A case of extremely chemoresistant pure pleomorphic rhabdomyosarcoma of the uterus associated with a high serum LDH level

Background: Pleomorphic rhabdomyosarcoma (RMS) of gynecologic origin is an exceedingly rare, highly malignant tumor. Only a few cases have been reported in the last decades. Case report: A 60-year-old postmenopausal woman sa presented with a high level of unknown origin. Ultimately, she was diagnosed with pleomorphic RMS. She underwent total hysterectomy, bilateral salpingo-oophorectomy, left pelvic and paraaortic lymphadenectomy and partial omentectomy. Surgery was followed by systemic c hemotherapy and pelvic irradiation. Unfortunately, the patient did not respond to treatment. Her disease course cot-related with the fluctuation of plasma LDH levels. Ultimately she died within 20 months of the diagnosis. Conclusion: It is important to have better insight and to set a standard multimodal treatment for adult RMS. In addition, plasma LDH levels can be considered as a prognostic marker for RMS, particularly in advanced stage.

Uterine sarcoma; Uterine rhabdomyosarcoma; Lactate dehydrogenase

[1] Little D.J., Ballo M.T., Zagars G.K., Pisters P.W., Patel S.R., El-Naggar A.K. et al.: “Adult rhabdomyosarcoma: outcome following multimodality treatment”. Cancer, 2002, 95, 377.

[2] Holcomb K., Francis M., Ruiz J., Abulafia O., Matthews R.P., Lee Y.C.: “Pleomorphic rhabdomyosarcoma of the uterus in a postmenopausal woman with elevated serum CA125”. Gynecol. Oncol., 1999, 74, 499.

[3] Borka K., Patai K., Rendek A., Sobel G., Paulin F.: “Pleomorphic rhabdomyosarcoma of the uterus in a postmenopausal patient”. Pathol. Oncol. Res., 2006, 12, 102.

[4] Ferrari A., Dileo P., Casanova M., Bertulli R., Meazza C., Gandola L. et al.: “Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution”. Cancer, 2003, 98, 571.

[5] Sleijfer S., Seynaeve C., Verweij J.: “Gynaecological sarcomas”. Current opinion in Oncology, 2007, 19, 492.

[6] Parham D.M.: “Pathologic classification of rhabdomyosarcomas and correlations with molecular studies”. Mod. Pathol., 2001, 14, 506.

[7] Cormier J.N., Pollock R.E.: “Soft tissue sarcomas”. CA: a cancer journal for clinicians, 2004, 54, 94.

[8] Ferguson S.E., Gerald W., Barakat R.R., Chi D.S., Soslow R.A.: “Clinicopathologic features of rhabdomyosarcoma of gynecologic origin in adults”. Am. J. Surg. Pathol., 2007, 31, 382.

[9] Case A.S., Kirby T.O., Conner M.G., Huh W.K.: “A case report of rhabdomyosarcoma of the uterus associated with uterine inversion”. Gynecol. Oncol., 2005, 96, 850.

[10] Emerich J., Senkus E., Konefka T.: “Alveolar rhabdomyosarcoma of the uterine cervix”. Gynecol. Oncol., 1996, 63, 398.

[11] Hawkins W.G., Hoos A., Antonescu C.R., Urist M.J., Leung D.H., Gold J.S. et al.: “Clinicopathologic analysis of patients with adult rhabdomyosarcoma”. Cancer, 2001, 91, 794.

[12] Breitfeld P.P., Lyden E., Raney R.B., Teot L.A., Wharam M., Lobe T. et al.: “Ifosfamide and etoposide are superior to vincristine and melphalan for pediatric metastatic rhabdomyosarcoma when administered with irradiation and combination chemotherapy: a report from the Intergroup Rhabdomyosarcoma Study Group”. J. Pediatr. Hematol. Oncol., 2001, 23, 225.

[13] Moritake H., Kamimura S., Akiyoshi K., Nagatoshi Y., Chuman H., Okamura J.: “Prognostic significance of elevated lactate dehydrogenase and creatine kinase in patients with rhabdomyosarcoma”. Med. Pediatr. Oncol., 2002, 40, 187.