A giant mesentery malignant solitary fibrous tumor recurring as dedifferentiated liposarcoma- a report of a very rare case and literature review.

We report a case of a 59-year-old woman with a very rare giant mesentery malignant solitary fibrous tumor that recurred as dedifferentiated liposarcoma. The woman was admitted to the hospital because of low abdominal pain. Radiological and biopsy findings revealed a multi-lobulated giant malignant solitary fibrous tumor that had invaded the inferior vena cava, abdominal aorta, and superior mesentery vessels. The tumor was completely removed during the first cytoreductive surgery. Histopathologically, tumor had a heterogeneous cell population, composed of spindle cells with fibrous collagen proliferation. The spindle cells were not arranged in a specific pattern. Immunohistochemistry revealed that the tumor cells were positive for CD34, CD99, Bcl-2, and smooth muscle actin( SMA) and negative for CD117, epithelial membrane antigen (EMA), CAM5.7, S100, desmin, and caldesmon. The tumor recurred 9 months after surgery, and another cytoreductive surgery was then performed. The postoperative histopathological appearance of the invaded area indicated a well-differentiated liposarcoma. Formation of tumorous bone was also noted in the same area, in addition to atypical mesenchymal cells and multi-vacuolated lipoblasts in the area of the well-differentiated liposarcoma. Proliferated spindle cells arranged in a storiform pattern were found in the area adjacent to the tumor. Immunohistochemical analysis revealed that the tumors cells were positive for SMA, HHF-35, and caldesmon and negative for CD117, CD34, and S100. A diagnosis of dedifferentiated liposarcoma was made.

Treatment results of diffuse malignant peritoneal mesothelioma.

During the last 7 years, 21 patients with DMPM were treated. Histologic types were epitheloid type in 18 patients, biphasic type in 2 patients and sarcomatoid type in 1 patient. Preoperative systemic chemotherapy, hyperthermic intraperitoneal chemotherapy(HIPEC) by laparoscopy(LHIPEC), and intraperitoneal(IP) chemotherapy were done in 14, 3 and 1 patients, respectively. Cytoreductive surgery(CRS) was done in 13 patients. Ten patients received HIPEC after CRS. Partial responses were experienced in 4 of 13 patients treated with preoperative systemic chemotherapy. One of three patients treated by LHIPEC showed complete response. Among 13 patients received laparotomy, complete removal of PC was done in 4(31%) patients. The other 9 patients who received incomplete cytoreduction had diffuse involvement on the small bowel and its mesentery. All over 5-year survival was 17%. Patients treated with HIPEC survived significantly longer than non-HIPEC group. Neoadjuvant laparoscopic HIPEC may have a great role in the preoperative control of small PC on the surface of small bowel.

Management of peritoneal dissemination of recurrences granulosa cell tumor of the ovary.

Recurrences of granulosa cell tumor (GCT) of the ovary often occur as disseminated peritoneal metastasis or local mass in the pelvis. Although, various treatment options including surgery with/without systemic chemotherapy and/or radiotherapy have also been reported for treatment of recurrent GCT, there is no standardized management for recurrence of this disease. Here, we report our management strategies for the patients with peritoneal dissemination of GCT. Prior to admission to our unit, four patients were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy with the diagnosis of primary adult type GCT of the ovary. They were not received adjuvant therapy because of the localized disease in the ovary and followed-up by their gynecologists until they referred to us with metastases. The median disease free survival after primary treatment was 4.7 (range, 1-9) years. All patients with peritoneal metastases from recurrent GCT were treated with cytoreductive surgery (CRS) using peritonectomy procedures and intraoperative hyperthermic intraperitoneal chemotherapy(HIPEC) using 100 mg cisplatin for 40 min at 43 °C in our unit. Postoperative complications were graded according to National Cancer Institute's Common Toxicity Criteria. No complication and no in-hospitalization mortality were experienced in all patients. The median length of operation was 3.55 (range, 2.50-5.50) hours. The median length of stay in hospital was 13(range, 12-21) days. After a median follow-up of 4(range, 1-6) years, 1 patient was died and other 3 patients were alive with no disease progression. Our study identified that recurrent adult type of GCT with peritoneal metastases could be managed with definitive CRS and HIPEC. Larger series and long term outcome data of CRS and HIPEC will be mandatory to develop standard management option in these patients.