Primary vaginal endometrial stromal sarcoma associated with endometriosis: a case report with a review of the literature

Extrauterine endometrial stromal sarcomas (ESSs) are quite rare tumors, and vagina is an unusual site for these tumors. This paper presents a very rare pathological entity of primary vaginal ESS. A 46-year-old woman with a complaint of postcoital vaginal bleeding, low abdominal pain, and constipation was admitted to the clinic. She had a mass of seven cm in size, located in the posterior fornix detected on physical examination. The preoperative biopsy showed ESS, surgical material, and evaluation of an endometrium confirmed the diagnosis of primary vaginal ESS. She underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and partial vaginectomy. The diagnosis of ESS performed by pathologic and immunohistochemical evaluation was: caldesmon (-), actin (-), desmin (-). CD10 (+), ER (+), PR (+), and vimentin (+). There was no ESS lesion in the endometrium. The patient was free of tumor for 22 months after the surgery without any additional therapy. In this study, the authors report the sixth case of primary vaginal ESS in the literature and aim to discuss diagnostic criteria and management protocols in the light of the literature.

Endometrial stromal sarcoma; Extrauterine; Extragonadal; Vaginal tumors; Uterine sarcoma.

[1] Tse K.Y., Crawford R., Ngan H.Y.: “Staging of uterine sarcomas”. Best Pract Res Clin Obstet Gynaecol., 2011, 25, 733.

[2] Tavassoli F.A., Devilee P.: “WHO classification of pathology and genetics of tumours of the breast and female genital organs”. Lyon France: IARC Press, 2003, 233.

[3] Masand R., Euscher E., Deavers M., Malpica A.: “Extrauterine endometrial stromal sarcoma: A pathologic study of 63 cases with clinical correlation”. Am. J. Surg. Pathol., 2013, 37, 1635.

[4] Yantiss R.K., Clement P.B., Young R.H.: “Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: a study of 17 cases”. Am. J. Surg. Pathol., 2000, 24, 513.

[5] Berkowitz R.S., Ehrmann R.L., Knapp R.C:. “Endometrial stromal sarcoma arising from vaginal endometriosis”. Obstet. Gynecol., 1978, 51, 34s.

[6] Kondhi-Paphtis A., Smirniotis B., Liapis A., Kontoyanni A., Deligeorgi H.: “Stromal sarcoma arising on endometriosis. A clinico-Gynaecol. Oncol., 1998, 19, 588.

[7] Chang Y.C., Wang T.Y., Tzen C.Y.: “Endometrial stromal sarcoma of the vagina”. Zhonghua Yi Xue Za Zhi (Taipei), 2000, 63, 714.

[8] Corpa M.V., Serafini E.P., Bacchi C.E.: “Low-grade endometrial stromal sarcoma presenting as vaginal nodule”. Ann. Diagn. Pathol., 2004, 8, 295.

[9] Liu Z., Ding J., Li X., Yu K.: “Endometrial stromal sarcoma arising in vagina”. Int. J. Clin. Exp. Pathol., 2013, 6, 2997.

[10] Stemme S., Ghaderi M., Carlson J.W.: “Diagnosis of endometrial stromal tumors: a clinicopathologic study of 25 biopsy specimens with identification of problematic areas”. Am. J. Clin. Pathol., 2014 , 141, 133.

[11] Verit F.F., Yucel O.: “Endometriosis, leiomyoma and adenomyosis: the risk of gynecologic malignancy”. Asian Pac. J. Cancer Prev., 2013, 14, 5589.

[12] Sampson J.A.: “Endometrial carcioma of the ovary arising inendometrial tissue in that organ”. Arch. Surg.,1925, 10, 1.

[13] Scott R.B.: “Malignant changes in endometriosis”. Obstet. Gynecol., 1953, 2, 283.

[14] Shah J.P., Bryant C.S., Kumar S., Ali-Fehmi R., Malone J.M. Jr., Morris R.T.: “Lymphadenectomy and ovarian preservation in lowgrade endometrial stromal sarcoma”. Obstet. Gynecol., 2008, 112, 1102.

[15] Amant F., Coosemans A., Debiec-Rychter M., Timmerman D., Vergote J.: “Clinical management of uterine sarcomas”. Lancet Oncol., 2009, 10, 1188.

[16] Li A.J., Giuntoli R.L. II, Drake R., Byun S.Y., Rojas F., Barbuto D., et al.: “Ovarian preservation in stage I low-grade endometrial stromal sarcomas”. Obstet. Gynecol., 2005, 106, 1304.

[17] Amant F., De K.A., Van C.B., Leunen K., Neven P., Berteloot P., et al.: “Clinical study investigating the role of lymphadenectomy, surgical castration and adjuvant hormonal treatment in endometrial stromal sarcoma”. Br. J. Cancer, 2007, 97, 1194.

[18] Chan J.K., Kawar N.M., Shin J.Y., Osann K., Chen L.M., PowellC.B., Kapp D.S.: “Endometrial stromal sarcoma: a populatio n-based analysis”. Br. J. Cancer, 2008, 99, 1210.