Paraneoplastic consumptive coagulopathy related to intramyometrial low-grade endometrial stromal sarcoma coexistent with adenomyosis diagnosed 7 years after laparoscopic-assisted myomectomy

Introduction Paraneoplastic coagulation disorders associated with benign and malignant gynecological tumors have been reported. However, reports on cutaneous purpural manifestation of paraneoplastic coagulopathy in cases of gynecological tumors are extremely limited. Case report A 40-year-old woman was referred due to generalized ecchymosis 7 years after laparoscopic-assisted myomectomy. Coagulogram was markedly deranged with severe thrombocytopenia. After hematological disorders were excluded, image diagnostic modalities showed multiple intramyometrial heterogeneous mass lesions with extensive intratumoral hemorrhage. Laparoscopic-assisted vaginal hysterectomy was performed with supplementation of coagulation factors and platelets. Histological examination showed intramyometrial low-grade endometrial stromal sarcoma (ESS) coexistent with adenomyosis. Coagulation disorder immediately disappeared after hysterectomy and the postoperative course was uneventful. Recurrence of either ESS or coagulopathy has not been noted 15 months postoperatively, to date. Conclusions This case illustrates a rare but potentially life-threatening consumptive coagulopathy caused by intramyometrial low-grade ESS that was assumed to secondarily arise from stromal cells of adenomyosis developed in postmyomectomy scar.