Pseudomyxoma peritonei natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
Pseudomyxoma peritonei is a rare disease characterized by the accumulation of mucinous ascites and mucinous tumor disseminated in the peritoneal cavity, mainly abdomen and pelvis; the disease almost always originates from primary appendiceal tumors and less commonly from the ovary and colon. In 2010, WHO published a classification which divides pseudomyxoma peritonei (PMP) into low and high grades. Cytoreductive surgery with hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) is the mainstay of treatment appendiceal pseudomyxoma peritonei (PMP).
Natural History
- Pseudomyxoma peritonei (PMP) is an uncommon disease characterized by mucinous ascites in intraperitoneal cavities, it is originating from a low-grade mucinous neoplasm of the appendix, if left untreated, patients with pseudomyxoma peritonei may progress to develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility due to compression impact of tumor on visceral organs.
- Psuedomyxoma peritonei tumour produces abundant mucins which accumulate because of to gravity effect in the mesentery, the greater and lesser omentum, and the under the diaphragm, around the liver and other visceral organs surfaces.
- The optimal treatment is cytoreductive surgery (CRS) combined with hyperthermic Intraperitoneal chemotherapy (HIPEC).[1]
Prognosis
- Prognosis of pseudomyxoma peritonei is generally good because of change in treatment modalities, mainly surgery techniques which developed by Sugarbaker. Traditional debulking surgery replaced by cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy(HIPEC).[2]
- Although pseudomyxoma peritonei is a neoplastic disease, it runs in a chronic, an indolent course with late invasion and only rare metastasis outside the peritoneum.
- According to WHO classification, pseudomyxoma peritonei (PMP) divides into low and high grades, and the most common primary tumor arises from a low grade appendiceal mucinous neoplasm with good prognosis with higher survival rate based on their histological morphology, in contrast, tumors arising from colorectal neoplasm are usually high grades with bad prognosis.[3]
References
- ↑ Mittal R, Chandramohan A, Moran B (August 2017). "Pseudomyxoma peritonei: natural history and treatment". Int J Hyperthermia. 33 (5): 511–519. doi:10.1080/02656736.2017.1310938. PMID 28540829.
- ↑ Sugarbaker PH (April 2001). "Cytoreductive surgery and peri-operative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome". Eur J Surg Oncol. 27 (3): 239–43. doi:10.1053/ejso.2000.1038. PMID 11373099.
- ↑ Carr, Norman J; Finch, Jenny; Ilesley, Ian Charles; Chandrakumaran, Kandiah; Mohamed, Faheez; Mirnezami, Alex; Cecil, Tom; Moran, Brendan (2012). "Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases". Journal of Clinical Pathology. 65 (10): 919–923. doi:10.1136/jclinpath-2012-200843. ISSN 0021-9746.