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Peritoneal metastases
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Pseudomyxoma peritonei occurs if a mucinous cystadenoacarcinoma or cystadenoma of the ovary or appendix ruptures into the peritoneal cavity, and the gelatinous mucoid material surrounds mesenteric reflections, bowel, and organs, and causes scalloping of the peritoneal surfaces, particularly adjacent to the liver (Figure 33.28). Septations may be seen and bowel loops are displaced posteriorly. Scalloping, septations, locules, and variable fluid density distinguish pseudomyxoma from ascites. In most cases of pseudomyxoma peritonei, the primary tumour arises from the appendix (54).
Gastrointestinal cancer
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Primary peritoneal mesothelioma is well described and has many features in common with pleural mesothelioma (see Chapter 7). Pseudomyxoma peritonei is a rare, low-grade malignant condition, which may have a very protracted natural history. It is characterized by the accumulation of large amounts of intra-abdominal mucin, often originating from a low-grade tumour of the appendix, ovary or pancreas. Both conditions are potentially treatable by cytoreductive radical peritonectomy (Sugarbaker procedure) and heated intraoperative intraperitoneal chemotherapy. The surgery is radical, involving: Removal of the right hemicolon, spleen, gallbladder, greater omentum and lesser omentumStripping of peritoneum from pelvis and diaphragmHepatic capsulectomyHysterectomy and bilateral salpingo-oophorectomy in womenRemoval of rectum in selected cases
Future perspectives in peritoneal malignancy
Published in Tom Cecil, John Bunni, Akash Mehta, A Practical Guide to Peritoneal Malignancy, 2019
Ioanna Panagiotopoulou, Alexios Tzivanakis, Tom Cecil
Despite the good outcomes that can be achieved by complete cytoreduction for pseudomyxoma peritonei, disease will recur in some patients who may or may not be reoperable and eventually progress. There are also those who had an initial tumour debulking who eventually develop progressive disease. When no further surgical options exist, mainly due to extensive small bowel involvement, systemic chemotherapy may be considered, but often intestinal failure will develop leading to dependence on parenteral nutrition [82]. Palliation is difficult as patients slowly develop progressive abdominal distention with nutritional and abdominal wall failure (Figure 15.1). These patients, often young with slowly progressing low-grade disease, can be difficult to manage and support.
Immunohistochemistry features and molecular pathology of appendiceal neoplasms
Published in Critical Reviews in Clinical Laboratory Sciences, 2021
Reger R. Mikaeel, Joanne P. Young, Gonzalo Tapia Rico, Peter J. Hewett, Jennifer E. Hardingham, Wendy Uylaki, Mehgan Horsnell, Timothy J. Price
The appendix is a small organ that gives rise to remarkably diverse histologic cancer types. The differential diagnosis of ANs includes epithelial ANs, lymphomas, and mesenchymal tumors [11]. Epithelial ANs are the most frequently diagnosed tumors. However, there is tremendous heterogeneity within the histology and biology of this type (Table 1). Therefore, the pathological classification of ANs, particularly appendiceal mucinous neoplasms (AMNs), has been confusing. The majority of pseudomyxoma peritonei (PMP) cases arise from AMNs that exhibit a wide spectrum of clinical and biological behaviors, ranging from significantly aggressive tumors with high risk for recurrence to slow-growing tumors with a decreased likelihood of recurrence. Clinically, PMP is characterized by a diffuse collection of mucinous tumor nodules and mucinous ascites throughout the abdominal cavity. PMP represents local spread within the abdominal peritoneal cavity and most frequently arises from ANs [1]. The prognosis of ANs has been associated mainly with the neoplastic cells within the mucinous tumors of PMP.
Incidentally found mucinous epithelial tumors of the appendix with or without pseudomyxoma peritonei: diagnostic and therapeutic algorithms based on current evidence
Published in Acta Chirurgica Belgica, 2021
Wim Ceelen, Marc De Man, Wouter Willaert, Gabrielle H. van Ramshorst, Karen Geboes, Anne Hoorens
Mucinous neoplasms of the appendix may rupture and give rise to the clinical entity of pseudomyxoma peritonei (PMP), characterized by progressive filling and distention of the peritoneal cavity with thick mucus. Based on recent data from the UK and Norway, an incidence rate of 3.2 per million per year and a prevalence of 22 people per million were calculated [6]. Although the disease has often spread to the entire abdomen at diagnosis, mechanical forces and the presence of lymphatic stomata usually result in extensive deposits at the right diaphragm, greater omentum, and pelvic peritoneum. Due to its indolent nature, specifically in patients with low-grade disease, PMP often is found incidentally during workup for non-specific abdominal complaints or distention, or during hernia surgery [7].
Multicystic peritoneal mesothelioma treated with cytoreductive surgery followed or not by hyperthermic intraperitoneal chemotherapy: results from a large multicentric cohort
Published in International Journal of Hyperthermia, 2021
Vahan Kepenekian, Julien Péron, Diane Goéré, Olivia Sgarbura, Jean-Baptiste Delhorme, Clarisse Eveno, Nazim Benzerdjeb, Isabelle Bonnefoy, Laurent Villeneuve, Pascal Rousset, Karine Abboud, Marc Pocard, Olivier Glehen
A major advantage of the laparoscopic approach could be the fertility preservation. Data on peritoneal surface malignancies and fertility are scarce and made of retrospective reports, most of them related to pseudomyxoma peritonei (PMP) [41–44]. In the RENAPE database, the specific survey performed in women of less than 40 years at diagnosis revealed that none had preoperative infertility and four had an assumed postoperative child desire. Three of them had a child, one after in vitro fertilization. The remaining women were expecting pregnancy for less than one year. Looking at the submitted treatment for MCPM showed that three had a laparoscopic-CRS-HIPEC (including the not answered one) and one had a conventional CRS without HIPEC. In literature, two pregnancies in the same patient were reported synchronously to a MCPM diagnosis (and three others in PMP patients) with vaginal delivery of two well children [42,43].