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肝脏的罕见入侵者:一例未分化多形性肉瘤

A Rare Intruder of the Liver: A Case of Undifferentiated Pleomorphic Sarcoma.

作者信息

Pradeep Sidart, Thomas Devine, Cain Daniel, Modi Apurva, Gupta Rohan, Gautam Shovendra

机构信息

Internal Medicine, Baylor Scott & White All Saints Medical Center, Fort Worth, USA.

Transplant Hepatology, Baylor Annette C. and Harold C. Simmons Transplant Institute, Baylor Scott & White All Saints Medical Center, Fort Worth, USA.

出版信息

Cureus. 2025 Mar 10;17(3):e80333. doi: 10.7759/cureus.80333. eCollection 2025 Mar.

DOI:10.7759/cureus.80333
PMID:40206937
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11980306/
Abstract

Undifferentiated pleomorphic sarcoma (UDS) is a rare and aggressive form of cancer that poses significant diagnostic and therapeutic challenges. The most common sites of this malignancy include the extremities. It is less common in visceral organs and the liver has rarely been reported as a primary site of disease. This subset of cancer is characterized by anaplastic and highly heterogeneous cells that lack specific markers of differentiation, making it difficult to identify and treat effectively. Due to vague symptoms that mirror other pathologies of the affected organ(s), UDS can present like various sarcomas or metastatic processes. A thorough microscopic analysis with the use of ancillary testing including immunohistochemistry can aid in the identification of UDS, as clinical symptoms can be non-specific. It is important to characterize primary malignant lesions since treatment options can range from medical therapy to surgical intervention. We present a unique case of UDS of the liver in a patient with obstructive jaundice.

摘要

未分化多形性肉瘤(UDS)是一种罕见且侵袭性强的癌症形式,带来了重大的诊断和治疗挑战。这种恶性肿瘤最常见的发病部位包括四肢。在内脏器官中较少见,而肝脏作为疾病的原发部位鲜有报道。这种癌症亚型的特征是细胞间变且高度异质性,缺乏特定的分化标志物,这使得其难以有效识别和治疗。由于症状模糊,与受影响器官的其他病变相似,UDS可能表现得像各种肉瘤或转移过程。通过使用包括免疫组织化学在内的辅助检测进行全面的显微镜分析,有助于识别UDS,因为临床症状可能不具有特异性。明确原发性恶性病变很重要,因为治疗方案可以从药物治疗到手术干预不等。我们报告了一例患有梗阻性黄疸的患者发生肝脏未分化多形性肉瘤的独特病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63c0/11980306/6b11a8d9354d/cureus-0017-00000080333-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63c0/11980306/e4e03e61db68/cureus-0017-00000080333-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63c0/11980306/6b11a8d9354d/cureus-0017-00000080333-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63c0/11980306/e4e03e61db68/cureus-0017-00000080333-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63c0/11980306/6b11a8d9354d/cureus-0017-00000080333-i02.jpg

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本文引用的文献

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Case report: Undifferentiated sarcoma with multiple tumors involved in Lynch syndrome: Unexpected favorable outcome to sintilimab combined with chemotherapy.病例报告:林奇综合征伴多发肿瘤的未分化肉瘤:信迪利单抗联合化疗取得意外良好疗效
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高危软组织肉瘤的新辅助化疗:意大利(ISG)、西班牙(GEIS)、法国(FSG)和波兰(PSG)肉瘤组随机试验的最终结果。
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The PTEN Tumor Suppressor Gene in Soft Tissue Sarcoma.软组织肉瘤中的PTEN肿瘤抑制基因
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Subclassification of pleomorphic sarcomas: How and why should we care?多形性肉瘤的亚分类:我们为何要关注以及如何关注?
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Rationale and emerging strategies for immune checkpoint blockade in soft tissue sarcoma.软组织肉瘤免疫检查点阻断的原理和新兴策略。
Cancer. 2018 Oct 1;124(19):3819-3829. doi: 10.1002/cncr.31517. Epub 2018 May 3.
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