VATS trisegmentectomy successfully resected a rare 2.4 x 1.9 cm primary pulmonary undifferentiated pleomorphic sarcoma in an asymptomatic 72-year-old man.
Case Report (n=1)
This case highlights the rare occurrence of primary pulmonary undifferentiated pleomorphic sarcoma and the importance of complete surgical resection for diagnosis and treatment.
Abstract Introduction Primary sarcomas of the lung are extremely uncommon, accounting for less than half a percent of all lung cancers. Among them, primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) is one of the rarest types, with fewer than 85 cases reported. Since undifferentiated pleomorphic sarcoma (UPS) usually arises in the extremities or retroperitoneum, finding it in the lung is unexpected, and it is almost always metastatic. Case Discussion A 72-year-old man with a 65-pack-year smoking history, who quit 6 years prior to presentation, was referred to the lung cancer screening clinic after the most recent CT chest showed a new 2.4 × 1.9 cm solid nodule in the left upper lobe. Earlier CT scans showed no significant abnormality. He was asymptomatic and denied cough, weight loss, or chest pain.A transbronchial forceps biopsy and transbronchial needle aspiration as well as staging endobronchial ultrasound with transbronchial needle aspiration of mediastinal and hilar lymph nodes were done. Pathology showed spindle cell proliferation with fibrosis, favoring a schwannoma, and benign lymph nodes. Because of the suspicious growth pattern, a VATS trisegmentectomy was performed. Intraoperatively, a lobulated lesion was found near the hilum. Frozen section showed a spindle cell tumor with negative margins. Final pathology revealed a malignant spindle cell neoplasm, weakly positive for PRAME and negative for keratin, HMB45, Melan-A, S100, and SOX10. Differential diagnoses included sarcomatoid carcinoma, sarcoma, and metastatic melanoma.Final pathology after consultation with subspecialist was PPUPS. PET-CT showed no evidence of disease elsewhere. Patient opted against further systemic therapy and remains on surveillance. Discussion UPS is among the least common types of primary pulmonary sarcomas. Most patients are men between their 30s and 80s and usually present with cough, chest pain, hemoptysis, or shortness of breath. Some are completely asymptomatic and diagnosed incidentally on imaging.Given that UPS is most frequently metastatic, confirming a primary pulmonary origin demands extensive workup to rule out extrapulmonary sites. There are no established treatment guidelines. Complete surgical resection remains the best option when feasible, as it offers the best long-term outcome. Chemotherapy and radiation have unclear benefit due to limited data.This case adds to the small number of reported PPUPS and highlights the need to keep rare mesenchymal tumors in mind when evaluating solitary pulmonary nodules. Early diagnosis and complete resection are key to improving outcomes in this rare lung malignancy. This abstract is funded by: None
Phirtskhalava et al. (2026) conducted a case report in Primary pulmonary undifferentiated pleomorphic sarcoma (n=1). VATS trisegmentectomy was evaluated. VATS trisegmentectomy successfully resected a rare 2.4 x 1.9 cm primary pulmonary undifferentiated pleomorphic sarcoma in an asymptomatic 72-year-old man.