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一例罕见的将腹膜后副神经节瘤误诊为十二指肠胃肠道间质瘤的严重病例:病例报告

A rare serious case of retroperitoneal paraganglioma misdiagnosed as duodenal gastrointestinal stromal tumor: a case report.

作者信息

Mahmoud Schauki, Salami Maissam, Salman Hosam

机构信息

Department of General Surgery, Albassel Hospital, Tartous, Syrian Arab Republic.

Department of Anaesthesiology, Albassel Hospital, Tartous, Syrian Arab Republic.

出版信息

BMC Surg. 2020 Mar 16;20(1):49. doi: 10.1186/s12893-020-00712-z.

DOI:10.1186/s12893-020-00712-z
PMID:32178651
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7077140/
Abstract

BACKGROUND

Pheochromocytoma (PCC) and Paraganglioma (PGL) are rare neuroendocrine neoplasms. These tumors harbour disastrous consequences during surgery due to catecholamine hypersecretion if they are undiagnosed or prepared inadequately preoperatively.

CASE PRESENTATION

A 41- year- old lady presented with mild left flank discomfort. She had experienced recurrent anxiety attacks accompanied by palpitations and headache which were managed previously as panic attacks. Radiologic investigations showed a retroperitoneal mass that located anteromedial to the left kidney, separated from the left adrenal gland and adherent to the 4th duodenal segment. During admission, her vital signs showed slight elevation of blood pressure (140\90-160\110) mmHg, thus 24-h urine metanephrine and normetanephrine were requested and the results revealed normal values. Upper gastrointestinal endoscopy failed to pass beyond the 3th duodenal segment and showed no pathologic evidence. According to her findings, a diagnosis of duodenal gastrointestinal stromal tumor (GIST) was suspected. During laparotomy, crises of hypertension and tachycardia followed by severe hypotension made the resection of the misdiagnosed mass very tricky. Immunohistochemical staining studies confirmed the diagnosis of paraganglioma.

CONCLUSION

Paraganglioma is a life threatening disease and should always be considered as a differential diagnosis of asymptomatic retroperitoneal mass. The aim of our study is to present a challenging case of an undiagnosed retroperitoneal paraganglioma and to alarm our colleagues from such troubles.

摘要

背景

嗜铬细胞瘤(PCC)和副神经节瘤(PGL)是罕见的神经内分泌肿瘤。如果这些肿瘤术前未被诊断或准备不充分,手术期间由于儿茶酚胺分泌过多会带来灾难性后果。

病例介绍

一名41岁女性出现轻度左腰部不适。她曾经历反复发作的焦虑发作,伴有心悸和头痛,之前被当作惊恐发作进行处理。影像学检查显示一个位于左肾前内侧的腹膜后肿块,与左肾上腺分离并附着于十二指肠第四段。入院期间,她的生命体征显示血压略有升高(140\90 - 160\110)mmHg,因此检测了24小时尿间甲肾上腺素和去甲间肾上腺素,结果显示正常。上消化道内镜未能通过十二指肠第三段,未发现病理证据。根据检查结果,怀疑为十二指肠胃肠道间质瘤(GIST)。剖腹手术期间,高血压和心动过速危机随后出现严重低血压,使得误诊肿块的切除非常棘手。免疫组织化学染色研究证实为副神经节瘤。

结论

副神经节瘤是一种危及生命的疾病,应始终被视为无症状腹膜后肿块的鉴别诊断。我们研究的目的是呈现一例未被诊断的腹膜后副神经节瘤的具有挑战性的病例,并提醒我们的同事注意此类问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/ec7971611952/12893_2020_712_Fig12_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/2e65512e3ff5/12893_2020_712_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/7d8bd0c64e8a/12893_2020_712_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/9137819ef2be/12893_2020_712_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/e8d2a387d644/12893_2020_712_Fig7_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/ed2f06be3557/12893_2020_712_Fig8_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/3fa0ff5bc94b/12893_2020_712_Fig9_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/88de1363c1bc/12893_2020_712_Fig10_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/48b7e261c0ea/12893_2020_712_Fig11_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69bd/7077140/ec7971611952/12893_2020_712_Fig12_HTML.jpg

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