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系统性硬化症患者中的一种罕见肺部恶性肿瘤

A Rare Lung Malignancy in a Case of Systemic Sclerosis.

作者信息

C Syed Akram, P Harshavardhini, Jayanthi Nalini

机构信息

Department of Respiratory Medicine, SRM Medical College Hospital and Research Centre, Chennai, IND.

出版信息

Cureus. 2024 Jul 22;16(7):e65146. doi: 10.7759/cureus.65146. eCollection 2024 Jul.

Abstract

Systemic sclerosis (SSc) is one of the chronic autoimmune diseases characterized by the infiltration of excess collagen in various organs, especially the skin. It is found to be associated with a higher prevalence of internal malignancies, particularly lung carcinoma. Herein, we report a case of adenosquamous carcinoma confining within the lung in a patient who had long-standing SSc. She was a 55-year-old female patient presenting with progressive dry cough and breathlessness for six months. She had been a known case of diffuse cutaneous SSc for over a decade, based on 2013 American College of Rheumatology (ACR) criteria. The diagnosis is made based on her findings of bilateral thickening of the fingers on both hands, extending up to the metacarpophalangeal joints. Furthermore, she had telangiectasia at the upper chest wall and neck, multiple pitting scars at the toes, Raynaud's esophageal dilatation, and interstitial lung disease (ILD). She had been treated on Mycophenolate Mofetil 500 mg twice daily and low-dose prednisolone 5 mg once daily for 10 years. The patient's high-resolution computed tomography (HRCT) of the chest revealed a subpleural nodule in the posterior basal segment of the left lower lobe with areas of reticular opacities and interlobular septal thickening on bilateral lung fields six months earlier. The current computed tomography of the lung revealed a new 2.6 x 2.5 cm ill-defined lesion with irregular margins at the left lower lobe. A CT-guided biopsy was done for the lesion, which revealed adenosquamous carcinoma. Immunohistochemistry was consistent with a diagnosis of primary pulmonary adenosquamous carcinoma. The patient did not accept any further investigations and/or treatment. Herein, we present a rare lung malignancy, adenosquamous carcinoma of the lung with an underlying long-term diffuse cutaneous SSc in a nonsmoking female, which highlights the importance of lung cancer screening in individuals with SSc complicated with ILD and supports the fact that there is an increased prevalence of lung cancer among SSc-ILD patients than that of the regular population.

摘要

系统性硬化症(SSc)是一种慢性自身免疫性疾病,其特征是各种器官尤其是皮肤中胶原蛋白过度浸润。研究发现它与内部恶性肿瘤的较高患病率相关,尤其是肺癌。在此,我们报告一例长期患有SSc的患者,其肺内局限发生腺鳞癌。她是一名55岁女性患者,出现进行性干咳和气短6个月。根据2013年美国风湿病学会(ACR)标准,她已知患有弥漫性皮肤型SSc超过十年。诊断依据是她双手手指双侧增厚,延伸至掌指关节。此外,她上胸壁和颈部有毛细血管扩张,脚趾有多个凹陷性瘢痕,雷诺现象,食管扩张,以及间质性肺疾病(ILD)。她接受霉酚酸酯500毫克每日两次和低剂量泼尼松龙5毫克每日一次治疗已有10年。患者6个月前的胸部高分辨率计算机断层扫描(HRCT)显示左下叶后基底段有一个胸膜下结节,双侧肺野有网状混浊和小叶间隔增厚区域。当前的肺部计算机断层扫描显示左下叶有一个新的2.6×2.5厘米边界不清、边缘不规则的病变。对该病变进行了CT引导下活检,结果显示为腺鳞癌。免疫组织化学结果与原发性肺腺鳞癌的诊断一致。患者未接受任何进一步检查和/或治疗。在此,我们展示了一例罕见的肺恶性肿瘤,即一名不吸烟女性患有的肺腺鳞癌合并潜在的长期弥漫性皮肤型SSc,这凸显了对合并ILD的SSc患者进行肺癌筛查的重要性,并支持了SSc-ILD患者中肺癌患病率高于普通人群这一事实。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c39/11341069/7b41c4f35ad1/cureus-0016-00000065146-i01.jpg

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