Primary Pulmonary Leiomyosarcoma: A Case of Prolonged Survival With Radiotherapy and Pazopanib in a 90-Year-Old Female.

IF 0.8 Q4 RESPIRATORY SYSTEM
Respirology Case Reports Pub Date : 2025-07-28 eCollection Date: 2025-07-01 DOI:10.1002/rcr2.70301
Sathish Krishnan, Vijaya Sivalingam Ramalingam, Chandrika Patel, Jennifer Heisick
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引用次数: 0

Abstract

Primary pulmonary leiomyosarcoma is an exceedingly rare malignancy, comprising less than 0.5% of all lung cancers. Due to its rarity, no standardised treatment guidelines exist. Prognosis is especially poor in cases which are deemed unresectable. We describe the case of a 90-year-old woman who presented with progressive dyspnea and cough over 1 month. Chest computed tomography revealed a large right hilar mass extending into the right upper and middle lobes, abutting the mediastinum and pericardium. Histopathological analysis confirmed primary pulmonary leiomyosarcoma. Given her advanced age and comorbidities, she was not a candidate for surgical intervention. Molecular profiling demonstrated PDL1 expression < 1% and no targetable mutations, ruling out the option of immunotherapy. She was managed with palliative radiotherapy followed by pazopanib therapy. Serial imaging demonstrated disease control, with an extended survival of approximately 5 years. Despite a median reported survival of 14 months, our patient achieved prolonged survival, highlighting the importance of an individualised therapeutic approach for elderly patients with rare malignancies.

Abstract Image

Abstract Image

Abstract Image

原发性肺平滑肌肉瘤:一个90岁女性放疗和帕唑帕尼延长生存的案例。
原发性肺平滑肌肉瘤是一种极为罕见的恶性肿瘤,占所有肺癌的不到0.5%。由于罕见,没有标准化的治疗指南。在被认为不能切除的病例中,预后尤其差。我们描述的情况下,一个90岁的妇女谁提出了进行性呼吸困难和咳嗽超过1个月。胸部计算机断层扫描显示一个大的右肺门肿块,延伸到右上叶和中叶,毗邻纵隔和心包膜。组织病理学分析证实为原发性肺平滑肌肉瘤。考虑到她的高龄和合并症,她不适合手术治疗。分子分析显示PDL1表达
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来源期刊
Respirology Case Reports
Respirology Case Reports RESPIRATORY SYSTEM-
CiteScore
1.40
自引率
0.00%
发文量
178
审稿时长
8 weeks
期刊介绍: Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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