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肺肉瘤样癌:一例罕见病例报告、诊断困境及文献复习。

Pulmonary sarcomatoid carcinoma: A rare case report, diagnostic dilemma and review of literature.

机构信息

Department of Hand and Foot Surgery, China-Japan Union Hospital of Jilin University, Changchun, China.

Department of Rehabilitation, The Second Hospital of Jilin University, Changchun, China.

出版信息

Medicine (Baltimore). 2024 Jul 5;103(27):e38797. doi: 10.1097/MD.0000000000038797.

DOI:10.1097/MD.0000000000038797
PMID:38968487
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11224848/
Abstract

RATIONALE

Pulmonary sarcomatoid carcinoma (PSC), a rare tumor, comprises 0.1% to 0.4% of all malignant lung tumors. Given the rarity of PSC, its clinical course, therapeutic guidelines, and patient outcomes remain largely unknown. Therefore, it is imperative to alert clinicians to this extremely rare and instructive early-onset cancer.

PATIENT CONCERNS

This report describes a 28-year-old woman with PSC, who was initially misdiagnosed with Whipple's disease. A conclusive diagnosis of PSC was made following careful clinical examination, imaging, and histopathological evaluation of the patient's biopsy sample. Radiological imaging revealed multiple nodules and mass formations in the left upper lobe of the patient's lung, with the largest measuring of 5.4 × 3.2 cm.

DIAGNOSIS

Histopathological examination indicated the presence of a malignant neoplasm associated with necrosis suggestive of sarcoma, which was pathologically staged as cT4N1M1.

INTERVENTIONS AND OUTCOMES

A regimen of doxorubicin and ifosfamide was administered therapeutically, resulting in a stable disease state.

LESSONS

The rarity and tumor origin challenge the diagnosis, which emphasizes the imperative role of histological examination, immunohistochemistry, and flow cytometry in achieving an accurate diagnosis. This report summarizes the existing publications to provide a comprehensive overview of PSC, including its clinical manifestations, radiographic imaging, pathologic features, diagnostic challenges, treatment strategies, and prognosis, and aims to improve the understanding of PSC.

摘要

背景

肺肉瘤样癌(PSC)是一种罕见的肿瘤,占所有恶性肺肿瘤的 0.1%至 0.4%。由于 PSC 的罕见性,其临床过程、治疗指南和患者预后在很大程度上仍然未知。因此,提醒临床医生注意这种极其罕见但具有启发性的早发性癌症是至关重要的。

患者关注

本报告描述了一例 28 岁女性患有 PSC,最初误诊为惠普尔病。通过对患者活检样本的仔细临床检查、影像学和组织病理学评估,最终明确诊断为 PSC。影像学检查显示患者左肺上叶有多个结节和肿块形成,最大的结节大小为 5.4×3.2cm。

诊断

组织病理学检查提示存在与肉瘤相关的恶性肿瘤,伴有坏死,病理分期为 cT4N1M1。

干预和结果

给予多柔比星和异环磷酰胺治疗,病情稳定。

教训

PSC 的罕见性和肿瘤起源对诊断构成挑战,这强调了组织学检查、免疫组织化学和流式细胞术在获得准确诊断中的重要作用。本报告总结了现有的文献,提供了 PSC 的全面概述,包括其临床表现、影像学表现、病理特征、诊断挑战、治疗策略和预后,并旨在提高对 PSC 的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/6ed50fc1666b/medi-103-e38797-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/0e1e5ba6b1ee/medi-103-e38797-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/6db0b23c9da0/medi-103-e38797-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/6ed50fc1666b/medi-103-e38797-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/0e1e5ba6b1ee/medi-103-e38797-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/6db0b23c9da0/medi-103-e38797-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80ef/11224848/6ed50fc1666b/medi-103-e38797-g003.jpg

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Rapidly Progressive Lung Sarcomatoid Carcinoma Managed with Doxorubicin Plus Ifosfamide and Pemetrexed.
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Case Rep Oncol. 2021 Nov 25;14(3):1677-1681. doi: 10.1159/000520190. eCollection 2021 Sep-Dec.
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