{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T18:21:51Z","timestamp":1790446911085,"version":"4.1.0"},"reference-count":19,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2021,8,16]],"date-time":"2021-08-16T00:00:00Z","timestamp":1629072000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A 2\u2010year\u2010old boy presented with a large cystic and solid chest mass arising from the lung, radiographically consistent with pleuropulmonary blastoma (PPB). He underwent right lower lobectomy with resection of a well\u2010circumscribed, mixed solid and cystic mass. The solid areas were composed of cords and nests of tumor cells in the myxoid stroma and retiform foci whose pathologic and immunophenotypic findings were consistent with a sex cord\u2010stromal tumor with features of a Sertoli\u2013Leydig cell tumor. Tumor testing showed a pathogenic variant in the <jats:italic>DICER1<\/jats:italic> RNase IIIb hotspot domain. Family history was suggestive of <jats:italic>DICER1<\/jats:italic> germline pathogenic <jats:italic>DICER1<\/jats:italic> variation in absence of a detectable germline variant. He received 12 cycles of chemotherapy with ifosfamide, vincristine, dactinomycin and doxorubicin (IVADo) and surgery with complete response. One year after completion of chemotherapy, imaging studies showed concern for recurrence confirmed by thorascopic biopsy of a pleural\u2010based mass. He is currently receiving cisplatin\u2010based chemotherapy with reduction in tumor size. Review of the literature showed no similar cases; however, review of our pathology files revealed a single similar case of anterior mediastinal Sertoli cell tumor in a 3\u2010year\u2010old girl.<\/jats:p>","DOI":"10.1002\/pbc.29284","type":"journal-article","created":{"date-parts":[[2021,8,16]],"date-time":"2021-08-16T14:28:50Z","timestamp":1629124130000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Thoracic Sertoli\u2013Leydig cell tumor: An alternative type of pleuropulmonary blastoma associated with <i>DICER1<\/i> variation"],"prefix":"10.1002","volume":"68","author":[{"given":"William","family":"Terry","sequence":"first","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Stead Family Children's Hospital  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erica M.","family":"Carlisle","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Stead Family Children's Hospital  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paige","family":"Mallinger","sequence":"additional","affiliation":[{"name":"International PPB\/DICER1 Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1611-4577","authenticated-orcid":false,"given":"Alexander T.","family":"Nelson","sequence":"additional","affiliation":[{"name":"International PPB\/DICER1 Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Gordon","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Stead Family Children's Hospital  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7990-1802","authenticated-orcid":false,"given":"Yoav H.","family":"Messinger","sequence":"additional","affiliation":[{"name":"International PPB\/DICER1 Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Field","sequence":"additional","affiliation":[{"name":"ResourcePath  Arlington Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis P.","family":"Dehner","sequence":"additional","affiliation":[{"name":"Lauren V. Ackerman Laboratory of Surgical Pathology, Department of Pathology and Immunology Washington University Medical Center  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Ashley","family":"Hill","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Children's National Medical Center  Washington District of Columbia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1788-5832","authenticated-orcid":false,"given":"Kris Ann P.","family":"Schultz","sequence":"additional","affiliation":[{"name":"International PPB\/DICER1 Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,8,16]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1174334"},{"key":"e_1_2_8_3_1","volume-title":"GeneReviews","author":"Schultz KAP","year":"1993"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ygyno.2011.03.024"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800311"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25614"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29017"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2017.04.009"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ygyno.2017.09.034"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2020-107434"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2015.115"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-019-0805-5"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1155\/2008\/619637"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19800301)45:5<985::AID-CNCR2820450525>3.0.CO;2-5"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.6746.1"},{"issue":"3","key":"e_1_2_8_16_1","first-page":"206","article-title":"Pleuropulmonary blastoma: more than a lung neoplasm of childhood","volume":"116","author":"Dehner LP","year":"2019","journal-title":"Mo Med"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-198508000-00001"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2013.242"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2016.03.002"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-17-3089"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.29284","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pbc.29284","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.29284","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T04:40:21Z","timestamp":1693284021000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.29284"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,16]]},"references-count":19,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2021,11]]}},"alternative-id":["10.1002\/pbc.29284"],"URL":"https:\/\/doi.org\/10.1002\/pbc.29284","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,16]]},"assertion":[{"value":"2021-06-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-07-28","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-08-16","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e29284"}}