spontaneous Regression of Stage IV Clear Cell Carcinoma of the Endometrium in a Patient with Essential Thrombocytosis

Case Details

Disease Location

Endometrium (uterus)

Personal Characteristics

73 -year-old woman history of essential thrombocytosis diagnosed 5 year previously, treated with hydroxyurea and remained well until 3 months before diagnosis of endometrial carcinoma

Clinical Characteristics

Presented with vaginal bleeding, weakness, occasional nausea, vomiting and a 13kg weight loss over 2 months she was found to be anemic which was believed secondary to dydrozyurea toxicity, she was transfused and hydroxyurea discontinued, platelet count was 2000x10^9/l at presentation physical exam found uterus indurated and firm ca-125 serum tumor marker was elevated and ca 19-9 abdominal and pelvic ultrasound, and MRI found a large complex pelvic mass associated with the uterus as well as a 4cm mass anterior to the lwoer pole of the right kidney and a 5cm mass inferior to the umbilicus the 5cm mass was biopsied via fna and revealed papillary carcinoma diagnosis of extensively necrotic clear cell endometrial carcinoma 5 weeks after fna biopsy, patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy operative findings included an enlarged firm uterus with a complex mass in the cul de sac and two small <2cm omental nodules, both ovaries were hard but not enlarged pathologic exam of the endometrial tumor revealed almost complete replacement by dense hyalinized material, chronic inflammatory cells and histiocytes and psammomma bodies, small microscopic foci of viable high-grade clear cell carcinoma were seen a similar tumor, largely replaced by hyalinized and inflamed stroma was seen involving serosal aspect of the uterus, left and right adnexae and the omentum

Remission Characteristics

Complete tumor necrosis of primary endometrial tumor and metastatic deposits in the adnexae and omentum, and apparent complete regression of abdominal disease (near right kidney and umbilicus) via imaging and fna serum ca-125 and 19-9 levels returned to normal she remains alive and clinically free 6 years later

Treatment & Mechanisms

Proposed Remission Mechanisms

Host immune response, hormonal factors, or removal of preexisting carcinogens compromise of the vascular supply to the tumor via biopsy or excision, rapid tumor growth, thrombosis perhaps the rise in platelet # after hyearsdoxyurea discontinuation is related

Clinical Treatment

Total abdominal hysterectomy, bilateral salpingo-oophorectomy and e monthstectomy

Non-Clinical Treatment

None reported