Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA.
John Sealy School of Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Am J Case Rep. 2024 Aug 7;25:e944582. doi: 10.12659/AJCR.944582.
BACKGROUND Amyloidosis refers to an assortment of diseases characterized by the accumulation and deposition of misfolded proteins in the extracellular matrix of tissues and organs. It may present systemically, affecting multiple organs, or locally by affecting a single organ. When the lungs or mediastinal structures are involved, the term pulmonary amyloid is used. Sole pulmonary involvement with amyloid is extremely rare. There is no definitive treatment for this disease, but proposed treatment options include surgery, cytotoxic medications, and external beam radiation therapy (EBRT). CASE REPORT A 68-year-old man with a left apical lung mass presented with subacute shortness of breath. Comprehensive evaluation of the patient's symptoms and findings, including infectious and oncologic evaluation, were performed. Infectious evaluation revealed positive acid-fast bacilli sputum cultures with Mycobacterium chimerea intracellulare. Biopsy of the mass revealed a Lambda restricted amyloidoma, which is usually seen in lymphoproliferative diseases and disorders. Bone marrow biopsy did not reveal any monoclonal cell lines or neoplasms. Abdominal fat pad biopsy was performed to rule out systemic amyloid and the results were negative. The diagnosis of isolated apical pulmonary amyloidoma was made. EBRT was performed over 12 fractions in 24 mGy, with improvement in the patient's symptoms. CONCLUSIONS The diagnosis of pulmonary amyloid necessitates comprehensive evaluation. There is no specific treatment for pulmonary amyloid; however, there has been success with surgical intervention, cytotoxic medications, and EBRT. Successful treatment of the amyloidoma is based on its anatomic location. We suggest EBRT in fractionated doses for optimal treatment of rare isolated apical pulmonary amyloidoma.
淀粉样变性是指一组疾病，其特征是组织和器官细胞外基质中错误折叠的蛋白质的积累和沉积。它可能全身性地发生，影响多个器官，也可能局部性地影响单个器官。当肺部或纵隔结构受到影响时，使用术语“肺部淀粉样变性”。仅肺部受累伴淀粉样变性极为罕见。目前尚无针对该疾病的明确治疗方法，但提出的治疗选择包括手术、细胞毒性药物和外照射放射治疗（EBRT）。
一名 68 岁男性，因左肺尖部肿块伴有亚急性呼吸急促而就诊。对患者的症状和检查结果进行了全面评估，包括感染和肿瘤学评估。感染评估显示分枝杆菌属细胞内的阳性抗酸杆菌痰培养。肿块活检显示 Lambda 限制型淀粉样瘤，通常见于淋巴增生性疾病和紊乱。骨髓活检未发现任何单克隆细胞系或肿瘤。进行了腹部脂肪垫活检以排除系统性淀粉样变性，结果为阴性。诊断为孤立性肺尖部淀粉样变性瘤。行 12 次 24 mGy 的 EBRT，患者症状改善。
肺部淀粉样变性的诊断需要全面评估。肺部淀粉样变性没有特异性治疗方法；然而，手术干预、细胞毒性药物和 EBRT 已取得成功。淀粉样瘤的成功治疗基于其解剖位置。我们建议对罕见的孤立性肺尖部淀粉样变性瘤进行分次 EBRT，以获得最佳治疗效果。
