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Herein, we report the first concurrent embryonic-type neuroectodermal tumor and nephroblastoma within the primary testicular tumor, arising associated with a mixed germ cell tumor. A 36-year-old man presented with a 4.5 cm right testicular mass. He underwent right orchiectomy. Gross examination revealed a well-circumscribed, solid to cystic mass confined to the testis (pT1). Histological examination demonstrated a mixed germ cell tumor composed of teratoma (40% of the tumor mass), seminoma (10%), embryonal carcinoma (2.5%), and yolk sac tumor (2.5%), with associated germ cell neoplasia in situ. Additionally, two somatic-type malignancy components were identified, including embryonic-type neuroectodermal tumor (40%) and nephroblastoma (5%). The embryonic-type neuroectodermal tumor consisted of primitive neural tubules, rosettes, and blastemal cells. The nephroblastoma was composed of tubules, blastemal cells, and stromal cells. Immunohistochemically, the embryonic-type neuroectodermal tumor showed diffuse positivity for SOX11, with focal expression of synaptophysin and CD99, while the nephroblastoma was positive for PAX8 and WT1. The patient developed metastasis to a retroperitoneal lymph node 1 month after the orchiectomy, subsequently received chemotherapy, and is alive without metastatic disease at 13 months of follow-up.<\/jats:p>","DOI":"10.1177\/10668969261465921","type":"journal-article","created":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T17:58:08Z","timestamp":1787075888000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Testicular Mixed Germ Cell Tumor with Concurrent Embryonic-Type Neuroectodermal Tumor and Nephroblastoma"],"prefix":"10.1177","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-6538-7918","authenticated-orcid":false,"given":"Linjie","family":"Xiong","sequence":"first","affiliation":[{"name":"Massachusetts General Hospital Mass"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-0768-4764","authenticated-orcid":false,"given":"Ting","family":"Zhao","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital Mass"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0898-123X","authenticated-orcid":false,"given":"Robert S.","family":"Wang","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital Mass General Brigham and Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0444-0068","authenticated-orcid":false,"given":"Chin-Lee","family":"Wu","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital Mass"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,8,18]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.5858\/2007-131-1267-AOOTGC"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.ju.0000116841.30826.85"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.14218\/JCTP.2022.00028"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-022-01136-1"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001789"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199708000-00003"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2010.70"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1177\/23247096231218145"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.eucr.2016.01.003"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.5114\/wo.2023.127193"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199809000-00010"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/his.14667"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2023.02.008"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800310"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2020-0352-OA"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-024-03911-8"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000002241"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.modpat.2024.100702"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200405000-00019"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1155\/2016\/7318672"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000262"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318033c7c4"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181adb9d7"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2025.1546607"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-009-0842-7"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1111\/pin.13293"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/7971019"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1159\/000447146"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1177\/10668969241235315"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.59955"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200203000-00005"},{"issue":"11","key":"e_1_3_2_33_2","first-page":"4265","article-title":"Expression and prognostic value of Wilms\u2019 tumor 1 and early growth response 1 proteins in nephroblastoma","volume":"6","author":"Ghanem MA","year":"2000","unstructured":"Ghanem MA, Van der Kwast TH, Den Hollander JC, et al. 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