Pseudomyxoma peritonei: unusual origin from an ovarian mature cystic teratoma: Figure 1

Abstract

Pseudomyxoma peritonei (PMP) is classified into pathologically and prognostically distinct categories, such as disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis. There is overwhelming evidence that DPAM arises from a mucinous adenoma of the appendix. The one exception to this is the presentation of a mature ovarian cystic teratoma as PMP where the appendix is normal. This report describes such a case and discusses the presentation, histopathology, and treatment options.

Ovarian Mature Teratomas With Mucinous Epithelial Neoplasms: Morphologic Heterogeneity and Association With Pseudomyxoma Peritonei

“…On the basis of the staging and immunohistochemical data in their study, which included a fully examined normal appendix in all 3 cases, it was suggested that the rare case of an ovarian origin of PMP was probably restricted to mucinous tumors arising in mature teratomas and others have endorsed this hypothesis. 8,14,25 Our study confirms and expands the findings of these previous investigators. Ovarian mucinous neoplasms may occur in association with mature ovarian teratomas and a significant proportion of these neoplasms present with PMP (24% in this series), particularly ovarian IM-LMP (38%) and mucinous carcinoma (60%).…”

“…Since that report, additional cases have been described with the similar conclusions. 8,14,25,28 The risk of intra-abdominal recurrence attached to mucinous tumors arising in ovarian teratomas, particularly those with associated PMP, is not known. In addition, the relative risk attached to gonadal teratomatous mucinous tumors with highgrade (ie, carcinomatous) histology and mucinous ascites has not been fully evaluated in a series.…”

A phase II study evaluating the use of concurrent mitomycin C and capecitabine in patients with advanced unresectable pseudomyxoma peritonei

“…We rationalised the following: (i) serum CEA and CA19-9 levels are elevated in over 50% PMP patients at presentation and drop markedly following cytoreductive surgery and HIPEC (van Ruth et al, 2002); (ii) elevated CA 19-9 levels prior to cytoreductive surgery may be an independent predictor of worse progression-free survival (Baratti et al, 2007); and (iii) in a case study of a patient with DPAM treated with capecitabine, there was a decrease in CEA and CA19-9 levels (Levitz et al, 2004). Serum CA125 concentrations are commonly raised in patients with PMP, and at initial presentation, may mimic gynaecological malignancies (Pranesh et al, 2005). However, it is noteworthy that serum CA125 levels may also be raised in conditions including peritoneal irritation (Sjovall et al, 2002), pelvic inflammatory disease (Halila et al, 1986) and benign hepatic disease (Topalak et al, 2002).…”

Low-grade mucinous epithelial neoplasm (intestinal type) arising in a mature sacrococcygeal teratoma with late recurrence as pseudomyxoma peritonei

“…Incomplete resection of a mature teratoma has been associated with high risk for recurrence [15]. Excluding carcinomas, at least 20 teratoma-associated mucinous epithelial neoplasms with clinical pseudomyxoma peritonei have been recently reported in the ovary, but to our knowledge, there are no known recurrences [2][3][4][5]12,13]. Additional series with long-term follow-up are needed to reach a more definitive conclusion regarding the absolute risk of recurrence in both the gonadal and extragonadal forms, especially in cases that have intraperitoneal low-grade mucinous epithelium.…”

“…Mucinous epithelial neoplasms occurring in association with mature teratomas, particularly in the ovary, are well documented in the literature [1][2][3][4][5]. This association has led to long-standing theories suggesting a teratomatous histogenesis for at least a subset of these tumors when they occur in the gonads [1].…”