{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,18]],"date-time":"2025-05-18T23:40:04Z","timestamp":1747611604658,"version":"3.40.5"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,5,19]],"date-time":"2025-05-19T00:00:00Z","timestamp":1747612800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,5,19]],"date-time":"2025-05-19T00:00:00Z","timestamp":1747612800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Egypt Natl Canc Inst"],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>Fibromas are common ovarian stromal tumors, while steroid cell tumors (SCTs) are rare, accounting for\u2009&lt;\u20090.1% of ovarian neoplasms. Approximately, one-third of SCTs exhibit malignant behavior, but predicting malignancy remains challenging.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Case presentation<\/jats:title>\n            <jats:p>A 73-year-old woman presented with nonspecific pelvic pain, and imaging revealed multiple pelvic masses. She underwent a simple hysterectomy and bilateral adnexectomy. Pathological examination revealed a unique colocalization of a fibroma and a SCT in the right ovary. One year later, the SCT recurred with lymph node metastasis. Morphological analysis and whole exome sequencing suggested a shared origin for the fibroma and SCT components. Notably, two missense mutations in <jats:italic>MUC4<\/jats:italic> were identified in the SCT, with immunohistochemistry confirming MUC4 overexpression. Steroidogenesis patterns in the SCT resembled those of adrenocortical carcinoma, indicating disorganized steroidogenesis and potentially explaining the absence of clinical endocrine abnormalities.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>This case underscores the rarity and complexity of concomitant ovarian fibroma and malignant SCT. The identification of <jats:italic>MUC4<\/jats:italic> mutations and disorganized steroidogenesis may provide insights into the pathogenesis of malignant SCTs. Further research is needed to understand the mechanisms and clinical implications of malignant SCT.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/s43046-025-00281-3","type":"journal-article","created":{"date-parts":[[2025,5,18]],"date-time":"2025-05-18T23:02:44Z","timestamp":1747609364000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A rare case of a concomitant ovarian fibroma and malignant steroid cell tumor: insights into pathogenesis and steroidogenesis"],"prefix":"10.1186","volume":"37","author":[{"given":"Chihiro","family":"Inoue","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuto","family":"Yamazaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hironobu","family":"Sasano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yayoi","family":"Aoyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toyoharu","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,5,19]]},"reference":[{"key":"281_CR1","unstructured":"WHO Classification of Tumours Editorial Board, editor. WHO classification of tumours, female genital tumours. 5th ed. Lyon: International agency for research on cancer; 2020."},{"key":"281_CR2","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1097\/00000478-198711000-00002","volume":"11","author":"MC Hayes","year":"1987","unstructured":"Hayes MC, Scully RE. Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases. Am J Surg Pathol. 1987;11:835\u201345. https:\/\/doi.org\/10.1097\/00000478-198711000-00002.","journal-title":"Am J Surg Pathol"},{"key":"281_CR3","doi-asserted-by":"publisher","first-page":"570","DOI":"10.1097\/PAS.0000000000002201","volume":"48","author":"O Fadare","year":"2024","unstructured":"Fadare O, Fard EV, Bhargava R, Desouki MM, Hanley KZ, Ip PPC, et al. The malignant potential of ovarian steroid cell tumors revisited: a multi-institutional clinicopathologic analysis of 115 cases. Am J Surg Pathol. 2024;48:570\u201380. https:\/\/doi.org\/10.1097\/PAS.0000000000002201.","journal-title":"Am J Surg Pathol"},{"key":"281_CR4","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1097\/PGP.0000000000000484","volume":"38","author":"RW-C Wong","year":"2019","unstructured":"Wong RW-C, McCluggage WG. Sex cord-stromal tumors with steroid cell tumor and fibroma components: report of 2 cases, including one of extraovarian origin. Int J Gynecol Pathol. 2019;38:151\u20136. https:\/\/doi.org\/10.1097\/PGP.0000000000000484.","journal-title":"Int J Gynecol Pathol"},{"issue":"1","key":"281_CR5","doi-asserted-by":"publisher","first-page":"1352","DOI":"10.1186\/s12885-021-09098-4","volume":"21","author":"F Fujishima","year":"2019","unstructured":"Fujishima F, Fukuhara N, Katsushima H, Nakamura Y, Harigae H, Sasano H, Ichinohasama R. Utility of a new notation to visualize flow cytometry analysis results: first preliminary comparison with immunohistochemistry to detect CD30 expression on T-cell lymphoma cells. BMC Cancer. 2019;21(1):1352. https:\/\/doi.org\/10.1186\/s12885-021-09098-4.","journal-title":"BMC Cancer"},{"key":"281_CR6","doi-asserted-by":"publisher","first-page":"1398","DOI":"10.1097\/PAS.0000000000002124","volume":"47","author":"RP Mendoza","year":"2023","unstructured":"Mendoza RP, Wang P, Smith HL, Fitzpatrick CA, Haridas R, Wanjari P, et al. Clinicopathologic analysis and molecular profiling of ovarian steroid cell tumors. Am J Surg Pathol. 2023;47:1398\u2013408. https:\/\/doi.org\/10.1097\/PAS.0000000000002124.","journal-title":"Am J Surg Pathol"},{"key":"281_CR7","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1530\/erc-23-0179","volume":"30","author":"A Chao","year":"2023","unstructured":"Chao A, Huang H-J, Lin C-Y, Lee C-H, Lin C-H, Chao A-S, et al. Hypoxia-associated genetic signature in ovarian steroid cell tumor NOS. Endocrine-Related Cancer. 2023;30:30. https:\/\/doi.org\/10.1530\/erc-23-0179.","journal-title":"Endocrine-Related Cancer"},{"key":"281_CR8","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2024.1331903","volume":"14","author":"CH Wei","year":"2024","unstructured":"Wei CH, Fadare O. Ovarian steroid cell tumors: what do we know so far? Front Oncol. 2024;14: 1331903. https:\/\/doi.org\/10.3389\/fonc.2024.1331903.","journal-title":"Front Oncol"},{"key":"281_CR9","doi-asserted-by":"publisher","first-page":"1551","DOI":"10.18203\/2320-6012.ijrms20201358","volume":"8","author":"R Mahendran","year":"2020","unstructured":"Mahendran R, Kanchana MP. Ovarian fibrothecoma with minor sex cord elements: a case report. Int J Res Med Sci. 2020;8:1551. https:\/\/doi.org\/10.18203\/2320-6012.ijrms20201358.","journal-title":"Int J Res Med Sci"},{"key":"281_CR10","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1159\/000475527","volume":"105","author":"L De Mestier","year":"2017","unstructured":"De Mestier L, Cros J, Neuzillet C, Hentic O, Egal A, Muller N, et al. Digestive system mixed neuroendocrine-non-neuroendocrine neoplasms. Neuroendocrinology. 2017;105:412\u201325. https:\/\/doi.org\/10.1159\/000475527.","journal-title":"Neuroendocrinology"},{"key":"281_CR11","doi-asserted-by":"publisher","first-page":"14147","DOI":"10.18632\/oncotarget.13122","volume":"8","author":"P Xia","year":"2017","unstructured":"Xia P, Choi AH, Deng Z, Yang Y, Zhao J, Wang Y, et al. Cell membrane-anchored MUC4 promotes tumorigenicity in epithelial carcinomas. Oncotarget. 2017;8:14147\u201357. https:\/\/doi.org\/10.18632\/oncotarget.13122.","journal-title":"Oncotarget"},{"key":"281_CR12","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1016\/0046-8177(93)90088-x","volume":"24","author":"H Sasano","year":"1993","unstructured":"Sasano H, Suzuki T, Nagura H, Nishikawa T. Steroidogenesis in human adrenocortical carcinoma: biochemical activities, immunohistochemistry, and in situ hybridization of steroidogenic enzymes and histopathologic study in nine cases. Hum Pathol. 1993;24:397\u2013404. https:\/\/doi.org\/10.1016\/0046-8177(93)90088-x.","journal-title":"Hum Pathol"}],"container-title":["Journal of the Egyptian National Cancer Institute"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43046-025-00281-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s43046-025-00281-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43046-025-00281-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,18]],"date-time":"2025-05-18T23:02:46Z","timestamp":1747609366000},"score":1,"resource":{"primary":{"URL":"https:\/\/jenci.springeropen.com\/articles\/10.1186\/s43046-025-00281-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,19]]},"references-count":12,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["281"],"URL":"https:\/\/doi.org\/10.1186\/s43046-025-00281-3","relation":{},"ISSN":["2589-0409"],"issn-type":[{"value":"2589-0409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,5,19]]},"assertion":[{"value":"3 February 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 March 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 May 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was conducted in accordance with the Declaration of Helsinki. The Ethics Committee of Tohoku University waived the requirement for review, given the nature of a case report. Written informed consent was obtained from the participants for genetic analysis and publication of this case report.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the participants for genetic analysis and publication of this case report.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"21"}}