Archiv für chirurgische Onkologie

Pseudomyxoma Peritonei from a Borderline Mucinous Tumor Arising inan Ovarian Mature Cystic Teratoma: A Rare Case Report

Abstract

Chiruvella A, Staley CA, Khanna N, Russell M, Maithel SK, Adsay V, Horowitz IR, Staley C and Winer J*

Pseudomyxoma peritonei (PMP) is a rare associated diagnosis of peritoneal carcinomatosis which, classically, has been characterized by a slowly progressive disease process. More recently, Ronnett et al., have histologically classified PMP into the slow growing disseminated peritoneal adenomucinosis (DPAM), the much more aggressive and invasive peritoneal mucinous carcinomatosis (PMCA) and an intermediate group (ID). Recent studies have shown that most cases of PMP arise from ruptured appendiceal tumors with dissemination of mucin producing epithelial cells into the peritoneal cavity. PMP, contrary to popular belief, almost never arises from a ruptured primary ovarian mucinous tumor, corroborated by two of the largest series on ruptured borderline mucinous ovarian neoplasms. A small number of cases have been reported, however, with peritoneal carcinomatosis secondary to rupture of an ovarian mucinous tumor associated with a mature cystic teratoma. These are usually CK7 negative and CK20 positive, and are immunohistochemically consistent with intestinal type of mucinous tumors that arise from intestinal elements of the teratoma. Other malignancies such as squamous cell carcinomas, malignant endodermal sinus tumors and chorio-carcinomas have been shown to arise from a mature teratoma. We present a case report describing one such rare case of DPAM arising from a ruptured borderline mucinous ovarian tumor in the background of a cystic teratoma.