Background: The study aimed to examine all available evidence regarding primary vaginal cancer arising from endometriosis from an evidence-based perspective. Methods: A literature search in Medline and EMBASE databases was conducted. The following key words were employed: ‘vaginal cancer' AND ‘endometriosis malignant transformation' OR ‘endometriosis'. Results: A total of 23 eligible studies were identified and included in the present review providing information for 37 patients. Endometrioid adenocarcinoma (17 cases) was the most common histological subtype followed by endometrial stromal sarcoma (6 cases). The majority of patients received cancer-directed surgery while adjuvant treatment was commonly employed for patients with sarcomas. Conclusion: Vaginal cancer arising from endometriosis is exceedingly rare. The mainstay of treatment is surgical excision. Mean follow-up of patients was relative short. Tumor recurrence was often observed, and the outcome was less favorable for patients with non-epithelial tumors.

Munksgaard PS, Blaakaer J: The association between endometriosis and gynecological cancers and breast cancer: a review of epidemiological data. Gynecol Oncol 2011;123:157-163.
Sayasneh A, Tsivos D, Crawford R: Endometriosis and ovarian cancer: a systematic review. ISRN Obstet Gynecol 2011;2011:140310.
Rogers PA, D'Hooghe TM, Fazleabas A, Gargett CE, Giudice LC, Montgomery GW, Rombauts L, Salamonsen LA, Zondervan KT: Priorities for endometriosis research: recommendations from an international consensus workshop. Reprod Sci 2009;16:335-346.
Staats PN, Clement PB, Young RH: Primary endometrioid adenocarcinoma of the vagina: a clinicopathologic study of 18 cases. Am J Surg Pathol 2007;31:1490-1501.
Scully RE, Richardson GS, Barlow JF: The development of malignancy in endometriosis. Clin Obstet Gynecol 1966;9:384-411.
Brooks JJ, Wheeler JE: Malignancy arising in extragonadal endometriosis: a case report and summary of the world literature. Cancer 1977;40:3065-3073.
Shah C, Pizer E, Veljovich DS, Drescher CW, Peters WA 3rd, Paley PJ: Clear cell adenocarcinoma of the vagina in a patient with vaginal endometriosis. Gynecol Oncol 2006;103:1130-1132.
Sampson JA: Endometrial carcinoma of the ovary, arising in endometrial tissue in that organ. Arch Surg 1925;10:1-72.
Wu X, Matanoski G, Chen VW, Saraiya M, Coughlin SS, King JB, Tao XG: Descriptive epidemiology of vaginal cancer incidence and survival by race, ethnicity, and age in the United States. Cancer 2008;113(10 suppl):2873-2882.
Stern RC, Dash R, Bentley RC, Snyder MJ, Haney AF, Robboy SJ: Malignancy in endometriosis: frequency and comparison of ovarian and extraovarian types. Int J Gynecol Pathol 2001;20:133-139.
Masand RP, Euscher ED, Deavers MT, Malpica A: Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases. Am J Surg Pathol 2013;37:1635-1647.
Leiserowitz GS, Gumbs JL, Oi R, Dalrymple JL, Smith LH, Ryu J, Scudder S, Russell AH: Endometriosis-related malignancies. Int J Gynecol Cancer 2003;13:466-471.
Heaps JM, Nieberg RK, Berek JS: Malignant neoplasms arising in endometriosis. Obstet Gynecol 1990;75:1023-1028.
Reimnitz C, Brand E, Nieberg RK, Hacker NF: Malignancy arising in endometriosis associated with unopposed estrogen replacement. Obstet Gynecol 1988;71(3 pt 2):444-447.
Kwon YS, Nam JH, Choi G: Clear cell adenocarcinoma arising in endometriosis of a previous episiotomy site. Obstet Gynecol 2008;112(2 pt 2):475-477.
Pontrelli G, Cozzolino M, Stepniewska A, Bruni F, Pesci A, Ceccaroni M: Primary vaginal adenosarcoma with sarcomatous overgrowth arising in recurrent endometriosis: feasibility of laparoscopic treatment and review of the literature. J Minim Invasive Gynecol 2016;23:833-838.
Rivard C, Carter JS, Downs LS Jr: Low-grade endometrial stromal sarcoma primarily arising in the vagina: a case report. J Reprod Med 2015;60:433-435.
Han X, Leng J, Guo L, Xiang Y, Lang J: Vaginal adenosarcoma arising from refractory endometriosis: a case report. Aust N Z J Obstet Gynaecol 2010;50:574-576.
Rachaneni S, Spencer C, Lloyd J, Farthing A: Malignant transformation of post-hysterectomy vault endometriotic nodule. J Obstet Gynaecol 2007;27:434-437.
Liu L, Davidson S, Singh M: Müllerian adenosarcoma of vagina arising in persistent endometriosis: report of a case and review of the literature. Gynecol Oncol 2003;90:486-490.
Anderson J, Behbakht K, De Geest K, Bitterman P: Adenosarcoma in a patient with vaginal endometriosis. Obstet Gynecol 2001;98(5 pt 2):964-966.
Judson PL, Temple AM, Fowler WC Jr, Novotny DB, Funkhouser WK Jr: Vaginal adenosarcoma arising from endometriosis. Gynecol Oncol 2000;76:123-125.
Berkowitz RS, Ehrmann RL, Knapp RC: Endometrial stromal sarcoma arising from vaginal endometriosis. Obstet Gynecol 1978;51(1 suppl):34s-37s.
Kondi-Paphitis A, Smyrniotis B, Liapis A, Kontoyanni A, Deligeorgi H: Stromal sarcoma arising on endometriosis. A clinicopathological and immunohistochemical study of 4 cases. Eur J Gynaecol Oncol 1998;19:588-590.
Nomoto K, Hori T, Kiya C, Fukuoka J, Nakashima A, Hidaka T, Saito S, Mikami Y, Tsuneyama K, Takano Y: Endometrioid adenocarcinoma of the vagina with a microglandular pattern arising from endometriosis after hysterectomy. Pathol Int 2010;60:636-641.
Fruscio R, Padula F, Mancini E, Pellegrino A, De Nictolis M: Malignant transformation of vaginal endometriosis treated with neoadjuvant chemotherapy and surgery. J Obstet Gynaecol Res 2008;34(4 pt 2):706-708.
Mahdavi A, Shamshirsaz AA, Peiretti M, Zakashansky K, Idrees MT, Nezhat F: Laparoscopic management of vaginal clear cell adenocarcinoma arising in pelvic endometriosis: case report and literature review. J Minim Invasive Gynecol 2006;13:237-241.
Lavery S, Gillmer M: Malignant transformation of residual endometriosis in women on unopposed oestrogen hormone replacement therapy. BJOG 2001;108:1106-1107.
Fishman A, Demirel D, Laucirica R, Ramzy I, Klima T, Lyzak J, Kaplan AL: Malignant tumors arising in endometriosis: clinical-pathological study and flow cytometry analysis. Eur J Obstet Gynecol Reprod Biol 1996;70:69-74.
Haskel S, Chen SS, Spiegel G: Vaginal endometrioid adenocarcinoma arising in vaginal endometriosis: a case report and literature review. Gynecol Oncol 1989;34:232-236.
Orr JW Jr, Holimon JL, Sisson PF: Vaginal adenocarcinoma developing in residual pelvic endometriosis: a clinical dilemma. Gynecol Oncol 1989;33:96-98.
Granai CO, Walters MD, Safaii H, Jelen I, Madoc-Jones H, Moukhtar M: Malignant transformation of vaginal endometriosis. Obstet Gynecol 1984;64:592-595.
Kapp DS, Merino M, LiVolsi V: Adenocarcinoma of the vagina arising in endometriosis: long-term survival following radiation therapy. Gynecol Oncol 1982;14:271-278.
Bamford DS: Primary adenocarcinoma of the vagina. Proc R Soc Med 1967;60:999-1000.
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