{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T14:16:34Z","timestamp":1787235394077,"version":"build-2736575974"},"reference-count":11,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T00:00:00Z","timestamp":1716336000000},"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":["APMIS"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:p>Renal cell carcinoma (RCC) accounts for 2% of all cancer cases worldwide, and majority are sporadic. The latest World Health Organization (WHO) classification of renal cell tumors (fifth edition, 2022) has molecularly defined renal tumor entities, which includes fumarate hydratase (FH)\u2013deficient RCC. FH\u2010deficient RCC is an aggressive carcinoma caused by pathogenic alterations in FH gene, seen in 15% of patients with hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC) syndrome. These tumors occur more frequently at a younger age and present at an advanced stage, carrying a dismal prognosis. We report a series of 10 cases of FH\u2010deficient RCC. The mean age was 49.8\u2009years, and all cases presented in advanced stages (III and IV). Morphologically, the cases had varied architectural patterns with characteristic eosinophilic macronucleoli and perinucleolar halo. On immunohistochemistry (IHC), all showed diffuse nucleo\u2010cytoplasmic expression of S\u2010(2\u2010succino)\u2010cysteine (2\u2010SC), with loss of FH in seven cases. FH\u2010deficient RCCs are aggressive neoplasms and can be diagnosed using specific IHC markers (FH and 2\u2010SC). These patients should undergo germline testing for FH gene mutation, genetic counseling, and surveillance of family members.<\/jats:p>","DOI":"10.1111\/apm.13425","type":"journal-article","created":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T11:29:03Z","timestamp":1716377343000},"page":"544-552","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Fumarate hydratase\u2013deficient renal cell carcinoma: an oncology care institutional experience"],"prefix":"10.1111","volume":"132","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0711-1718","authenticated-orcid":false,"given":"Meenakshi","family":"Kamboj","sequence":"first","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gurudutt","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunil","family":"Pasricha","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anurag","family":"Mehta","sequence":"additional","affiliation":[{"name":"Laboratory Services &amp; Molecular Diagnostics Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudhir","family":"Rawal","sequence":"additional","affiliation":[{"name":"Department of GenitoUro\u2010Oncology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amitabh","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of GenitoUro\u2010Oncology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anila","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garima","family":"Durga","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Divya","family":"Bansal","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Himanshi","family":"Diwan","sequence":"additional","affiliation":[{"name":"Department of Histopathology and Cytopathology Rajiv Gandhi Cancer Institute &amp; Research Centre  Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,5,22]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"78","volume-title":"Urinary and Male Genital Tumours","author":"Hes O","year":"2022"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001372"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2018.01.005"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2021-207830"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13727"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000617"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10689-014-9735-2"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-018-0017-7"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2019.07.004"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001476"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13183"}],"container-title":["APMIS"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apm.13425","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T13:11:53Z","timestamp":1720617113000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apm.13425"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,22]]},"references-count":11,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2024,8]]}},"alternative-id":["10.1111\/apm.13425"],"URL":"https:\/\/doi.org\/10.1111\/apm.13425","archive":["Portico"],"relation":{},"ISSN":["0903-4641","1600-0463"],"issn-type":[{"value":"0903-4641","type":"print"},{"value":"1600-0463","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,22]]},"assertion":[{"value":"2023-10-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-04-26","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}