{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T10:38:10Z","timestamp":1780569490372,"version":"3.54.1"},"reference-count":56,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T00:00:00Z","timestamp":1745193600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background\/Objectives: Extra-gastrointestinal stromal tumors (EGISTs) are rare mesenchymal tumors arising outside the gastrointestinal tract, making up &lt;5% of all GISTs. Rectovaginal EGISTs are particularly uncommon, with limited available data. This study systematically reviews the clinicopathological features, management, and outcomes of rectovaginal EGISTs. Methods: A systematic review of the English-language literature was conducted for studies on rectovaginal EGISTs (search date: 15 January 2025). Results: Thirty-one studies, including 40 female patients (mean age: 55.2 \u00b1 15.4 years), met the inclusion criteria. Presenting symptoms included vaginal bleeding (24.3%), palpable mass (13.5%), constipation (10.8%), and abdominal pain (8.1%); however, the majority of patients (45.9%) were asymptomatic. Surgical excision was undertaken in 95% of patients, more often via local resection (61.1%). A high-grade mitotic index (&gt;5\/50 HPF) was noted in 63.2%. CD117, DOG-1, and vimentin was expressed in all cases, while CD34 was positive in 97.1%. Adjuvant therapy with tyrosine kinase inhibitors (TKIs) was administered in 57.5%, and neoadjuvant therapy was rare (8.6%). Recurrence occurred in 39.4% over a median follow-up of 40 \u00b1 61.5 months, with a median disease-free survival (DFS) of 48 months. One death occurred 13 months postoperatively. Conclusions: Rectovaginal EGISTs are exceedingly rare and often asymptomatic, complicating preoperative diagnosis. Surgical resection remains the cornerstone of treatment, complemented by stage-specific neoadjuvant or adjuvant TKI therapy. The challenging location predisposes to recurrence, underscoring the need for further studies to optimize management and improve outcomes.<\/jats:p>","DOI":"10.3390\/cancers17081382","type":"journal-article","created":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T20:38:26Z","timestamp":1745267906000},"page":"1382","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Rectovaginal Extra-Gastrointestinal Stromal Tumors (EGISTs): A Systematic Review of the Literature and a Pooled Survival Analysis"],"prefix":"10.3390","volume":"17","author":[{"given":"Eleni","family":"Papamattheou","sequence":"first","affiliation":[{"name":"First Department of Obstetrics and Gynecology, National and Kapodistrian University of Athens, Alexandra General Hospital, 11528 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6743-8942","authenticated-orcid":false,"given":"Ioannis","family":"Katsaros","sequence":"additional","affiliation":[{"name":"First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1583-1125","authenticated-orcid":false,"given":"Stavros P.","family":"Papadakos","sequence":"additional","affiliation":[{"name":"First Department of Gastroenterology, National and Kapodistrian University of Athens, Laikon General Hospital, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evangelos","family":"Lianos","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Metaxa Cancer Hospital, 18537 Piraeus, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2619-1058","authenticated-orcid":false,"given":"Elissaios","family":"Kontis","sequence":"additional","affiliation":[{"name":"Department of Surgical Oncology, Metaxa Cancer Hospital, 18537 Piraeus, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1038\/s41572-021-00254-5","article-title":"Gastrointestinal stromal tumours","volume":"7","author":"Blay","year":"2021","journal-title":"Nat. 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