Rare late mandibular recurrence of uterine leiomyosarcoma: a case report

Sarcomas represent 1‒2% of all uterine malignancies and leiomyosarcomas (LMSs) are the most common histological subtype, accounting for nearly 30% of all uterine sarcomas. The diagnosis is incidental following routine hysterectomy, and the disease exhibits a high rate of relapse and distant metastases. The most common location for metastasis is the lung, while the finding of bone metastasis in the oral region is exceedingly rare. Here the authors report the case of an exceedingly rare mandibular disease recurrence from a previously uterine LMS refractory to multiple treatments, which was beneficially managed by systemic trabectedin administration. The authors also discuss the features connected with the diagnosis and management of such an uncommon presentation of the metastatic disease.

Leiomyosarcoma; Mandibular; Recurrence; Trabectedin.

[1] Brooks S.E., Zhan M., Cote T., Baquet C.R.: “Surveillance, epidemiology and end results analysis of 2677 cases of uterine sarcoma 1989-1999”. Gynecol. Oncol., 2004, 93, 204.

[2] Prat J., Mbatani N.: “Uterine sarcomas”. International Journal of Gynecol.Obstet., 2015, 131, S105.

[3] Ferreira M., Malpica A.: “Metastatic uterine leiomyosarcoma to the submandibular gland”. Ann. Diagn. Pathol., 2009, 13, 208.

[4] Dizon D.S., Birrer M.J.: “Advances in the diagnosis and treatment of uterine sarcomas”. Discov. Med., 2014, 17, 339.

[5] Kapp D.S., Shin J.Y., Chan J.K.: “Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy”. Cancer, 2008, 112, 820.

[6] Oláh K.S., Dunn J.A., Gee H: “Leiomyosarcomas have a poorer prognosis than mixed mesodermal tumours when adjusting for known prognostic factors: the result of a re.trospective study of 423 cases of uterine sarcoma”. Br. J. Obstet. Gynaecol., 1992, 99, 590.

[7] Berchuck A., Rubin S.C., Hoskins W.J., Saigo P.E., Pierce V.K., Lewis J.L. Jr.: “Treatment of uterine leiomyosarcoma”. Obstet. Gynecol., 1988, 71, 845.

[8] Sutton G., Blessing J.A., Malfetano J.H.: “Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a Gynecologic Oncology Group study”. Gynecol. Oncol., 1996, 62, 226.

[9] Pusceddu C., Capobianco G., Valle E., Dessole S., Cherchi P.L., Meloni G.B.: “Computed tomography-guided cryoablation of pelvic metastasis from uterine leiomyosarcoma”. Int J Gynaecol Obstet, 2011, 114, 87.

[10] Cassoni A., Terenzi V., Bartoli D., Zadeh O.R., Battisti A., Pagnoni M., et al.: “Metastatic uterine leiomyosarcoma in the upper buccal gingiva misdiagnosed as an epulis”. Case Rep. Oncol. Med., 2014, 2014, 402342.

[11] El-Khalfaoui K., du Bois A., Heitz F., Kurzeder C., Sehouli J., Harter P.: “Current and future options in the management and treatment of uterine sarcoma”. Ther. Adv. Med. Oncol., 2014, 6, 21.

[12] Amant F., Lorusso D., Mustea A., Duffaud F., Pautier P.: “Management Strategies in Advanced Uterine Leiomyosarcoma: Focus on Trabectedin”. Sarcoma, 2015, 2015, 704124.

[13] Maki R., Wathen K., Patel S., Priebat D., Okuno S., Samuels B., et al.: “Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: result of sarcoa Allianace for Research Through Collaboration Study 002”. J. Clin. Oncol., 2007, 25, 2755.

[14] Anderson S., Aghajanian C.: “Temozolomide in uterine leiomyosarcomas”. Gynecol Oncol, 2005, 98, 99.

[15] Miolo G., Viel A., Canzonieri V., Baresic T., Buonadonna A., Santeufemia D.A., Lara D.P., Corona G.: “Association of the germline BRCA2 missense variation Glu2663Lys with high sensitivity to trabectedin-based treatment in soft tissue sarcoma”. Cancer Biol. Ther., 2016, 17, 1017.

[16] Monk B.J., Blessing J.A., Street D.G., Muller C.Y., Burke J.J., Hensley M.L.: “A phase II evaluation of trabectedin in the treatment of advanced, persistent, or recurrent uterine leiomyosarcoma: A gynecologic oncology group study”. Gynecol. Oncol., 2012, 124, 48