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Many of the associated benign and malignant lesions occur almost exclusively in DICER1 syndrome. One such tumour, pituitary blastoma (pitB), overexpresses <jats:italic>PRAME<\/jats:italic> 500x above control levels. PRAME (PReferentially expressed Antigen in MElanoma) is expressed in malignancies that are not DICER1\u2010related (e.g. melanoma). To address whether PRAME expression is part of the DICER1 phenotype, or simply a feature of pitB, a series of 75 <jats:italic>DICER1<\/jats:italic>\u2010mutated specimens and 33 non\u2010mutated specimens was surveyed using immunohistochemistry for PRAME, together with EZH2, which complexes with PRAME. In <jats:italic>DICER1<\/jats:italic>\u2010mutated specimens, positive staining for PRAME was only seen in malignant tumours; 7 of 11 histological types and 34\/62 individual tumours were positive, while non\u2010tumourous lesions were always negative. Pleuropulmonary blastoma (PPB) showed a continuum in staining, with type I lesions being PRAME negative (<jats:italic>n<\/jats:italic>\u00a0=\u00a07) but all type II and type III lesions PRAME positive (<jats:italic>n<\/jats:italic>\u00a0=\u00a07). Similarly, cystic nephroma (CN) was negative (<jats:italic>n<\/jats:italic>\u00a0=\u00a08), with anaplastic sarcoma of the kidney being positive (<jats:italic>n<\/jats:italic>\u00a0=\u00a02). However, one atypical CN with mesenchymal cell proliferation was PRAME\u2010positive. Embryonal rhabdomyosarcoma (RMS) with <jats:italic>DICER1<\/jats:italic> pathogenic variants (PVs) was positive for PRAME (5\/6), but the same tumour type without <jats:italic>DICER1<\/jats:italic> PVs was also positive (9\/15). Staining for EZH2 corresponded to that seen with PRAME, validating the latter. This study leads us to conclude that (1) PRAME expression occurs in two\u2010thirds of DICER1\u2010related malignancies; (2) PRAME may be a marker for the progression that certain DICER1\u2010related lesions are thought to undergo, such as PPB and CN; and (3) PRAME expression in some tumours, such as RMS, appears to be an intrinsic feature of the tumour, rather than specifically related to <jats:italic>DICER1<\/jats:italic> PVs. Therapy directed against PRAME may offer novel treatment options in patients with the DICER1 syndrome.<\/jats:p>","DOI":"10.1002\/cjp2.264","type":"journal-article","created":{"date-parts":[[2022,3,16]],"date-time":"2022-03-16T23:00:34Z","timestamp":1647471634000},"page":"294-304","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["<scp>PRAME<\/scp> protein expression in <scp>DICER1<\/scp>\u2010related tumours"],"prefix":"10.1002","volume":"8","author":[{"given":"Paul S","family":"Thorner","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne\u2010Sophie","family":"Chong","sequence":"additional","affiliation":[{"name":"Department of Human Genetics McGill University  Montreal QC Canada"},{"name":"Cancer Axis Lady Davis Institute for Medical Research, Jewish General Hospital  Montreal QC Canada"},{"name":"Cancer Research Program Research Institute of the McGill University Health Centre  Montreal QC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javad","family":"Nadaf","sequence":"additional","affiliation":[{"name":"Department of Human Genetics McGill University  Montreal QC Canada"},{"name":"Cancer Axis Lady Davis Institute for Medical Research, Jewish General Hospital  Montreal QC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naciba","family":"Benlimame","sequence":"additional","affiliation":[{"name":"Research Pathology Facility Lady Davis Institute, Jewish General Hospital  Montreal QC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Marrano","sequence":"additional","affiliation":[{"name":"Division of Pathology Hospital for Sick Children  Toronto ON 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