Undifferentiated pleomorphic sarcoma with focally rhabdomyosarcomatous differentiation of the ovary

Purpose of investigation: Primary sarcomas account for of 2-3% of all female genital tract cancers and ovaries are unusual sites for sarcomas. The authors aimed to report a case of primary ovarian sarcoma. Materials and Methods:A 46-year-old woman presented with abdominal pain and distention. Abdominal MRI revealed a 20-cm-sized complex mass in the right adnexa and ascites. Preoperative CA-125 was 289 U/ml. She underwent optimal debulking surgery and diagnosed with undifferentiated pleomorphic sarcoma with focally rhabdomyosarcomatous differentiation. Results: The patient presented with severe abdominal pain and distention 32 days after the operation and recurrent disease was diagnosed. First cure of adjuvant chemotherapy was administered, but she died 15 days later. Conclusion: Coexistence of undifferentiated pleomorphic sarcoma and rhabdomyosarcoma shows highly aggressive behavior and its prognosis is extremely poor. To the best of the authors' knowledge, this is the second case report of the coexistence of these type tumors in the literature.

Undifferentiated pleomorphic sarcoma; Rhabdomyosarcoma; Ovary.

[1] Leppien G.: “Non-uterine gynecological sarcomas.” Arch. Gynecol. Obstet., 1987, 241, 25.

[2] Talerman A., Auerbach W.M., van Meurs A.J.: “Primary chondrosarcoma of the ovary.” Histopathology, 1981, 5, 319.

[3] Yeasmin S., Nakayama K., Ishibashi M., Katagiri A., Iida K., Manabe A., et al.: “Primary osteosarcoma of ovary”. Int. J. Clin. Oncol., 2009, 14, 163.

[4] Quesenberry C.D., Li C., Chen A.H., Zweizig S.L., Ball H.G. 3rd., et al.: “Primary angiosarcoma of the ovary: a case report of Stage I disease.” Gynecol Oncol., 2005, 99, 218.

[5] Kransdorf M.J., Murphey M.D.: “Malignant fibrous and fibrohistiocytic tumors”. In: Kransdorf M.J., Murphey M.D., (eds). Imaging of soft tissue tumors. Philadelphia: Lippincott Williams&Wilkins, 2006, 257.

[6] Mukherjee S., Sen S., Biswas P., Choudhuri M.: “Primary pleomorphic sarcoma of the ovary with rhabdomyosarcomatous differentiation.” Indian J. Pathol. Microbiol., 2009, 52, 217.

[7] Choi SY, Kim TH, Park HS, Sung CO, Lee JW, Song SY, et al.: “Myxoid malignant fibrous histiocytoma of the ovary: a case report.” Int J Clin Oncol., 2011, 16, 601-4.

[8] Dilek T.U., Dilek S., Pata O., Tataroglu C., Tok E.: “Malignant fibrous histiocytoma of the ovary: a case report.” Int. J. Gynecol. Cancer, 2006, 16, 352.

[9] Stefanovic A., Stojnic J., Jeremic K., Jeftovic M., Arsenijevic L., Zecevic N., et al.: “Malignant fibrous histiocytoma of the ovary: a case report.” Eur. J. Gynaecol. Oncol., 2012, 33, 236.

[10] Yamakawa Y., Fujimura M., Hidaka T., Yasoshima K., Saito S.: “Inflammatory malignant fibrous histiocytoma of the ovary producing interleukin- 6: A case report.” Gynecol Oncol., 1999, 75, 484.

[11] Roque D.M., Jones D.F., Carter G., Kelley J.L.: “Primary giant cell malignant fibrous histiocytoma of the ovary: case report and review of the literature”. Gynecol. Oncol., 2010, 119, 397.

[12] Zagars G.K., Mullen J.R., Pollack A.: “Malignant fibrous histiocytoma: outcome and prognostic factors following conservation surgery and radiotherapy”. Int. J. Radiat. Oncol. Biol. Phys., 1996, 34, 983.

[13] Pezzi C.M., Rawlings M.S. Jr., Esgro J.J., Pollock R.E., Romsdahl M.M.: “Prognostic factors in 227 patients with malignant fibrous histiocytoma”. Cancer, 1992, 69, 2098.