Uterine Myxoid Leiomyosarcoma with Paraneoplastic Syndrome: A Rare Combination

Abstract

Myxoid leiomyosarcoma of uterus is a very rare malignancy with only 36 cases documented worldwide and no cases reported in the Indian subcontinent. The present case is of uterine myxoid leiomyosarcoma with associated pancytopenia that has not been described previously in the south-east asia and only one such case reported in world literature. A 32 year old woman who is a known case of uterine fibromyoma discovered in her previous pregnancy , presented to the opd with heavy bleeding per vagina. On examination, she had a large abdominal mass. Her hemoglobin was 6.3 g/dl and platelet count was 80,000/cu.mm and also developed leucopenia(4500/cu.mm).She had received five units of blood transfusion, but her haemoglobin and platelet counts remained low. Her bone marrow biopsy report came out to be inconclusive. She underwent laparotomy followed by total hysterectomy with bilateral salphingoophorectomy, omentectomy and appendicectomy. Histopathology reports showed uterine myxoid leiomyosarcoma ( FIGO stage IIIA). Her haematological profile improved considerably following the surgery and recurred with the recurrence of the disease. Hence, this is considered to be a case of pancytopenia due to paraneoplastic syndrome as a result of the myxoid leiomyosarcoma of the uterus.