Key result
Primary pulmonary undifferentiated pleomorphic sarcoma is an exceptionally rare, highly malignant tumor with a mean survival of 23.7 months, primarily treated with surgical resection.
Why the study?
Primary pulmonary undifferentiated pleomorphic sarcoma is exceptionally rare and lung involvement is usually due to metastasis.
Case Report (n=85)
Primary pulmonary undifferentiated pleomorphic sarcoma is an exceptionally rare, highly malignant tumor that requires extensive sampling and immunohistochemistry to exclude other lines of differentiation.
Requires comprehensive sampling and molecular testing for UPS; leaves open standardized exclusion criteria in larger series.
Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.
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Qorbani et al. (2019) conducted a case report in Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) (n=85). Surgical resection and chemotherapy was evaluated. Primary pulmonary undifferentiated pleomorphic sarcoma is an exceptionally rare, highly malignant tumor with a mean survival of 23.7 months, primarily treated with surgical resection.
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