What makes it a Primary Ovarian Pseudomyxoma Peritonei When there are Mucinous Tumours of both Ovary and Appendix

Abstract

Introduction Pseudomyxoma peritonei (PMP) is a rare condition characterized by gelatinous material within the abdominal cavity and on peritoneal surfaces arising from a mucinous neoplasm originating most commonly from appendix followed by ovary. Case Report A 48-year-old woman presented with abdominal bloating and weight loss for four months. Imaging studies showed malignant mass lesion arising from left ovary probably a mucinous adenocarcinoma with mucinous material in abdomen. She underwent cytoreduction and hyperthermic intraperitoneal chemotherapy. The left ovary was multiloculated filled with mucinous material and a focal solid area containing pultaceous material and tufts of hair. The appendix showed mucinous areas on the serosa. Microscopically, the left ovary showed multiple closely apposed cysts lined by a single layer of cuboidal to columnar mucinous epithelium with focal undulation and stratification. Also noted were few cysts walled by hair follicles and lobules of adipocytes. The appendix mucosa focally showed tubular glands with tufting lined by mucinous cells. Mucin pools were seen within the lamina propria, serosa and mesoappendicular fat. Pelvic peritoneum, falciform ligament, left upper and right lower quadrant peritoneum, rectal peritoneum, liver capsule and omentum showed acellular mucin. Low-grade appendiceal mucinous neoplasm (LAMN) and primary ovarian mucinous cystadenoma arising in mature cystic teratoma (MCT) with PMP were rendered. Conclusion We report this case for its rarity. We would like to emphasize with this case the need to completely examine the appendix and thoroughly sample ovarian mucinous tumours to look for teratomatous component in all cases of PMP.