A unique presentation of superinfected pseudomyxoma peritonei secondary to a low-grade appendiceal mucinous neoplasm

Brianne J Sullivan,Umut Sarpel,Deepa Magge,Nathan Bolton

doi:10.1186/s12957-019-1578-8

Abstract

BackgroundPseudomyxoma peritonei (PMP) is an uncommon condition characterized by diffuse mucinous material in the abdomen and pelvis, generally arising from a perforated epithelial neoplasm. Typically, the disease presents as suspected acute appendicitis, ovarian mass, abdominal distension, or ventral hernia. Our case represents a very rare presentation of superinfected PMP.Case presentationA 46-year-old female with a past medical history notable for depression, asthma, and uterine leiomyomas presented to an urgent care with 5 days of progressive abdominal pain, bloating, nausea, and subjective fevers. The patient had a diffusely tender abdomen, without peritonitis, was mildly tachycardic, and had a white blood cell count of 15 K. A CT of the abdomen/pelvis was consistent with PMP with a ruptured appendiceal mucocele versus PMP secondary to an adnexal ovarian neoplastic pathology with an infectious component. The patient initially improved on antibiotics but ultimately required two surgeries, the first of which controlled intraabdominal sepsis while the second permitted definitive management of PMP with cytoreductive surgery (CRS) and HIPEC.ConclusionSuperinfected PMP is a rare entity with very few documented cases. A staged approach that incorporates clearing the peritoneal infection, with or without resection of the primary tumor, followed by rehabilitation and definitive surgery appears to be a safe and effective management strategy.

Highlights

Pseudomyxoma peritonei (PMP) is an uncommon condition characterized by diffuse mucinous material in the abdomen and pelvis, generally arising from a perforated epithelial neoplasm
Pseudomyxoma peritonei (PMP) is an uncommon condition characterized by diffuse mucinous material in the abdomen and pelvis, along with implants on peritoneal surfaces and omentum, secondary to a perforated mucinous neoplasm [1]
In 1995, Ronnet et al classified PMP into three groups based upon the clinicopathologic features of 109 cases, disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and a hybrid type

Summary

Conclusion

When superinfected PMP is highly suspected from imaging or biopsy results, surgical intervention likely should not be delayed, regardless of the lack of peritoneal signs. A staged approach that incorporates clearing the peritoneal infection, with or without resection of the primary tumor, followed by rehabilitation and definitive surgery appears to be a successful management strategy. In a patient with concurrent intraabdominal infection and psuedomyxoma peritonei, a staged approach, similar to damage control surgery, allows for a safe and appropriate oncologic resection at a later date. Superinfected pseudomyxoma peritonei is unlikely to respond solely to antibiotics and supportive care given the diffuse nature of the disease; operative intervention with the intention of clearing the infection should be an early management strategy

Background

Discussion