{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T23:40:15Z","timestamp":1774482015902,"version":"3.50.1"},"reference-count":26,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T00:00:00Z","timestamp":1745452800000},"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":["Cytopathology"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Fumarate hydratase\u2010deficient renal cell carcinoma (FH\u2010dRCC) is a high\u2010grade renal cell carcinoma characterised by mutations in the <jats:italic>FH<\/jats:italic> gene. FH\u2010dRCC is highly aggressive and has a poor prognosis, underscoring the importance of accurate diagnosis. Although the histopathological features of this neoplasm have been reported in previous studies, descriptions of its cytopathological features are limited. Here, we report the cytopathological morphologies of two cases of metastatic FH\u2010dRCC in male patients aged 48 and 35\u2009years. Case 1 presented with a cystic solid mass in the right retroperitoneal lumbar rib triangle region after the excision of a cystic tumour in the right kidney. Cytopathological examination of the fine\u2010needle aspiration sample revealed papillary and tubular tumour cells. Case 2 developed abdominal ascites after bilateral nephrectomy, and cytopathological examination of ascitic fluid\u2010characterised tumour cells with a high nuclear grade arranged in papillary and glandular patterns. In both cases, large tumour cells were observed with abundant eosinophilic cytoplasm, coarse chromatin and irregular nuclear shapes. Some cells exhibited characteristic eosinophilic macronucleoli and perinucleolar halos. Tumour cell clusters with a high nuclear grade, papillary structures, prominent eosinophilic macronucleoli and perinucleolar halos are the major cytopathological traits of FH\u2010dRCC. CK7\u2010negative\/CA9\u2010negative and PAX8\u2010positive immunophenotypes may serve as important diagnostic markers. Moreover, the AKR1B10\u2010positive\/2SC\u2010positive\/FH\u2010deficient immunophenotype contributes to the diagnosis of this tumour. Consequently, if the tumour cells have these characteristics, the diagnosis of FH\u2010dRCC should be considered in cytology.<\/jats:p>","DOI":"10.1111\/cyt.13504","type":"journal-article","created":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T09:14:56Z","timestamp":1745486096000},"page":"418-422","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Cytopathological Diagnosis of Fumarate Hydratase (<scp>FH<\/scp>)\u2010Deficient Renal Cell Carcinoma in Two Patients With <scp>FH<\/scp> Gene Mutation"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-0679-5670","authenticated-orcid":false,"given":"Xianglan","family":"Zhu","sequence":"first","affiliation":[{"name":"Department of Pathology West China Hospital of Sichuan University  Chengdu 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