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肾原始神经外胚层肿瘤。来自叙利亚的首个病例系列。

Renal primitive neuroectodermal tumor. The first case series from Syria.

作者信息

Al Mousa Ahmad, Kitaz Mohammad Nour, Brimo Alsaman Muhamad Zakaria, Rezkallah Vairy, Ghabreau Lina, Al-Hadid Ibrahim

机构信息

Department of Urology, Aleppo University Hospital, Aleppo, Syria.

Neurosurgery Department, Aleppo University Hospital, Aleppo, Syria.

出版信息

Ann Med Surg (Lond). 2022 Sep 25;82:104740. doi: 10.1016/j.amsu.2022.104740. eCollection 2022 Oct.

DOI:10.1016/j.amsu.2022.104740
PMID:36268368
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9577866/
Abstract

Primitive neuroectodermal tumor (PNET) mainly arises from soft tissues of the extremities such as humerus, femur, C tibia. It rarely arises from kidney; less than 200 cases have been reported in the literature. The clinical presentation and radiography findings are not specific. Here we first report two cases of renal primitive neuroectodermal tumor in Syria. the first patient was 26-year-old- female that presented to urology clinic complaining of right flank pain. Ultrasonography of the abdomen showed a large mixed heterogeneous mass in the right kidney with no hemorrhage or calcification and MSCT of abdomen and pelvis demonstrate a mixed well-demarcated heterogeneous mass measuring (74117) mm in the right kidney right radical nephrectomy was performed. The second patient 19-year-old-male presented with left flank pain. Ultrasonography of the abdomen showed mixed large mass involving the left kidney, with unmarked border. The CT of the abdomen and pelvis demonstrating a (3011090) mm left renal mass and periaortic lymphadenopathy measuring (4528) mm. The patient underwent Left radical nephrectomy with periaortic lymphadenectomy dissection. The final diagnosis for both cases was Renal PNET based on microscopic and immunohistochemistry examination. In patient with suspected renal mass in the radiographic images, the diagnosis of renal primitive neuroectodermal tumor should be kept in the mind despite its rarity. The final diagnosis is done by histopathological study in association with immunohistochemical examination.

摘要

原始神经外胚层肿瘤(PNET)主要起源于四肢的软组织,如肱骨、股骨、胫骨。它很少起源于肾脏;文献报道不足200例。临床表现和影像学表现不具有特异性。在此我们首次报告叙利亚的两例肾原始神经外胚层肿瘤病例。首例患者为一名26岁女性,因右侧腰痛就诊于泌尿外科门诊。腹部超声显示右肾有一个大的混合性不均匀肿块,无出血或钙化,腹部和盆腔多层螺旋CT(MSCT)显示右肾有一个边界清晰的混合性不均匀肿块,大小为(74×117)mm,遂行右根治性肾切除术。第二例患者为一名19岁男性,因左侧腰痛就诊。腹部超声显示左肾有一个混合性大肿块,边界不清。腹部和盆腔CT显示左肾有一个(30×110×90)mm的肿块以及大小为(45×28)mm的主动脉旁淋巴结肿大。该患者接受了左根治性肾切除术及主动脉旁淋巴结清扫术。根据显微镜检查和免疫组化检查,两例病例的最终诊断均为肾PNET。对于影像学图像上怀疑有肾肿块的患者,尽管肾原始神经外胚层肿瘤罕见,但仍应考虑其诊断。最终诊断需通过组织病理学研究并结合免疫组化检查来完成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2470/9577866/dfe8cfa92cba/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2470/9577866/5b6a52dd864a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2470/9577866/dfe8cfa92cba/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2470/9577866/5b6a52dd864a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2470/9577866/dfe8cfa92cba/gr2.jpg

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引用本文的文献

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Front Surg. 2023 Apr 19;10:1180107. doi: 10.3389/fsurg.2023.1180107. eCollection 2023.

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