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原发性腹膜后精原细胞瘤——一种具有重要意义的罕见表现

Primary Retroperitoneal Seminoma-An Uncommon Presentation With Significant Implications.

作者信息

Sain S, Tripathi S, Bakshi N, Das S A P, Nundy S

机构信息

Institute of Surgical Gastroenterology, GI and HPB Oncosurgery and Liver Transplant, Sir Ganga Ram Hospital, New Delhi, India.

Department of Pathology (Histopathology Division), Sir Ganga Ram Hospital, New Delhi, India.

出版信息

Case Rep Surg. 2024 Dec 18;2024:4376859. doi: 10.1155/cris/4376859. eCollection 2024.

DOI:10.1155/cris/4376859
PMID:39723237
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11669426/
Abstract

Primary retroperitoneal seminoma is an exceedingly rare type of germ cell tumor, accounting for less than 5% of all such tumors. These tumors are typically large at presentation due to their slow growth and the nonspecific nature of symptoms, which often leads to delayed diagnosis. A 40-year-old male presented with intermittent abdominal pain and a palpable lump in the right paraumbilical region. Ultrasonography revealed a large retroperitoneal mass. Fine needle aspiration cytology confirmed the diagnosis of poorly differentiated malignant tumor, for which he was evaluated with CT-angiogram of the abdomen and FDG PET-CT scans, which showed a large retroperitoneal mass. The patient, then, had a surgical resection of the mass, with postoperative histopathological and immunohistochemical diagnosis of primary retroperitoneal seminoma, and then underwent three cycles of BEP chemotherapy. Scrotal ultrasonography showed no testicular abnormalities, obviating the need for orchiectomy. FDG PET showed a complete response following treatment completion. Postoperative management included routine monitoring of tumor markers and follow-up imaging, which showed a complete response. This case highlights the diagnostic and therapeutic challenges of primary retroperitoneal seminoma. A multidisciplinary approach, including accurate histopathological diagnosis and a combination of chemotherapy and surgery, is essential for optimal management. Early diagnosis and tailored treatment strategies significantly improve patient outcomes.

摘要

原发性腹膜后精原细胞瘤是一种极其罕见的生殖细胞肿瘤,占所有此类肿瘤的比例不到5%。由于其生长缓慢且症状不具特异性,这些肿瘤在出现时通常较大,这往往导致诊断延迟。一名40岁男性出现间歇性腹痛,右脐旁区域可触及肿块。超声检查发现一个巨大的腹膜后肿块。细针穿刺细胞学检查确诊为低分化恶性肿瘤,为此对他进行了腹部CT血管造影和FDG PET-CT扫描评估,结果显示有一个巨大的腹膜后肿块。随后,患者接受了肿块的手术切除,术后经组织病理学和免疫组化诊断为原发性腹膜后精原细胞瘤,然后接受了三个周期的BEP化疗。阴囊超声检查未发现睾丸异常,无需进行睾丸切除术。治疗完成后,FDG PET显示完全缓解。术后管理包括常规监测肿瘤标志物和进行随访影像学检查,结果显示完全缓解。 本病例突出了原发性腹膜后精原细胞瘤的诊断和治疗挑战。多学科方法,包括准确的组织病理学诊断以及化疗与手术相结合,对于优化管理至关重要。早期诊断和量身定制的治疗策略可显著改善患者预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/7d8ced8cc0fc/CRIS2024-4376859.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/ce71cf80d533/CRIS2024-4376859.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/9772b33389ef/CRIS2024-4376859.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/d6f459d7dd6a/CRIS2024-4376859.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/7d8ced8cc0fc/CRIS2024-4376859.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/ce71cf80d533/CRIS2024-4376859.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/9772b33389ef/CRIS2024-4376859.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/d6f459d7dd6a/CRIS2024-4376859.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c76/11669426/7d8ced8cc0fc/CRIS2024-4376859.004.jpg

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本文引用的文献

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原发性腹膜后精原细胞瘤——胚胎学、组织病理学及治疗特点
Rom J Morphol Embryol. 2016;57(3):1045-1050.
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