Inflammatory and Nested Testicular Sex Cord Tumor
\nA Novel Neoplasm With Aggressive Clinical Behavior and Frequent EWSR1::ATF1 Gene Fusions<\/p>\n
FREE LINK<\/strong> https:\/\/journals.lww.com\/ajsp\/Abstract\/2023\/04000\/Inflammatory_and_Nested_Testicular_Sex_Cord_Tumor_.10.aspx<\/p>\n Acosta, Andres M. MD*; Bridge, Julia A. MD†,‡; Dal Cin, Paola S. PhD*; Sholl, Lynette M. MD*; Cornejo, Précis<\/u><\/p>\n The authors describe clinicopathologic and molecular characteristics of a series of 13 malignant Comment<\/u> Dr. Ulbright responded: Inflammatory and Nested Testicular Sex Cord Tumor A Novel Neoplasm With Aggressive Clinical Behavior and Frequent EWSR1::ATF1 Gene Fusions FREE LINK https:\/\/journals.lww.com\/ajsp\/Abstract\/2023\/04000\/Inflammatory_and_Nested_Testicular_Sex_Cord_Tumor_.10.aspx Acosta, Andres M. MD*; Bridge, Julia A. MD†,‡; Dal Cin, Paola S. PhD*; Sholl, Lynette M. MD*; Cornejo, Kristine M. MD§; Fletcher, Christopher D.M. MD*; Ulbright, Thomas M. MD∥ The American Journal of … Continue reading SECOND QUARTER OF 2023<\/span>
\nKristine M. MD§; Fletcher, Christopher D.M. MD*; Ulbright, Thomas M. MD∥
\nThe American Journal of Surgical Pathology 47(4):p 504-517, April 2023.<\/p>\n
\ntesticular sex cord tumors that show features of the distinctive neoplasm newly described in this article.
\nMorphologically the tumors were showing overlapping features with seminoma and Sertoli cell tumor
\nwith mixed inflammatory background and were lacking beta-catenin nuclear immunopositivity.
\nEWSR1::ATF1 fusions were found in 10 of 13 cases and ATF1 amplification was found in one of the 3
\ncases negative for EWSR1::ATF1 fusion. Clinical follow-up data show that all 11 patients with available
\ndata developed metastases.<\/p>\n
\nTesticular sex cord-stromal tumors (TSCST) are relatively rare neoplasms, that commonly present a
\ndiagnostic challenge due to their rarity and overlapping morphologic features. It is difficult to predict the aggressive clinical behavior of such tumors. Currently, there are very limited effective systemic therapy modalities and reliable biomarkers for malignant TSCST. Therefore, with the advances and availability of molecular pathology techniques, it became possible to further define the classification of TSCST. The hallmark finding of EWSR1::ATF1 fusion described in this timely article will be helpful to the broad audience of both subspecialized GU pathologists, soft tissue pathologists, and pathologists in general practice as break-apart FISH tests are widely available. Not only this will aid in diagnosing and
\nprognosticating this newly described variant of TSCST but also expands the differential diagnostic
\nspectrum of tumors with EWSR1 and ATF1 rearrangements in other locations to include the possibility of
\nmetastatic inflammatory and nested TSCST when the EWSR1 rearrangement is detected. More studies
\nare needed to further define this entity as the number of cases is relatively small. Whether EWSR1::ATF1
\nmutation can be present in other less aggressive TSCST types and whether all inflammatory and nested
\nTSCST show malignant behavior are questions that remain to be answered in further studies.
\nReviewed by: Oleksandr Kravtsov, MD<\/p>\n
\n“Many of the tumors resemble seminoma because they form sheets and nests of pale cells with inflammatory infiltrates. The cytology is, however, different, and the lack of GCNIS and seminoma IHC allow for distinction. I think that many cases of IN-TSCT may provoke consideration of Sertoli cell tumor, NOS (SCT-NOS). Helpful clues to IN-TSCT instead of SCT-NOS include lack of nuclear beta-catenin, more than focal inhibin and\/or calretinin expression, and absence of distinct tubules\/glands. Also, a subset of the cases shows CD30 expression, which was something uncovered because the cases were consults and the contributors had done CD30, with the positivity causing them some confusion regarding embryonal carcinoma. So, CD30 reactivity may be helpful in supporting IN-TSCT. I also agree we do not know the true biologic potential of these neoplasms because of the consultative nature of the series. A number of patients were referred because of malignant behavior, so the outcomes of the patients suffer from referral bias. We need more cases with follow-up.<\/p>\n<\/body><\/html>\n","protected":false},"excerpt":{"rendered":"