{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:37:22Z","timestamp":1791999442393,"version":"4.4.0"},"reference-count":46,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2024,3,31]],"date-time":"2024-03-31T00:00:00Z","timestamp":1711843200000},"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>Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30\u201340% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.<\/jats:p>","DOI":"10.3390\/cancers16071375","type":"journal-article","created":{"date-parts":[[2024,3,31]],"date-time":"2024-03-31T13:28:00Z","timestamp":1711891680000},"page":"1375","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group"],"prefix":"10.3390","volume":"16","author":[{"given":"Miriam","family":"Dellino","sequence":"first","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari \u201cAldo Moro\u201d, Piazza Giulio Cesare 11, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francescapaola","family":"Magazzino","sequence":"additional","affiliation":[{"name":"Complex Operating Unit Ginecologia E Ostetricia, Ospedale Civile Di San Dona\u2019 Di Piave (Venezia), Aulss4 Veneto Orientale, 30027 San Don\u00e0 di Piave, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lavinia","family":"Domenici","sequence":"additional","affiliation":[{"name":"Division of Obstetrics and Gynecology, Azienda Ospedaliera Universitaria Pisana, University of Pisa, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Cicogna","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health-IRCCS \u201cBurlo Garofolo\u201d, 34145 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salvatora Tindara","family":"Miano","sequence":"additional","affiliation":[{"name":"Complex Operating Unit of Oncology, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8836-2633","authenticated-orcid":false,"given":"Sandro","family":"Pignata","sequence":"additional","affiliation":[{"name":"Department of Urology and Gynecology, Istituto Nazionale Tumori IRCCS \u2018Fondazione G Pascale\u2019, 80144 Napoli, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8451-429X","authenticated-orcid":false,"given":"Giorgia","family":"Mangili","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Unit, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5745-372X","authenticated-orcid":false,"given":"Gennaro","family":"Cormio","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari \u201cAldo Moro\u201d, Piazza Giulio Cesare 11, 70124 Bari, Italy"},{"name":"Gynecologic Oncology, IRCCS Istituto Tumori \u201cGiovanni Paolo II\u201d, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,3,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1097\/00000478-198307000-00009","article-title":"Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm","volume":"7","author":"Steeper","year":"1983","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1046\/j.1365-2559.1999.00835.x","article-title":"Myxoid tumours of soft tissue","volume":"35","author":"Hogendoorn","year":"1999","journal-title":"Histopathology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"5661","DOI":"10.1038\/s41598-021-85030-x","article-title":"Four-decade trends in lymph node status of patients with vulvar squamous cell carcinoma in northern Italy","volume":"11","author":"Preti","year":"2021","journal-title":"Sci. Rep."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Dellino, M., Cicogna, S., Falcone, F., Mitidieri, M., Mazzeo, R., Pignata, S., Mangili, G., and Cormio, G. (2022). \u201cIntestinal-Type\u201d Vulvar Adenocarcinoma: A Review of the MITO Rare Tumors Group. Cancers, 14.","DOI":"10.3390\/cancers14205171"},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Cicogna, S., Dellino, M., Miano, S.T., Magazzino, F., Domenici, L., Pignata, S., Mangili, G., and Cormio, G. (2023). Aggressive Angiomyxoma of the Lower Female Genital Tract in Pregnancy: A Review of the MITO Rare Tumors Group. Cancers, 15.","DOI":"10.3390\/cancers15133403"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1001\/archderm.1986.01660190068018","article-title":"Cutaneous myxomas. A major component of the complex of myxomas, spotty pigmentation, and endocrine overactivity","volume":"122","author":"Carney","year":"1986","journal-title":"Arch. Dermatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1136\/ijgc-00009577-200401000-00012","article-title":"Vulvar soft tissue tumors","volume":"14","author":"Behranwala","year":"2004","journal-title":"Int. J. Gynecol. Cancer"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1034\/j.1600-0412.2000.079003216.x","article-title":"Aggressive angiomyxoma in females: Is radical resection the only option?","volume":"79","author":"Chan","year":"2000","journal-title":"Acta Obstet. Gynecol. Scand."},{"key":"ref_9","first-page":"292304","article-title":"Aggressive angiomyxoma of the vulva: A bizarre perineal lesion","volume":"2015","author":"Myoteri","year":"2015","journal-title":"Case Rep. Oncol. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s00428-004-1135-9","article-title":"Aggressive angiomyxoma: A clinicopathological and immunohistochemical study of 11 cases with long-term follow-up","volume":"446","author":"Hogendoorn","year":"2005","journal-title":"Virchows Arch."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1007\/s00428-006-0186-5","article-title":"Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15)","volume":"448","author":"Micci","year":"2006","journal-title":"Virchows Arch."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1002\/gcc.1179","article-title":"Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva","volume":"32","author":"Nucci","year":"2001","journal-title":"Genes Chromosomes Cancer"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1007\/s00428-022-03435-z","article-title":"Real-world histopathological approach to malignancy of undefined primary origin (MUO) to diagnose cancers of unknown primary (CUPs)","volume":"482","author":"Pisacane","year":"2023","journal-title":"Virchows Arch."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1772","DOI":"10.1056\/NEJM199912023412315","article-title":"Metastasizing aggressive angiomyxoma","volume":"341","author":"Siassi","year":"1999","journal-title":"N. Engl. J. Med."},{"key":"ref_15","first-page":"1553","article-title":"Clinicopathological analysis of aggressive angiomyxoma","volume":"92","author":"Wei","year":"2012","journal-title":"Zhonghua Yi Xue Za Zhi"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1097\/LGT.0000000000000737","article-title":"Multiple Vulvar Polyps in Pregnancy: A Benign Disease With a Challenging Diagnosis","volume":"27","author":"Pinto","year":"2023","journal-title":"J. Low. Genit. Tract Dis."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Gulino, F.A., Gulisano, M., Ettore, C., Giorlandino, A., Russo, E., and Ettore, G. (2023). Aggressive Angiomyxoma of the Vulva: Which Is the Best Management Strategy? Description of a Case Report and Review of Literature of the Last Ten Years. J. Clin. Med., 12.","DOI":"10.3390\/jcm12051726"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"0300060520936414","DOI":"10.1177\/0300060520936414","article-title":"Aggressive angiomyxoma in pregnancy: A case report and literature review","volume":"48","author":"Xu","year":"2020","journal-title":"J. Int. Med. Res."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e24485","DOI":"10.1097\/MD.0000000000024485","article-title":"A strengthening the reporting of observational studies in epidemiology (STROBE): Are HE4 and CA 125 suitable to detect a Paget disease of the vulva?","volume":"100","author":"Dellino","year":"2021","journal-title":"Medicine"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.ijscr.2016.05.047","article-title":"Aggressive angiomyxoma presenting with back and perineal bulge; a complex surgical approach: A case report","volume":"24","author":"Faraj","year":"2016","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/0163-8343(80)90081-X","article-title":"Training outcome in liaison psychiatry. Literature review and methodological proposals","volume":"2","year":"1980","journal-title":"Gen. Hosp. Psychiatry"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"525","DOI":"10.5468\/ogs.2015.58.6.525","article-title":"Alternative surgical approaches for aggressive angiomyxoma at different sites in the pelvic cavity","volume":"58","author":"Choi","year":"2015","journal-title":"Obstet. Gynecol. Sci."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1007\/s00404-009-0934-2","article-title":"Aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis","volume":"280","author":"Salman","year":"2009","journal-title":"Arch. Gynecol. Obstet."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1016\/j.ygyno.2005.09.033","article-title":"Aggressive angiomyxoma of the vulva: Dramatic response to gonadotropin-releasing hormone agonist therapy","volume":"100","author":"McCluggage","year":"2006","journal-title":"Gynecol. Oncol."},{"key":"ref_25","first-page":"715","article-title":"Five cases\u2019 female aggressive angiomyxoma experience","volume":"38","author":"Yuan","year":"2017","journal-title":"Eur. J. Gynaecol. Oncol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"E55","DOI":"10.1097\/LGT.0b013e3182a22019","article-title":"Hormonal therapy for aggressive angiomyxoma: A case report and proposed management algorithm","volume":"18","author":"Schwartz","year":"2014","journal-title":"J. Low. Genit. Tract Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1145","DOI":"10.1007\/s00404-010-1575-1","article-title":"Aggressive angiomyxoma of vulva and vagina: A series of three cases and review of literature","volume":"283","author":"Dahiya","year":"2011","journal-title":"Arch. Gynecol. Obstet."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2050313X19843391","DOI":"10.1177\/2050313X19843391","article-title":"Aggressive angiomyxoma of the perineal region","volume":"7","author":"Raptin","year":"2019","journal-title":"SAGE Open Med. Case Rep."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1053\/S0046-8177(03)00419-2","article-title":"Aggressive angiomyxoma: A second case of metastasis with patient\u2019s death","volume":"34","author":"Blandamura","year":"2003","journal-title":"Hum. Pathol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"102706","DOI":"10.1016\/j.amsu.2021.102706","article-title":"Aggressive angiomyxoma of vulva in 28-years old patient: A case report of second recurrence","volume":"69","author":"Zamani","year":"2021","journal-title":"Ann. Med. Surg."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1007\/s00261-005-0378-5","article-title":"Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: Report of three cases and review of the literature","volume":"31","author":"Wiser","year":"2006","journal-title":"Abdom. Imaging"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1111\/j.1442-2042.2004.00816.x","article-title":"Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist","volume":"11","author":"Shinohara","year":"2004","journal-title":"Int. J. Urol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1097\/01.coc.0000170581.28275.e9","article-title":"External beam radiotherapy for perirectal angiomyxoma results in a dramatic clinical response and allows a patient to avoid abdominoperineal resection","volume":"29","author":"Bhandari","year":"2006","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1016\/S0046-8177(85)80112-X","article-title":"Aggressive angiomyxoma of pelvic soft parts: A clinicopathologic study of nine cases","volume":"16","author":"Begin","year":"1985","journal-title":"Hum. Pathol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1097\/00004347-199710000-00006","article-title":"Superficial angiomyxoma (cutaneous myxoma): A clinicopathologic study of 17 cases arising in the genital region","volume":"16","author":"Fetsch","year":"1997","journal-title":"Int. J. Gynecol. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1046\/j.1365-2559.1997.d01-556.x","article-title":"Aggressive angiomyxoma: Reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases","volume":"30","author":"Granter","year":"1997","journal-title":"Histopathology"},{"key":"ref_37","first-page":"5604460","article-title":"A Paraurethral Aggressive (Deep) Angiomyxoma","volume":"2022","author":"York","year":"2022","journal-title":"Case Rep. Obstet. Gynecol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1016\/j.ejso.2006.06.008","article-title":"Aggressive angiomyxoma: Multimodality treatments can avoid mutilating surgery","volume":"32","author":"Bakker","year":"2006","journal-title":"Eur. J. Surg. Oncol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1007\/s00192-012-1867-z","article-title":"Periurethral aggressive angiomyxoma: A case report","volume":"24","author":"Allen","year":"2013","journal-title":"Int. Urogynecol. J."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"e240687","DOI":"10.1136\/bcr-2020-240687","article-title":"Vulvar aggressive angiomyxoma: A surgical challenge","volume":"14","author":"Srivastava","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_41","first-page":"1304","article-title":"Aggressive angiomyxoma in the ischiorectal fossa","volume":"70","author":"Wahid","year":"2020","journal-title":"J. Pak. Med. Assoc."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"107313","DOI":"10.1016\/j.ijscr.2022.107313","article-title":"A deep giant aggressive angiomyxoma of the labia majora: A case report","volume":"96","author":"Ayati","year":"2022","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"151","DOI":"10.4103\/2229-5178.131085","article-title":"Aggressive angiomyxoma","volume":"5","author":"Padmavathy","year":"2014","journal-title":"Indian Dermatol. Online J."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Li, J., You, L., Wang, C., Zhao, H., Guo, W., Yu, J., Yuan, Z., Qi, S., and Huang, Y. (2022). Clinicopathological characteristics and prognosis analysis of Aggressive angiomyxoma: A Retrospective Study. Res. Sq.","DOI":"10.21203\/rs.3.rs-2335657\/v1"},{"key":"ref_45","first-page":"27","article-title":"Rare case of metastatic aggressive angiomyxoma-first case of renal metastasis","volume":"6","author":"Siddiqui","year":"2020","journal-title":"Gynecol. Obstet. Case Rep."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1186\/s13256-022-03284-z","article-title":"Aggressive (deep) angiomyxoma of the vulva: A case report","volume":"16","author":"Goyal","year":"2022","journal-title":"J. Med. Case Rep."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/16\/7\/1375\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:21:44Z","timestamp":1760106104000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/16\/7\/1375"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,31]]},"references-count":46,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2024,4]]}},"alternative-id":["cancers16071375"],"URL":"https:\/\/doi.org\/10.3390\/cancers16071375","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,31]]}}}