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一例促纤维组织增生性小圆细胞肿瘤:凸显诊断挑战并强调标准化治疗方案的必要性

A Case of Desmoplastic Small Round Cell Tumor: Highlighting Diagnostic Challenges and Underscoring the Need for Standardized Treatment Protocols.

作者信息

Carpenter Sydney, Calicchia Taylor, Yoakam Marcus, Dunbar Brett

机构信息

Surgery, Kansas City University, Joplin, USA.

General Surgery, Cleveland Clinic Akron General, Akron, USA.

出版信息

Cureus. 2024 Oct 2;16(10):e70712. doi: 10.7759/cureus.70712. eCollection 2024 Oct.

Abstract

Desmoplastic small round cell tumor is a rare and aggressive neoplasm, primarily affecting serosal surfaces within the abdomen and pelvic peritoneum in young males. Because of a low reported incidence, underdiagnosis and lack of current treatment guidelines pose a unique challenge for physicians and patients dealing with this disease. Therefore, it is imperative to present new information regarding this disease so that better detection and treatment guidelines can be created. This case report presents a 30-year-old male presenting with vague abdominal symptoms, ultimately diagnosed with desmoplastic small round cell tumor after image-guided biopsy. The ambiguity of initial symptoms highlights the diagnostic challenges associated with desmoplastic small round cell tumor, further complicated by the absence of standardized treatment guidelines. This report aims to raise awareness of this exceptionally rare cancer, contribute to the existing knowledge base, and emphasize the critical need for collaborative efforts to develop standardized treatment regimens.

摘要

促纤维组织增生性小圆细胞瘤是一种罕见的侵袭性肿瘤,主要影响年轻男性的腹部浆膜表面和盆腔腹膜。由于报告的发病率较低,诊断不足以及缺乏现行治疗指南给治疗这种疾病的医生和患者带来了独特的挑战。因此,必须提供有关这种疾病的新信息,以便制定更好的检测和治疗指南。本病例报告介绍了一名30岁男性,表现为模糊的腹部症状,最终在影像引导活检后被诊断为促纤维组织增生性小圆细胞瘤。初始症状的模糊性突出了与促纤维组织增生性小圆细胞瘤相关的诊断挑战,而缺乏标准化治疗指南则使情况更加复杂。本报告旨在提高对这种极其罕见癌症的认识,为现有知识库做出贡献,并强调迫切需要共同努力制定标准化治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fe1/11530231/8a347933f696/cureus-0016-00000070712-i01.jpg

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