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.