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Primary malignant mixed müllerian tumor of the peritoneum a case report with review of the literature

DOI: 10.1186/1477-7819-9-17

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Malignant mixed Müllerian tumor (MMMT) is a rare entity that arises from structures that are embryologically related to the Müllerian system [1,2]. The usual location of MMMT is the female genital tract. Extragenital origin is extremely rare [3-5]. Histologically and by immunohistochemistry, the tumor exhibits both epithelial and mesenchymal components.Since the first report in 1955 by Ober and Black [5], to our knowledge there have been only 30 well documented reports of extragenital malignant mixed Müllerian tumors [6-15]. This prompted us to report on a tumor with primary peritoneal location and to review the relevant existing literature.A 72-year-old woman, with unremarkable gynaecological history presented with chest pain and dyspnoea, increasing in intensity over the last three weeks. A chest X-ray showed pleural effusion and the subsequent fine needle aspiration cytology revealed malignant epithelial cells. The serum level of CA125 was 712 U/ml.CT scan of abdomen and chest revealed ascites and pleural effusion but no tumor mass. Pelvic ultrasonography, however, revealed excrescences adjacent to the interior surface of the abdominal wall and tumor load in the lower part of abdomen. The uterus and the right ovary were described as normal; the left ovary was not visualised.An ultrasound-guided biopsy from the tumor reported a carcinosarcoma (see later).The patient underwent exploratory laparotomy. A widely spread peritoneal carcinosis and a tumor measuring 20 × 15 × 10 cm in the vesicouterine and Duglas' pouch were found.Biopsy samples were taken from the tumor as well as from the serosa of the urinary bladder. Also, complete hysterectomy with partial omentectomy was performed. There was no suspicion of intrahepatic metastasis. Gallbladder, stomach, pancreas and appendix were unremarkable.Histopathology was consistent with the diagnosis of a primary peritoneal malignant mixed Müllerian tumor given that the uterus and the adnexa were unremarkable.Postoperatively,


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