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肾恶性副神经节瘤:1例罕见手术病例,无病生存期达3年。

Malignant paraganglioma of the kidney: a rare surgical case with 3-year disease-free survival.

作者信息

Lin Fu-Xiang, Zhao Pengpeng, Xie Le, Xu Zhan-Ping

机构信息

The Eighth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 6 Qinren Road, Foshan 528000, Guangdong, People's Republic of China.

Department of Urology, Foshan Hospital of Traditional Chinese Medicine, No. 6 Qinren Road, Foshan 528000, Guangdong, People's Republic of China.

出版信息

J Surg Case Rep. 2025 Jul 11;2025(7):rjaf494. doi: 10.1093/jscr/rjaf494. eCollection 2025 Jul.

DOI:10.1093/jscr/rjaf494
PMID:40656155
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12253953/
Abstract

A 36-year-old woman presented with abdominal pain and a left renal mass on imaging. Laboratory findings showed anemia (Hb 88 g/l), leukocytosis (12.31 × 10/l), and pyuria. Computed tomography (CT) revealed a 14 cm left renal cystic-solid mass suspicious for malignancy. Radical nephrectomy was performed, retrieving a 2600 g tumor. Histopathology demonstrated malignant paraganglioma with expansive growth confined to renal parenchyma. No adjuvant therapy was administered. At 3-year follow-up, surveillance positron emission tomography-computed tomography (PET-CT) confirmed no recurrence or metastasis. Renal paragangliomas represent <1% of such tumors, with malignant variants posing diagnostic and therapeutic challenges. This case underscores surgical resection as definitive management for localized disease and suggests favorable outcomes are achievable without adjuvant treatment in select malignant cases. Long-term surveillance remains critical.

摘要

一名36岁女性因腹痛就诊,影像学检查发现左肾有肿块。实验室检查结果显示贫血(血红蛋白88 g/l)、白细胞增多(12.31×10⁹/l)和脓尿。计算机断层扫描(CT)显示左肾有一个14 cm的囊实性肿块,怀疑为恶性。进行了根治性肾切除术,切除了一个2600 g的肿瘤。组织病理学显示为恶性副神经节瘤,呈浸润性生长,局限于肾实质。未给予辅助治疗。在3年随访中,监测正电子发射断层扫描-计算机断层扫描(PET-CT)证实无复发或转移。肾副神经节瘤占此类肿瘤的比例不到1%,恶性变体带来诊断和治疗挑战。该病例强调手术切除是局限性疾病的确定性治疗方法,并表明在某些恶性病例中,不进行辅助治疗也可取得良好结果。长期监测仍然至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/2d0a0f9fdadf/rjaf494f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/b2805ef69269/rjaf494f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/b5b39707fd33/rjaf494f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/2d0a0f9fdadf/rjaf494f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/b2805ef69269/rjaf494f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/b5b39707fd33/rjaf494f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6137/12253953/2d0a0f9fdadf/rjaf494f3.jpg

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本文引用的文献

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TNM Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma.嗜铬细胞瘤和交感神经节细胞瘤患者的TNM分期与总生存期
J Clin Endocrinol Metab. 2023 Apr 13;108(5):1132-1142. doi: 10.1210/clinem/dgac677.
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High-Specific-Activity-I-MIBG versus Lu-DOTATATE Targeted Radionuclide Therapy for Metastatic Pheochromocytoma and Paraganglioma.高比活度碘-131 间碘苄胍与镥- DOTATATE 靶向放射性核素治疗转移性嗜铬细胞瘤和副神经节瘤。
Clin Cancer Res. 2021 Jun 1;27(11):2989-2995. doi: 10.1158/1078-0432.CCR-20-3703. Epub 2021 Mar 8.
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Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension.
嗜铬细胞瘤和副神经节瘤的遗传学、诊断、治疗和未来研究方向:欧洲高血压学会内分泌高血压工作组的立场声明和共识。
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Pheochromocytoma and Paraganglioma.嗜铬细胞瘤和副神经节瘤。
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Ann Surg Oncol. 2015 Jan;22(1):256-63. doi: 10.1245/s10434-014-3965-2. Epub 2014 Oct 15.