However immuno-histochemistry and molecular genetic techniques support the hypothesis that in the majority of women, the ovarian tumour is metastatic from a perforated appendiceal mucinous tumour. In women synchronous ovarian and appendiceal disease is common, and PMP appears more prevalent. Most acknowledge that PMP predominantly originates in the appendix in men and increasingly evidence suggests a similar site of origin in females. Additionally intestinal mucinous tumours, particularly colorectal cancers, or indeed any mucinous neoplasm may present with clinical, radiological and pathological features resembling PMP. This ranges from mucinous ascites, in association with a cystadenoma of the appendix (resulting in true PMP) to frank mucinous adenocarcinoma. In reality the clinico-pathological entity “PMP syndrome” or “jelly belly” probably represents a spectrum of disease. Both the clinical caseload experience of the two UK national centres (Basingstoke and Manchester), and a recent publication from the Netherlands reporting on a nationwide epidemiological and pathological database, suggests that the incidence of PMP is approximately two per million, per year. The exact incidence of PMP remains speculative. Since these early reports there has been ongoing debate as to the primary origin of PMP, particularly in women. In 1901, Frankel described a case associated with a cyst of the appendix. Werth in 1884 coined the term PMP, describing it in association with a mucinous tumour of the ovary. There has recently been a global interest in the management of PMP, particularly in macroscopic removal of tumour by complex surgical techniques combined with heated intraperitoneal chemotherapy (HIPEC). The long term survival in most patients remains poor with reported 5 and 10 year survival rates of 50% and 10%-30%, respectively. PMP has generally been considered benign however its behaviour suggests that it should, at best, be considered a borderline malignancy with disease progression over time, to massive abdominal distension and nutritional compromise in most cases. Thus all who operate within the abdominal cavity will encounter an occasional case and will be faced with diagnostic and therapeutic uncertainty due to the rarity of PMP and the lack of an evidence base, or consensus, on management. Many cases present unexpectedly at laparoscopy or laparotomy, or may be suspected at cross-sectional imaging during the investigation, or staging, of another pathological entity. Classically it is characterized by diffuse intra-abdominal gelatinous collections (jelly belly) with mucinous implants on peritoneal surfaces and the omentum. Most people with these symptoms won't have PMP, but it's important to have any symptoms checked by your doctor.Īt The Christie, the colorectal and peritoneal oncology centre treats PMP.Pseudomyxoma peritonei (PMP) is an uncommon clinical entity with an estimated incidence of one to two per million per year. Most people don't have any symptoms for a long time. It usually remains inside the tummy, spreading along its internal surfaces. Unlike other cancers, PMP rarely spreads via the lymphatic system or the bloodstream. It can be many years before symptoms become obvious. ![]() The tumour cells and mucin build up in the lining of the tummy, putting pressure on the bowel and causing symptoms. The tumour can then break through the wall of the appendix and spread tumour cells into the lining of the tummy (the peritoneum). This can cause the appendix to swell up like a balloon. ![]() Over time, the tumour produces a jelly-like substance called mucin. Rarely, PMP starts in other parts of the bowel, ovary or bladder. Pseudomyxoma peritonei (PMP) usually begins as a slow-growing tumour in the appendix, called a Low-Grade Mucinous Appendiceal Neoplasm (LAMN). Toggle About The Foundation Trust sub menu Toggle Continuing professional development sub menu Patient Advice and Liaison Service (PALS)
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