A Case of Ovarian Fibromatosis and Massive Ovarian Oedema Associated With Intra-Abdominal Fibromatosis, Sclerosing Peritonitis and Meig's Syndrome

Purpose:To discuss a case of ovarian fibromatosis/massive ovarian oedema, intra-abdominal fibromatosis, sclerosing peritonitis and Meig's syndrome. To review the reported therapeutic options

Adipocyte-specific protein tyrosine phosphatase 1B deletion increases lipogenesis, adipocyte cell size and is a minor regulator of glucose homeostasis

Peer reviewedPublisher PD

Lymphatic Mapping and Sentinel Lymph Node Biopsy in Women With Squamous Cell Carcinoma of the Vulva: A Gynecologic Oncology Group Study

To determine the safety of sentinel lymph node biopsy as a replacement for inguinal femoral lymphadenectomy in selected women with vulvar cancer

Is bilateral lymphadenectomy for midline squamous carcinoma of the vulva always necessary? An analysis from Gynecologic Oncology Group (GOG) 173

To determine which patients with near midline lesions may safely undergo unilateral groin dissection based on clinical exam and lymphoscintigraphy (LSG) results

Published text: A case of ovarian fibromatosis and massive ovarian oedema associated with intra-abdominal fibromatosis, sclerosing peritonitis and Meig's syndrome

Abstract Purpose: To discuss a case of ovarian fibromatosis/massive ovarian oedema, intra-abdominal fibromatosis, sclerosing peritonitis and Meig's syndrome. To review the reported therapeutic options. Patients: Case report of a 27-year-old female with the combined pathology of ovarian fibromatosis/massive ovarian oedema, intra-abdominal fibromatosis, sclerosing peritonitis and Meig's syndrome. Methods: This patient was treated with supportive care and cytotoxic chemotherapy. Results: Despite the benign nature of the ovarian pathology, this patient presented with life-threatening complications. Response to treatment was probably multi-factorial combining the effects of cytotoxics, use of steroids and good supportive care. She remains in complete remission 4 years post completion of chemotherapy. Conclusion: There are reports in the literature of ovarian fibromatosis/massive ovarian oedema, luteinised thecomas, intraabdominal fibromatosis and Meig's syndrome occurring together in a variety of combinations. Treatment has been described with radiotherapy, cytotoxic and non-cytotoxic chemotherapy regimens. This case provides a link between ovarian fibromatosis/massive ovarian oedema, intra-abdominal fibromatosis, sclerosing peritonitis and Meig's syndrome not previously described. Presentation of case AP is a 27-year-old female who presented to her local accident and emergency department in July 1999 with a history of bloating, abdominal pain and altered bowel habit. On examination she was found to have an abdominal mass and clinical ascites. An ultra-sound scan confirmed bilateral ovarian enlargement with significant ascites. Beta-HCG, CA-125 and alpha-fetoprotein were within normal range. Past medical history was significant for a termination of pregnancy 2 years previously and a renal calculus. There was no history of colonic polyps and no significant family history. The patient was taking the combined oral contraceptive at the time of presentation. A laparotomy with bilateral ovarian biopsies was undertaken. At laparotomy, 4.5 l of ascites were drained and the abdominal structures appeared normal. The histology from the ovarian biopsies was thought to be consistent with a sclerosing stromal tumour of the ovary. Bilateral salpingo-oophorectomy and infra-colic omentectomy was then undertaken. Following the second laparotomy the patient became increasingly symptomatic with worsening ascites and vomiting. A barium follow-through revealed incomplete small bowel obstruction in the pelvis as well as a 15 cm stricture in the sigmoid colon thought to be due to extrinsic compression. A flexible sigmoidoscopy was performed and biopsies taken which revealed inflammation only. The subacute bowel obstruction was initially managed conservatively and the patient commenced total parenteral nutrition (TPN). At the end of July 1999 a third laparotomy was undertaken for persistent small bowel obstruction and clinical evidence of recurring mass in the abdomen. At laparotomy the entire pelvis and lower abdomen were involved in a retroperitoneal mass matting the bowel together. Biopsies were taken from the retroperitoneal mass, the omentum and the sigmoid colon