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: Nima Nasiri, M.D.[2]Parminder Dhingra, M.D. [3]
Overview
Pseudomyxoma peritonei is a rare disease characterized by the accumulation of mucinous ascites and mucinous tumor disseminated in the peritoneal cavity, mainly the abdomen and pelvis. The disease mainly 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, 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, pelvic mass and infertility due to compression impact of tumor on visceral organs.[1]
- The tumor produces abundant mucins which accumulate because of gravity effect in the mesentery, the greater and lesser omentum, under the diaphragm, around the liver and other visceral organ surfaces.[2]
- The optimal treatment is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemoperfusion (HIPEC).[3]
Prognosis
- Prognosis of pseudomyxoma peritonei is generally good because of change in treatment modalities, traditional debulking surgery has been replaced by cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion (HIPEC).[4]
- According to WHO classification, pseudomyxoma peritonei (PMP) is divided into disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA), with DPAM type having better prognosis with higher survival rate based on their histological morphology. In contrast, tumors arising from colorectal neoplasm are usually high grade with a bad prognosis.[5][6][7]
References
- ↑ Pugin F, Bouquet De Jolinière J, Major A, Khomsi F, Guillou L, Peter M, Ben Ali N, Egger B, Feki A (2017). "Pseudomyxoma Peritonei: A Case Report Diagnosed in a 47-Year-Old Woman with Chronic Pelvic Abdominal Pain and Appendicular Origin: Review of the Literature and Management". Front Surg. 4: 41. doi:10.3389/fsurg.2017.00041. PMC 5741642. PMID 29326943.
- ↑ Levy, Angela D.; Shaw, Janet C.; Sobin, Leslie H. (2009). "Secondary Tumors and Tumorlike Lesions of the Peritoneal Cavity: Imaging Features with Pathologic Correlation". RadioGraphics. 29 (2): 347–373. doi:10.1148/rg.292085189. ISSN 0271-5333.
- ↑ 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.
- ↑ Mohamed F, Gething S, Haiba M, Brun EA, Sugarbaker PH (April 2004). "Clinically aggressive pseudomyxoma peritonei: a variant of a histologically indolent process". J Surg Oncol. 86 (1): 10–5. doi:10.1002/jso.20038. PMID 15048674.
- ↑ Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei"". Am. J. Surg. Pathol. 19 (12): 1390–408. PMID 7503361.