Primary Undifferentiated Pleomorphic Sarcoma (UPS) of the Lung: Review

Primary Undifferentiated Pleomorphic Sarcoma (UPS) of the lung is an exceptionally rare malignancy, accounting for less than 0.2% of all lung tumors. This disease has posed considerable diagnostic and therapeutic challenges. This review delves into the complexity of primary lung UPS, encompassing diagnostic intricacies, therapeutic modalities, and prognosis.

Formerly known as Malignant Fibrous Histiocytoma (MFH) until its reclassification in 2012, UPS's diagnostic journey often relies on the exclusion of other well-characterized soft-tissue sarcomas. Our exploration underscores the significance of early disease recognition, emphasizing factors that influence recurrence and metastasis risk, such as surgical margin adequacy and tumor size.

Treatment strategies encompass a multidisciplinary approach, involving aggressive en bloc surgical excision with microscopically negative margins, complemented by adjuvant therapies like radiotherapy or chemotherapy. Recent studies offer valuable insights into refining these strategies, striving to enhance the overall prognosis for UPS patients. Survival rates for primary lung UPS remain modest, prompting ongoing investigations into novel frontiers, notably immunotherapy, in the quest for improved therapeutic outcomes.

This review not only sheds light on the rare and intricate nature of primary lung UPS but also amalgamates data, clinical experiences, and emerging research, this review contributes to a deeper understanding of UPS in the lung, and further advancements in diagnosis and treatment.