{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T17:17:11Z","timestamp":1762363031273,"version":"build-2065373602"},"reference-count":0,"publisher":"International Institute of Anticancer Research","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CDP"],"published-print":{"date-parts":[[2025,11,1]]},"abstract":"<jats:p>Renal angiomyolipomas (AMLs) are typically benign, fat-rich mesenchymal tumours composed of vascular, smooth muscle, and adipose elements. However, rare fat-poor subtypes, particularly epithelioid angiomyolipoma (EAML), pose significant diagnostic and therapeutic challenges due to their malignant potential and morphological overlap with renal cell carcinoma (RCC). EAML represents less than 1% of AMLs but differs markedly in its biological behaviour, with a propensity for local invasion, lymphadenopathy, and distant metastasis, most commonly to the lungs and liver. Preoperative diagnosis is often delayed or incorrect, as imaging may fail to detect fat-deficient lesions, making histopathological evaluation necessary. EAML is defined by sheets of polygonal epithelioid cells with eosinophilic or clear cytoplasm, prominent nucleoli, and frequent mitotic figures. Genetic alterations in TSC1\/TSC2 and activation of the mTOR pathway are central to pathogenesis, particularly in tuberous sclerosis complex-associated cases. Management hinges on surgical resection, yet recurrence and metastasis may occur years after nephrectomy. mTOR inhibitors have demonstrated efficacy in tumour reduction, especially in TSC-linked cases, while VEGF pathway inhibitors and immune checkpoint blockade represent promising but underexplored avenues. Prognostic features include tumour size &gt;9 cm, necrosis, vascular invasion, and high epithelioid cell proportion. Given the rarity and aggressive potential of EAML, long-term surveillance and multidisciplinary care are essential. Continued research into molecular drivers and targeted therapies will be critical to improving diagnostic precision and patient outcomes.<\/jats:p>","DOI":"10.21873\/cdp.10495","type":"journal-article","created":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T17:12:35Z","timestamp":1762362755000},"page":"788-795","source":"Crossref","is-referenced-by-count":0,"title":["Renal Epithelioid Angiomyolipomas: Clinicopathological Features, Diagnosis, and Management"],"prefix":"10.21873","volume":"5","author":[{"given":"KATHRINE S.","family":"RALLIS","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"9336","published-online":{"date-parts":[[2025,11,1]]},"container-title":["Cancer Diagnosis &amp; Prognosis"],"original-title":[],"deposited":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T17:12:38Z","timestamp":1762362758000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cancerdiagnosisprognosis.org\/article\/550\/renal-epithelioid-angiomyolipomas-clinicopathological-features-diagnosis-and-management"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,1]]},"references-count":0,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2025,11,1]]},"published-print":{"date-parts":[[2025,11,1]]}},"URL":"https:\/\/doi.org\/10.21873\/cdp.10495","relation":{},"ISSN":["2732-7787"],"issn-type":[{"value":"2732-7787","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11,1]]}}}