A rare case of primary ovarian leiomyosarcoma

Primary ovarian leiomyosarcoma (LMS) is very rare, with only 72 cases reported. Its behavior is aggressive, and there is no standard therapy. A 62-year-old woman with a giant pelvic tumor underwent CT and MRI. An irregular, 30-cm, solid tumor that was suspected to be malignant was seen. On laparotomy, the tumor was derived from her right ovary and adhered widely to the retroperitoneum. On pathological examination, it was an ovarian leiomyosarcoma. Adjuvant chemotherapy (docetaxel and gemcitabine) was given. Three months after surgery, CT showed multiple liver metastases. Pazopanib, ifosfamide, doxorubicin, and eribulin were given with no effect, and the woman died 18 months after the primary operation. With the administration of pazopanib, proteinuria of 2.3 g/dl occurred, which made continuous administration impossible. Since ovarian LMS may not respond to chemotherapy for other soft tissue sarcomas, study of more ovarian LMS cases is needed.

Primary ovarian leiomyosarcoma (LMS; Laparotomy; Adjuvant chemotherapy; Soft tissue sarcomas

[1] Dixit S., Singhal S., Baboo H.A., Vyas R.K., Neema J.P., Murthy R., Sooryanaraya U.: “Leiomyosarcoma of the ovary”. J. Postgrad. Med., 1993, 39, 151.

[2] Tsuyoshi H., Yoshida Y., Kurrokawa T., Kotsuji F.: “Diagnosis and management of leiomyosarcoma arising from ovarian vein: case report and literature review”. J. Obstet. Gynaecol. Res., 2012, 38, 466.

[3] He M., Deng Y.J., Zhao D.Y., Zhang Y., Wu T.: “Synchronous leiomyosarcoma and fibroma in a single ovary: A case report and review of the literature”. Oncol. Lett., 2016, 11, 2510.

[4] Bacalbasa N., Taras C., Orban C., Iliescu L., Hurjui I, Hurjui M., et al.: “Atypical right hepatectomy for liver metastasis from ovarian leiomyosarcoma

[5] Rasmussen C.C., Skilling J.S., Sorosky J.I., Lager D.R., Buller R.E.: “Stage IIIC ovarian leiomyosarcoma in a premenopausal woman with multiple recurrences: prolonged survival with surgical therapy”. Gynecol. Oncol., 1997, 66, 519.

[6] Anderson B., Turner D.A., Benda J.: “Ovarian sarcoma”. Gynecol. Oncol., 1987, 26, 183.

[7] Taskin S., Taskin E.A., Uzum N., Ataoglu O., Ortac F.: “Primary ovarian leiomyosarcoma. A review of the clinical and immunohistochemical features of the rare tumors”. Obstet. Gynecol. Surv., 2007, 62, 480.

[8] Monk B.J., Nieberg R., Berek J.S.: “Primary leiomyosarcoma of the ovary in a perimenarchal female”. Gynecol. Oncol., 1993, 48, 389.

[9] Lerwill M.F., Sung R., Oliva E., Prat J., Young R.H.: “Smooth muscle tumors of the ovary: A clinicopathologic study of 54 cases emphasizing prognostic criteria, histologic variants, and differential diagnosis”. Am. J. Surg. Pathol., 2004, 28, 1436.

[10] Hensley M.L., Barrette B.A., Baumann K., Gaffney D., Hamilton A.L., Kim J.W., et al.: “GCIG Consensus Review: Uterine and ovarian leiomyosarcomas”. Int. J. Gynecol. Cancer, 2014, 24, S61.

[11] Koh W.J., Greer B.E., Abu-Rustum N.R., Apte S.M., Campos S.M., Cho K.R., et al.: “Uterine Sarcoma, Version 1.2016: Featured Updates to the NCCN Guidelines”. J. Natl. Compr. Canc. Netw., 2015, 13, 1321.

[12] Judson I., Verweij J., Gelderblom H., Hartmann J.T., Schöffski P., Blay J.Y., et al.: “Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial”. Lancet Oncol., 2014, 15, 415.

[13] Leyvraz S., Zweifel M., Jundt G., Lissoni A., Cerny T., Sessa C., et al.: “Long-term results of a multicenter SAKK trial on high-dose ifosfamide and doxorubicin in advanced or metastatic gynecologic sarcomas”. Ann. Oncol., 2006, 17, 646.

[14] Sagae S., Yamashita K., Ishioka S., Nishioka Y., Terasawa K., Mori M., et al.: “Preoperative diagnosis and treatment results in 106 patients with uterine sarcoma in Hokkaido, Japan”. Oncology, 2004, 67, 33.

[15] Yamagami W., Susumu N., Ninomiya T., Kuwahata M., Takigawa A., Nomura H., et al.: “A retrospective study on combination therapy with ifosfamide, adriamycin and cisplatin for progressive or recurrent uterine sarcoma”. Molecul. Clin. Oncol., 2014, 2, 591.

[16] van der Graaf W.T., Blay J.Y., Chawla S.P., Kim D.W., Bui-Nguyen B., Casali P.G., et al.: “Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial”. Lancet 2012, 379, 1879.

[17] Demetri G.D., von Mehren M., Jones R.L., Hensley M.L., Schuetze S.M., Staddon A., et al.: “Efficacy and safety of trabectedin or dacarbazine for metastatic liposarcoma or leiomyosarcoma after failure of conventional chemotherapy: results of a phase III randomized multicenter clinical trial”. J. Clin. Oncol., 2016, 34, 786.

[18] Schöffski P., Chawla S., Maki R.G., Italiano A., Gelderblom H., Choy E., et al.: “Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomized, open-label, multicenter, phase 3 trial”. Lancet, 2016, 387, 1629.

[19] Sleifer S., Ray-Coquard I., Papai Z., Le Cesne A., Scurr M., Schöffski P., et al.: “Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: A phase 2 study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC Study 62043)”. J. Clin. Oncol., 2009, 27, 3126.