Pseudomyxoma peritonei overview
|
Pseudomyxoma peritonei Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Pseudomyxoma peritonei overview On the Web |
|
American Roentgen Ray Society Images of Pseudomyxoma peritonei overview |
|
Risk calculators and risk factors for Pseudomyxoma peritonei overview |
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 an uncommon tumor known for its production of abundant mucus in the abdominal cavity, it's a rare condition consisting of intraperitoneal mucinous tumors and ascites, most commonly arises from mucinous tumors of the appendix. The tumor is not harmful by itself, but the mucus has no place to go inside the abdominal cavity. If left untreated, it will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc. The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.
Pathophysiology
Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes:
- Disseminated peritoneal adenomucinosis (DPAM)
- Peritoneal mucinous carcinomatosis (PMCA).
Causes
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm.
- The K-Ras and p53 genes may be involved in the oncogenesis.[1]
Differentiating Pseudomyxoma peritonei from other Diseases
Differential diagnosis of pseudomyxoma peritonei include:
- Peritoneal carcinomatosis without mucinous ascites
- Peritoneal sarcomatosis
- Peritonitis
- Peritoneal mesothelioma
Epidemiology and Demographics
- The incidence of pseudomyxoma peritonei is approximately 0.5 to 1 per 100,000 individuals worldwide.
- Females are more commonly affected with pseudomyxoma peritonei than males.
- The median age at diagnosis is 50 years.[2]
Risk Factors
There are no established risk factors for pseudomyxoma peritonei.
Natural History, Complications and Prognosis
- Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility.[3]
- Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.[4]
History and Symptoms
Symptoms of pseudomyxoma peritonei include:[5]
- abdominal pain
- bloating
- abdominal distention
- Shortness of breath
- weight changes
- Menstrual irregularity in women
Physical Examination
Common physical examination findings of pseudomyxoma peritonei include: [5]
Laboratory Findings
The laboratory findings associated with pseudomyxoma peritonei include:
- Increased levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen 19.9 (CA19.9), carbohydrate antigen 125 (CA125).[6]
CT
- On abdominal CT scan, pseudomyxoma peritonei is characterized by a low attenuation, accumulation of fluid throughout the peritoneum, omentum and mesentery with scalloping of visceral surfaces, particularly the liver.[7]
MRI
- On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, it can further clarify the cystic structure of mass.[8]
Ultrasound
- Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei.
- Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.[9][10][11]
Medical Therapy
- The predominant therapy for pseudomyxoma peritonei is surgical resection and debulking. [12]
- Supportive therapy for pseudomyxoma peritonei includes chemotherapy which can be used as adjuvant treatment.
- The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with Hyperthermic Intra-Peritoneal Chemotherapy (HIPEC).[13]
Surgery
Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.[6][14]
References
- ↑ Borazanci E, Millis SZ, Kimbrough J, Doll N, Von Hoff D, Ramanathan RK (February 2017). "Potential actionable targets in appendiceal cancer detected by immunohistochemistry, fluorescent in situ hybridization, and mutational analysis". J Gastrointest Oncol. 8 (1): 164–172. doi:10.21037/jgo.2017.01.14. PMC 5334061. PMID 28280620.
- ↑ Zhong Y, Deng M, Xu R, Kokudo N, Tang W (August 2012). "Pseudomyxoma peritonei as an intractable disease and its preoperative assessment to help improve prognosis after surgery: A review of the literature". Intractable Rare Dis Res. 1 (3): 115–21. doi:10.5582/irdr.2012.v1.3.115. PMC 4204597. PMID 25343082.
- ↑ Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ (March 2011). "Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin". Dis. Colon Rectum. 54 (3): 293–9. doi:10.1007/DCR.0b013e318202f026. PMID 21304299.
- ↑ Amini A, Masoumi-Moghaddam S, Ehteda A, Morris DL (May 2014). "Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects". Orphanet J Rare Dis. 9: 71. doi:10.1186/1750-1172-9-71. PMC 4013295. PMID 24886459.
- ↑ 5.0 5.1 Wambura C, Jusabani A, Sherman O, Surani S (October 2018). "Pseudomyxoma pleurii and peritonei secondary to sigmoid colon adenocarcinoma: a rare clinico-pathologico-radiological presentation". Oxf Med Case Reports. 2018 (10): omy057. doi:10.1093/omcr/omy057. PMC 6142713. PMID 30250743.
- ↑ 6.0 6.1 Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Morris, David (2014). "Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects". Orphanet Journal of Rare Diseases. 9 (1): 71. doi:10.1186/1750-1172-9-71. ISSN 1750-1172.
- ↑ Sulkin TV, O'Neill H, Amin AI, Moran B (July 2002). "CT in pseudomyxoma peritonei: a review of 17 cases". Clin Radiol. 57 (7): 608–13. PMID 12096860.
- ↑ Wrafter PF, Connelly T, Khan JS, Joyce WP (October 2015). "Pseudomyxoma peritonei diagnosed 19 years after appendicectomy". BMJ Case Rep. 2015. doi:10.1136/bcr-2015-211706. PMC 4620202. PMID 26494720.
- ↑ Khan S, Patel AG, Jurkovic D (April 2002). "Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman". Ultrasound Obstet Gynecol. 19 (4): 410–2. doi:10.1046/j.1469-0705.2002.00611.x. PMID 11952975.
- ↑ Qi ZH, Cai S, Li JC, L K, Zhang Y, Xiao MS, Su N (August 2015). "[Ultrasonographic Features of Pseudomyxoma Peritonei and TheirComparison with Computed Tomographic Findings]". Zhongguo Yi Xue Ke Xue Yuan Xue Bao (in Chinese). 37 (4): 424–9. doi:10.3881/j.issn.1000-503X.2015.04.010. PMID 26564459.
- ↑ Li Y, Guo A, Tang J, Wang L, Wang J, Yu D (September 2013). "Role of preoperative sonography in the diagnosis and pathologic staging of pseudomyxoma peritonei". J Ultrasound Med. 32 (9): 1565–70. doi:10.7863/ultra.32.9.1565. PMID 23980216.
- ↑ 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.
- ↑ Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FA (January 2007). "Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy". Ann. Surg. 245 (1): 104–9. doi:10.1097/01.sla.0000231705.40081.1a. PMC 1867935. PMID 17197972.
- ↑ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. PMID 15701175.