{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T05:59:22Z","timestamp":1763445562917,"version":"3.41.2"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,10,5]],"date-time":"2022-10-05T00:00:00Z","timestamp":1664928000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004731","name":"Natural Science Foundation of Zhejiang Province","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004731","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Metanephric stromal tumor (MST) is a rare, benign pediatric renal neoplasm of uncertain histogenesis that belongs to the metanephric family of tumors. MST involving adult patients is very uncommon, which could cause significant diagnostic confusions. Recent molecular studies have revealed recurrent <jats:italic>BRAF<\/jats:italic> mutations in MST in pediatric patients which may serve as powerful diagnostic tools for distinguishing MST from other renal stromal tumors. We present a <jats:italic>BRAF<\/jats:italic>-mutated MST in an adult patient with a brief review of the pertinent literature. To our knowledge, our case represents to date the sixth report of adult MST and the first adult MST proven to harbor <jats:italic>BRAF<\/jats:italic> mutation. This is a 41-year-old man who was incidentally identified to have a left renal mass by ultrasonography. He had a 5-year history of hypertension which could be controlled with oral antihypertensive drug. Partial nephrectomy was performed which demonstrated a 2.6-cm, oval, circumscribed mass with a fibrotic and firm texture. Microscopic examination showed a hypocellular, spindle cell neoplasm with entrapped nephrons, within a predominantly fibrous and focally myxoid stroma. Foci of hyalinized stroma surrounding entrapped native renal tubules or blood vessels to form concentric collarettes-like structures, and small-sized arterioles showing angiodysplasia, were observed. Immunostains showed the tumor cells to be diffusely positive for CD34. Fluorescence <jats:italic>in-situ<\/jats:italic> hybridization analysis was negative for rearrangements involving both the <jats:italic>EWSR1<\/jats:italic> and <jats:italic>FUS<\/jats:italic> loci. Targeted next-generation sequencing disclosed a pathogenic mutation of <jats:italic>BRAF<\/jats:italic> exon15: c.1799T&amp;gt;A (p.V600E). The patient\u2019s hypertension normalized without oral antihypertensive drugs 2 months postoperatively and he was in good status 12 months after the surgery. Our case highlights the diagnostic dilemma of MST occurring in adults and points to the usefulness of molecular detection of <jats:italic>BRAF<\/jats:italic> mutation for arriving at accurate diagnosis.<\/jats:p>","DOI":"10.3389\/fonc.2022.993414","type":"journal-article","created":{"date-parts":[[2022,10,5]],"date-time":"2022-10-05T08:53:13Z","timestamp":1664959993000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Metanephric stromal tumor\u00a0with BRAF V600E mutation in an adult patient: Case report and literature review"],"prefix":"10.3389","volume":"12","author":[{"given":"Yuqian","family":"Fan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jingjing","family":"Yu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,10,5]]},"reference":[{"first-page":"87","volume-title":"WHO Classification of Tumours. Urinary and Male Genital Tumours","year":"2022","key":"B1"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200007000-00003","article-title":"Metanephric stromal tumor: report of 31 cases of a distinctive pediatric renal neoplasm","volume":"24","author":"Argani","year":"2000","journal-title":"Am J Surg Pathol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1097\/01.ju.0000030152.14265.e9","article-title":"Adult presentation of metanephric stromal tumor","volume":"168","author":"Bluebond-Langner","year":"2002","journal-title":"J Urol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"88","DOI":"10.4321\/s0004-06142006000100015","article-title":"Tumor del estroma metan\u00e9frico: presentaci\u00f3n de dos casos en adultos y revisi\u00f3n de la literatura [Metanephric stromal tumor: report of two cases and bibliographic review]","volume":"59","author":"Amat Villegas","year":"2006","journal-title":"Arch Esp Urol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1177\/1066896909344850","article-title":"Metanephric stromal tumor arising in a patient with neurofibromatosis type 1 syndrome","volume":"19","author":"McDonald","year":"2011","journal-title":"Int J Surg Pathol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1177\/1066896912453851","article-title":"Cellular metanephric stromal tumor in a postmenopausal woman: a case report with review of the literature","volume":"21","author":"Chaudhri","year":"2013","journal-title":"Int J Surg Pathol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000603","article-title":"Frequent BRAF V600E mutations in metanephric stromal tumor","volume":"40","author":"Argani","year":"2016","journal-title":"Am J Surg Pathol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2016.09.025","article-title":"BRAF exon 15 mutations in pediatric renal stromal tumors: prevalence in metanephric stromal tumors","volume":"60","author":"Marsden","year":"2017","journal-title":"Hum Pathol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1016\/j.eururo.2012.05.051","article-title":"BRAF mutations in metanephric adenoma of the kidney","volume":"62","author":"Choueiri","year":"2012","journal-title":"Eur Urol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000501","article-title":"Application of BRAF V600E mutation analysis for the diagnosis of metanephric adenofibroma","volume":"39","author":"Mangray","year":"2015","journal-title":"Am J Surg Pathol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/j.pathol.2015.11.007","article-title":"Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement","volume":"48","author":"El Demellawy","year":"2016","journal-title":"Pathology"},{"key":"B12","doi-asserted-by":"publisher","first-page":"294","DOI":"10.1097\/MPH.0000000000002262","article-title":"Role of cyclin D1 and BCOR immunohistochemistry in differentiating clear cell sarcoma of kidney from its mimics","volume":"43","author":"Singh","year":"2021","journal-title":"J Pediatr Hematol Oncol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"8891","DOI":"10.1038\/ncomms9891","article-title":"Recurrent internal tandem duplications of BCOR in clear cell sarcoma of the kidney","volume":"6","author":"Roy","year":"2015","journal-title":"Nat Commun"},{"key":"B14","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1002\/path.3985","article-title":"Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney","volume":"227","author":"O'Meara","year":"2012","journal-title":"J Pathol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000338","article-title":"Primary renal sclerosing epithelioid fibrosarcoma: report of 2 cases with EWSR1-CREB3L1 gene fusion","volume":"39","author":"Argani","year":"2015","journal-title":"Am J Surg Pathol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1093\/ajcp\/aqab211.","article-title":"Solitary fibrous tumor with extensive epithelial inclusions","volume":"158","author":"Zhao","year":"2022","journal-title":"Am J Clin Pathol"},{"volume-title":"WHO Classification of Tumours. Urinary and Male Genital Tumours","year":"2022","key":"B17"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2022.993414\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,10,5]],"date-time":"2022-10-05T08:53:18Z","timestamp":1664959998000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2022.993414\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,5]]},"references-count":17,"alternative-id":["10.3389\/fonc.2022.993414"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2022.993414","relation":{},"ISSN":["2234-943X"],"issn-type":[{"type":"electronic","value":"2234-943X"}],"subject":[],"published":{"date-parts":[[2022,10,5]]},"article-number":"993414"}}