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Diagnostic Pathology

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Open Access 01-12-2021 | Case Report

A case of Pseudomyxoma Peritonei of an unexpected origin

Authors: Marie Csanyi-Bastien, France Blanchard, Aude Lamy, Jean-Christophe Sabourin

Published in: Diagnostic Pathology | Issue 1/2021

Abstract

Background

Pseudomyxoma peritonei (PMP) is a complex and partially understood disease defined by mucin deposits in the peritoneal cavity, mostly of appendiceal origin caused by the rupture of a mucocele often containing Low or High grade Appendiceal Mucinous Neoplasm (LAMN/HAMN). Other origins include primitive ovarian mucinous cystadenoma or cystadenocarcinoma almost always with an associated teratoma, but to our knowledge no case of ovarian teratomatous appendiceal-like mucocele with LAMN has been reported as a cause of PMP.

Case presentation

A 25-year old female with infertility was diagnosed with an isolated left ovarian tumor in a context of PMP. Histological examination revealed an ovarian teratoma containing an appendiceal-like structure with mucocele and LAMN, without any associated lesion of the appendix on full histological analysis. Molecular characterization of the ovarian lesion showed co-KRAS and GNAS mutations, as described in PMP of appendiceal origin, while only KRAS mutations are reported in primitive ovarian mucinous tumor.

Conclusions

Detection of co-KRAS and GNAS mutations in our case of ovarian teratomatous appendiceal-like mucocele with LAMN shows that when PMP derives from a mucinous ovarian lesion (with histological proof of none-appendiceal involvement), it is probably of a digestive teratomatous origin, emphasizing the need to actively search for tetatomatous signs in a context of ovarian PMP.

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Title: A case of Pseudomyxoma Peritonei of an unexpected origin
Authors: Marie Csanyi-Bastien
France Blanchard
Aude Lamy
Jean-Christophe Sabourin
Publication date: 01-12-2021
Publisher: BioMed Central
Published in: Diagnostic Pathology / Issue 1/2021
Electronic ISSN: 1746-1596
DOI: https://doi.org/10.1186/s13000-021-01179-z

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A case of Pseudomyxoma Peritonei of an unexpected origin

Abstract

Background

Case presentation

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusions

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