2/27/2024 0 Comments Jelly belly pseudomyxoma peritonei![]() Since initial descriptions of PMP as a syndrome in association with an ovarian tumor or an appendiceal mucocele, a pre-existing intraperitoneal mucinous neoplasm has been implicated as the primary cause of PMP. PMP is characterized by dissemination of mucinous tumor implants on peritoneal surfaces and progressive accumulation of mucinous ascites throughout the peritoneal cavity resulting in the so-called “jelly belly”. In this article, the current knowledge on the crucial role of mucin in the pathogenesis of the disease is reviewed and a number of potential therapeutic strategies for mucin elimination in PMP are addressed. Since PMP and mucin are inextricably linked, any therapeutic intervention needs to properly target the mucin ectopy. To enhance outcomes of the conventional therapy, novel approaches based on in-depth understanding of the pathological processes and biological events in the pathogenesis of the disease are warranted. This is despite the fact that “ rare diseases are rare, but rare disease patients are numerous” and those with progressive, life-threatening courses deserve to be further explored. Being ignored in research, however, PMP remains poorly understood and enigmatic. Despite a multidisciplinary approach composed of an extensive surgical procedure and chemotherapy, PMP frequently recurs and increasingly jeopardizes quality of life. Moreover, it is a challenging entity with debilitating, even fatal complications. As an indolent neoplasm with unspecific manifestations, PMP tends to be misdiagnosed, or discovered at advanced stages. With an estimated incidence of 1–2 out of a million, pseudomyxoma peritonei (PMP, ORPHA26790)-also known as adenomucinosis or gelatinous ascites- is listed as a rare disease by the NIH Office of Rare Diseases Research (ORDR) and National Organization for Rare Disorders (NORD). ![]() Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. ![]() Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Being ignored in research, however, PMP remains a challenging, enigmatic entity. It tends to be diagnosed at advanced stages and frequently recurs after treatment. ![]() Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Computed tomography and histopathology are the most reliable diagnostic modalities. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis.
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