The valid evidence to reveal the effect of sex hormone on primary retroperitoneal malignant mixed müllerian tumor: a case report

Extragenital malignant mixed Müllerian tumor (MMMT) is rare, and the retroperitoneum is one of the rarest sites. The etiology and risk factors are yet ill-understood. The authors report a case of primary retroperitoneal Müllerian carcinosarcoma in a pregnant woman. Initially, a pure cystic mass was found when she was four months’ pregnant. After drainage of the cyst, the follow-up ultrasound detected rapid recovery of the cyst, the appearance of septation within the cyst, blood flow signal in the septation, more and thickened septation, and nodules on the septation and cystic wall with progressing pregnancy. After delivery, the follow-up imaging examination revealed a relatively dormant mass. The contrary clinical manifestations were consistent with sex hormone levels, which surged during pregnancy and dropped rapidly postpartum. Moreover, immunohistochemistry staining showed that both ER and PR were positive for the epithelial and stromal component. Given the clinical manifestations and pathology, for the first time, the authors can explicitly conclude that sex hormone played a direct and evident role in the development of extragenital malignant mixed Müllerian tumor.

Malignant mixed Müllerian tumor; Sex hormone; Retroperitoneum; Hormonal receptor status

[1] Garde J.R., Jones M.A., McAfee R., Tarraza H.M.: “Extragenital malignant mixed müllerian tumor: review of the literature”. Gynecol. Oncol., 1991, 43, 186.

[2] Booth C., Zahn CM., McBroom J., Maxwell G.L.: “Retroperitoneal müllerian carcinosarcoma associated with endometriosis, a case report”. Gynecol. Oncol., 2004, 93, 546.

[3] Tanaka K., Kobayashi Y., Shibuya H., Nishigaya Y., Momomura M., Matsumoto H., Iwashita M.: “Primary retroperitoneal Müllerian adenocarcinoma arising from endometriosis”. J. Obstet. Gynaecol. Res., 2014, 40, 1823.

[4] Fotiou S., Hatjieleftheriou G., Kyrousis G., Kokka F., Apostolikas N.: “Long-term tamoxifen treatment: a possible aetiological factor in the development of uterinecarcinosarcoma, two case-reports and review of the literature”. Anticancer Res., 2000, 20, 2015.

[5] McCluggage W.G., Abdulkader M., Price J.H., Kelehan P., Hamilton S., Beattie J., Al-Nafussi A.: “Uterine carcinosarcomas in patients receiving tamoxifen. A report of 19 cases”. Int. J. Gynecol. Cancer, 2000, 10, 280.

[6] Hubalek M., Ramoni A., Mueller-Holzner E., Marth C.: “Malignant mixed mesodermal tumor after tamoxifen therapy for breast cancer”. Gynecol. Oncol., 2004, 95, 264.

[7] Curtis R.E, Freedman D.M., Sherman M.E., Fraumeni J.F. Jr.: “Risk of malignant mixed mullerian tumors after tamoxifen therapy for breast cancer”. J. Natl. Cancer Inst., 2004, 96, 70.

[8] Chumas J.C., Thanning L., Mann W.J.: “Malignant mixed mullerian tumor arising in extragenital endometriosis, report of a case and review of the literature”. Gynecol. Oncol., 1986, 23, 227.

[9] Fujii S.: “Secondary müllerian system and endometriosis”. Am. J. Obstet. Gynecol., 1991, 165, 219.

[10] Ferrie R.K., Ross R.C.: “Retroperitoneal Müllerian carcinosarcoma”. Can. Med. Assoc. J., 1967, 97, 1290.

[11] Teng C.S., Teng C.T.: “Study on sex-organ development. Oestrogen-receptor translocation in the developing chick Müllerian duct”. Biochem. J., 1976, 154, 1.

[12] Shi H., Chen X., Lv B., Zhang X.: “Concurrent tamoxifen-related Müllerian adenofibromas in uterus and ovary”. Int. J. Clin. Exp. Pathol., 2015, 8, 15381.

[13] Hu R., Hilakivi-Clarke L., Clarke R.: “Molecular mechanisms of tamoxifen-associated endometrial cancer (Review)”. Oncol. Lett., 2015, 9, 1495.

[14] Seagle B.L., Falter K.J. 2nd., Lee S.J., Frimer M., Samuelson R., Shahabi S.: “Mullerian adenosarcoma of the cervix, Report of two large tumors with sarcomatous overgrowth or heterologous elements”. Gynecol. Oncol. Case Rep., 2014, 9, 7.

[15] Huang G.S., Arend R.C., Sakaris A., Hebert T.M., Goldberg G.L.: “Extragenital adenosarcoma, a case report, review of the literature, and management discussion”. Gynecol. Oncol., 2009, 115, 472.

[16] Ansink A.C., Cross P.A., Scorer P., de Barros Lopes A., Monaghan J.M.: “The hormonal receptor status of uterine carcinosarcomas (mixed müllerian tumours): animmunohistochemical study”. J. Clin. Pathol., 1997, 50, 328.