症状、主诉慢性腹痛且生化检查结果为阴性,这可能会阻碍医生对嗜铬细胞瘤进行全面评估并误导最终诊断。因此,该实体的实际患病率可能更高,尚有待确定。

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