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副神经节瘤还是嗜铬细胞瘤?一个特殊的诊断。

Paraganglioma or pheochromocytoma? A peculiar diagnosis.

作者信息

Marques Ricardo Rodrigues, Bello Carlos Tavares, Rafael Ana Alves, Fernandes Luís Viana

机构信息

Serviço de Cirurgia II, Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, 1349-019 Lisboa, Portugal.

Serviço de Endocrinologia, Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, 1349-019 Lisboa, Portugal.

出版信息

J Surg Case Rep. 2018 Apr 3;2018(4):rjy060. doi: 10.1093/jscr/rjy060. eCollection 2018 Apr.

DOI:10.1093/jscr/rjy060
PMID:29644041
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5887952/
Abstract

Paragangliomas and pheochromocytomas are rare catecholamine secreting neoplasms that arise in the extra-adrenal autonomic paraganglia and adrenal medulla, respectively. Although typically presenting with paroxysms of headaches, palpitations, diaphoresis and hypertension, a broad spectrum of clinical manifestations may occur. Diagnosis relies on biochemical studies followed by adequate imaging investigation. Cross sectional morphological and functional imaging modalities have improved diagnostic accuracy and are crucial in the surgical planning. The authors report on a case of a 64-year-old female that presented with severe hypertension, palpitations and fatigue as the manifestations of a catecholamine secreting neoplasm. Abdominal contrast enhanced computer tomography revealed a right sided 78 mm adrenal medullary tumor suggestive of a pheochromocytoma. Standard therapeutical strategies were initially unsuccessful, and additional investigation and therapy were required to cure the patient. The challenges faced by the multidisciplinary team in the pre-operative evaluation, medical management and surgical treatment are reported.

摘要

副神经节瘤和嗜铬细胞瘤是分别起源于肾上腺外自主神经副神经节和肾上腺髓质的罕见儿茶酚胺分泌性肿瘤。虽然通常表现为头痛、心悸、多汗和高血压发作,但也可能出现广泛的临床表现。诊断依赖于生化研究,随后进行充分的影像学检查。横断面形态学和功能成像方式提高了诊断准确性,对手术规划至关重要。作者报告了一例64岁女性病例,该患者以严重高血压、心悸和疲劳为儿茶酚胺分泌性肿瘤的表现。腹部增强计算机断层扫描显示右侧有一个78毫米的肾上腺髓质肿瘤,提示为嗜铬细胞瘤。标准治疗策略最初未成功,需要进一步检查和治疗以治愈该患者。报告了多学科团队在术前评估、药物管理和手术治疗中面临的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/087d2a5d5f76/rjy060f06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/4eb323ebfb4a/rjy060f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/ab1e4fa7935e/rjy060f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/d1a7a88de0a9/rjy060f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/12325307d0a7/rjy060f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/d14c488a79bf/rjy060f05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/087d2a5d5f76/rjy060f06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/4eb323ebfb4a/rjy060f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/ab1e4fa7935e/rjy060f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/d1a7a88de0a9/rjy060f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/12325307d0a7/rjy060f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/d14c488a79bf/rjy060f05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fc3/5887952/087d2a5d5f76/rjy060f06.jpg

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本文引用的文献

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2
Imaging work-up for screening of paraganglioma and pheochromocytoma in SDHx mutation carriers: a multicenter prospective study from the PGL.EVA Investigators.针对 SDHx 基因突变携带者的嗜铬细胞瘤和副神经节瘤的筛查的影像学检查:来自 PGL.EVA 研究人员的一项多中心前瞻性研究。
J Clin Endocrinol Metab. 2013 Jan;98(1):E162-73. doi: 10.1210/jc.2012-2975. Epub 2012 Nov 15.
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Clinical spectrum of pheochromocytoma.
嗜铬细胞瘤和副神经节瘤患者的种系SDHB和SDHD突变
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嗜铬细胞瘤的临床特征。
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Pheochromocytoma: new concepts and future trends.嗜铬细胞瘤:新概念与未来趋势。
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