Pseudomyxoma Peritonei and Ovarian Mucinous Borderline Tumor

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Pseudomyxoma Peritonei and Ovarian Mucinous Borderline Tumor

Journal Title: Interventions in Gynaecology and Women’s Healthcare - Year 2018, Vol 2, Issue 4

Abstract

Pseudomyxoma peritonei is only rarely seen in conjunction with primary ovarian tumors. We describe a case of a late recurrence of a mucinous intestinal-type borderline ovarian tumor with disseminated peritoneal adenomucinosis. Pseudomyxoma peritonei (PMP) is a rare entity with an incidence of 2 per million individuals per year [1]. It is slowly progressive mucinous malignant disease confined to the peritoneal cavity, usually originating from the appendix [2]. The predisposed areas within the peritoneal cavity of PMP are defined by fluid absorption through lymphatic lacunae and lymphoid aggregates (greater omentum, right hemidiaphragm) and gravity (pelvic cavity, paracolic gutters) [3] The natural course of the disease is slow progression of peritoneal mucinous masses until death caused by complications due to the bulk of tumor. A 36-year old woman without comorbidities who underwent unilateral adnexectomy and appendectomy in 2011 with diagnosis of low grade ovarian intestinal type borderline mucinous tumor. CA19.9 tumor marker testing was slightly elevated, 78 U/ml; the others tumor markers were normal. Surgical exploration by median laparotomy showed widespread gelatinous mucinous ascites and a huge ruptured right ovarian tumor of 30 cm in diameter. All visible mucinous deposits were resected. The Immunohistochemistry study was: Ck7: +, Ck20: +++, CDX2: +, R.E: (-), R.P: (-), CEA: +, Muc2: +, Ki-67: + 5%. No adjuvant therapy was given. In 2013 she had a pregnancy by in vitro fertilization with cesarean delivery and no evidence of tumor recurrence at that time. In 2017 the patient wanted a new pregnancy. Due to the diagnosis of early ovarian failure, she was remitted to the Reproduction Unit again. During the ovarian stimulation, a large column of fluid was observed through sonographic exploration in the douglas space. The Ca19.9 tumor marker testing was elevated again, 80 U/ml. In the CT- scan there were signs of peritoneal carcinomatosis, so a diagnostic laparoscopy was performed showing a gelatinous-mucinous ascites and disseminated peritoneal carcinomatosis with implants widespread on the peritoneum, small intestine, colon, diaphragm and liver (Figures 1 & 2). A complete surgical debulking was judged to be technically impossible. Anatomopathological examination showed multiple deposits of mucus without visible tumor epithelial cells and some mucinous cells intestinal type in biopsied peritoneal implants with very mild atypia. Postoperatively no major wound complication occurred and 4 weeks after surgery, the woman was sent to Oncology to assess starting chemotherapy treatment.

Authors and Affiliations

Baquedano L, Lanzón A, Bolea R, José Y, Ruiz-Conde MA

Keywords

Pseudomyxoma Peritonei and Ovarian Mucinous Borderline Tumor

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EP ID EP574537
DOI 10.32474/IGWHC.2018.02.000142
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