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Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”
Sarcoma Service, Department of Medical Oncology, Peter Mac Callum Cancer Centre, Melbourne, Victoria, VIC8006, Australia
Department of Medical Oncology, St. Vincent's University Hospital, Dublin 4, Ireland
St. Andrea University Hospital, Rome 000189, Italy
* Author to whom correspondence should be addressed.
Received: 19 December 2012; in revised form: 26 January 2013 / Accepted: 17 February 2013 / Published: 22 February 2013
Abstract: Undifferentiated pleomorphic sarcoma (UPS) is an inclusive term used for sarcomas that defy formal sub-classification. The frequency with which this diagnosis is assigned has decreased in the last twenty years. This is because when implemented, careful histologic assessment, immunohistochemistry, and ultra-structural evaluation can often determine lineage of differentiation. Further attrition in the diagnostic frequency of UPS may arise by using array-comparative genomic hybridization. Gene expression arrays are also of potential use as they permit hierarchical gene clustering. Appraisal of the literature is difficult due to a historical perspective in which specific molecular diagnostic methods were previously unavailable. The American Joint Committee on Cancer (AJCC) classification has changed with different inclusion criteria. Taxonomy challenges also exist with the older term “malignant fibrous histiocytoma” being replaced by “UPS”. In 2010 an analysis of multiple sarcoma expression databases using a 170-gene predictor, re-classified most MFH and “not-otherwise-specified” (NOS) tumors as liposarcomas, leiomyosarcomas or fibrosarcomas. Interestingly, some of the classifier genes are potential molecular therapeutic targets including Insulin-like growth factor 1 (IGF-1), Peroxisome proliferator-activated receptor γ (PPARγ), Nerve growth factor β (NGF β) and Fibroblast growth factor receptor (FGFR).
Keywords: undifferentiated pleomorphic sarcoma; malignant fibrous histiocytoma; gene expression arrays; lineage differentiation
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MDPI and ACS Style
Kelleher, F.C.; Viterbo, A. Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”. Cancers 2013, 5, 218-233.
Kelleher FC, Viterbo A. Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”. Cancers. 2013; 5(1):218-233.
Kelleher, Fergal C.; Viterbo, Antonella. 2013. "Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”." Cancers 5, no. 1: 218-233.