{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T15:01:18Z","timestamp":1788188478233,"version":"build-2803163510"},"reference-count":37,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn\u2013Werner\u2013Wunderlich syndrome (HWWS), is a rare M\u00fcllerian malformation. Gynaecologic malignancies reported in association with OHVIRA syndrome\/HWWS are exceptional and scattered across isolated case reports and small case series, leading to significant challenges for screening, early diagnosis, and optimal management. The primary aim of this study was to comprehensively review and synthesize the clinicopathologic features, treatment approaches, and reported outcomes of patients with OHVIRA-associated gynaecologic neoplasms. Methods: A systematic review of published cases of gynecologic malignancies in OHVIRA\/HWWS was conducted using PubMed\/MEDLINE and Scopus from database inception through January 2026. To expand the current evidence, an illustrative novel institutional case is also presented and integrated into the analysis. Results: A total of 21 cases were identified and analysed; reported tumours predominantly involved the lower genital tract (vagina\/cervix), with a recurrent representation of adenocarcinoma, particularly clear cell histology, and a frequent origin from the obstructed or non-visible compartment when side was described. Endometrial and ovarian involvement was uncommon. Conclusions: The anatomical complexity of OHVIRA syndrome creates a diagnostic blind spot that warrants heightened clinical suspicion, rigorous MRI-based anatomic delineation, and side-specific evaluation in symptomatic patients. By synthesizing the available literature, this review underscores the necessity for tailored, multidisciplinary management and proactive surveillance strategies in this rare population.<\/jats:p>","DOI":"10.3390\/jcm15082824","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T09:49:44Z","timestamp":1775641784000},"page":"2824","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Gynecologic Malignancies in Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: A Systematic Review"],"prefix":"10.3390","volume":"15","author":[{"given":"Giuseppe","family":"Parisi","sequence":"first","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0938-2493","authenticated-orcid":false,"given":"Emanuele","family":"Perrone","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Capasso","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Bruno","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-0203-5055","authenticated-orcid":false,"given":"Maria Consiglia","family":"Giuliano","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3547-4853","authenticated-orcid":false,"given":"Nicola","family":"Macellari","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6588-8686","authenticated-orcid":false,"given":"Marco","family":"D\u2019Indinosante","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Fanfani","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Complessa di Ginecologia Oncologica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanit\u00e0 Pubblica, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo Agostino Gemelli, 8, 00136 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"222","DOI":"10.4103\/0366-6999.149208","article-title":"New classification of Herlyn-Werner-Wunderlich syndrome","volume":"128","author":"Zhu","year":"2015","journal-title":"Chin. Med. 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