{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T09:00:37Z","timestamp":1776330037077,"version":"3.50.1"},"reference-count":30,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Oncology"],"abstract":"<jats:p>Background: POLE-mutated endometrial carcinomas are associated with exceptionally favorable outcomes, forming the basis for treatment de-escalation in early-stage disease. Nevertheless, rare adverse clinical courses have been reported. This study describes an unusual case of late metastatic recurrence in a POLE-mutated tumor and provides a review of similar cases in the literature. Methods: We present a detailed clinical, radiological, pathological, and molecular description of a patient who developed metastatic recurrence 16 years after initial surgery. A systematic literature search was conducted to identify reports of recurrence, progression, or cancer-related death in POLE-mutated endometrial carcinoma, with extraction of recurrence patterns, genomic features, treatment, and outcomes. Results: The patient experienced sequential pulmonary, cerebral, and leptomeningeal metastases despite harboring a canonical POLE hotspot mutation, proficient mismatch repair status, wild-type TP53, no additional known driver mutation beyond PTEN alterations. The literature review identified a small number of similarly adverse cases. Reported recurrences were heterogeneous, though distant and occasionally central nervous system involvement were noted. Conclusions: While POLE-mutated tumors overall retain an excellent prognosis, rare cases may follow an atypical and aggressive course. Improved molecular annotation and integrated risk-stratification models are needed to better identify this minority of higher-risk patients.<\/jats:p>","DOI":"10.3390\/curroncol33040219","type":"journal-article","created":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T07:37:19Z","timestamp":1776325039000},"page":"219","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Unusual Case of Neuromeningeal Late Relapse of POLE Mutated Endometrioid Carcinoma: A Case Report and Systematic Review"],"prefix":"10.3390","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-6897-2083","authenticated-orcid":false,"given":"Emma","family":"Donati","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1910-4777","authenticated-orcid":false,"given":"Michel","family":"Fabbro","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"},{"name":"Groupe d\u2019Investigateurs Nationaux pour l\u2019Etude des Cancers de l\u2019Ovaire et du Sein (GINECO), 75008 Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"No\u00e9mie","family":"Drappier","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2281-3999","authenticated-orcid":false,"given":"Alexis","family":"Marguerit","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Leaha","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"St\u00e9phanie","family":"Nougaret","sequence":"additional","affiliation":[{"name":"Department of Radiology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0697-109X","authenticated-orcid":false,"given":"Pierre-Emmanuel","family":"Colombo","sequence":"additional","affiliation":[{"name":"Groupe d\u2019Investigateurs Nationaux pour l\u2019Etude des Cancers de l\u2019Ovaire et du Sein (GINECO), 75008 Paris, France"},{"name":"Department of Surgical Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6338-9306","authenticated-orcid":false,"given":"Stanislas","family":"Quesada","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Institut du Cancer de Montpellier (ICM), 34090 Montpellier, France"},{"name":"Groupe d\u2019Investigateurs Nationaux pour l\u2019Etude des Cancers de l\u2019Ovaire et du Sein (GINECO), 75008 Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,16]]},"reference":[{"key":"ref_1","first-page":"12","article-title":"Cancer Statistics, 2024","volume":"74","author":"Siegel","year":"2024","journal-title":"CA Cancer J. 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