Paraganglioma and phaeochromocytoma in adult Fontan patients.

IF 2.2 4区 医学 Q2 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
Stephanie Bm Tan, Maria Luz Garagiola, Xiaoyang Liu, Tae Kyoung Kim, Ciara Mo Brien
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引用次数: 0

Abstract

Introduction: The purpose of this study was to address the gap in knowledge in the incidence, presentation and imaging features of PPGL in Fontan patients. It has been hypothesised in the literature that Fontan circulation patients have an increased incidence of these tumours. This study is the largest cohort of adult Fontan patients in North America. The Fontan procedure is a palliative procedure for single ventricle congenital heart disease where the systemic venous return is directed into the pulmonary circulation. Fontan patients are at increased risk of developing several extracardiac complications including phaeochromocytoma and paraganglioma (PPGL).

Methods: This retrospective single-centre study includes all adult patients who were treated between 1 September 2015 and 1 September 2022 in a tertiary referral centre for adult congenital heart disease. Imaging and electronic medical records were retrospectively searched for the presence of PPGL.

Results: Five hundred and forty-seven Fontan patients were included in the study. Three patients had PPGL with an incidence of 0.5%. Two patients were diagnosed with paraganglioma and one with phaeochromocytoma. All patients were female. Median age of Fontan procedure was 1.8 years (range: 1.75-27 years). Median age of PPGL diagnosis was 19 years (range: 12-39 years). Median time from Fontan operation to diagnosis of PPGL was 12 years (range: 10.3-17.2 years).

Conclusion: Fontan patients are at increased risk of PPGL. Clinically, the diagnosis of PPGL is challenging as symptoms and serum markers can be confounding in this population; however, the imaging features of PPGL remain typical making the role of the radiologist crucial.

成人丰坦患者中的副神经节瘤和嗜铬细胞瘤。
导言:本研究的目的是填补有关丰坦患者PPGL发病率、表现和影像学特征的知识空白。有文献假设丰坦循环患者的此类肿瘤发病率较高。这项研究是北美最大的成人丰坦患者队列。丰坦手术是一种治疗单心室先天性心脏病的姑息性手术,它将全身静脉回流导入肺循环。丰坦患者罹患多种心外并发症的风险增加,其中包括辉铬细胞瘤和副神经节瘤(PPGL):这项回顾性单中心研究包括2015年9月1日至2022年9月1日期间在一家成人先天性心脏病三级转诊中心接受治疗的所有成人患者。研究人员对影像学和电子病历进行了回顾性检索,以确定是否存在PPGL:研究共纳入547名Fontan患者。三名患者患有PPGL,发生率为0.5%。两名患者被诊断为副神经节瘤,一名患者被诊断为嗜铬细胞瘤。所有患者均为女性。接受丰坦手术的中位年龄为1.8岁(范围:1.75-27岁)。确诊PPGL的中位年龄为19岁(范围:12-39岁)。从Fontan手术到确诊PPGL的中位时间为12年(范围:10.3-17.2年):结论:Fontan患者罹患PPGL的风险增加。临床上,PPGL的诊断具有挑战性,因为在这一人群中,症状和血清标志物可能会造成混淆;然而,PPGL的影像学特征仍然具有典型性,因此放射科医生的作用至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.30
自引率
6.20%
发文量
133
审稿时长
6-12 weeks
期刊介绍: Journal of Medical Imaging and Radiation Oncology (formerly Australasian Radiology) is the official journal of The Royal Australian and New Zealand College of Radiologists, publishing articles of scientific excellence in radiology and radiation oncology. Manuscripts are judged on the basis of their contribution of original data and ideas or interpretation. All articles are peer reviewed.
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