Pseudomyxoma peritonei overview

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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]

Overview

Pseudomyxoma peritonei is a very rare tumor characterized by the presence of abundant mucus in the abdominal cavity, consisting of intraperitoneal mucinous tumors and ascites, most commonly arising from mucinous tumors of the appendix, however, the ovary or the colon may be the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two pathological subtypes with different etiology and prognosis, these two subtypes are peritoneal adenomucinosis and peritoneal mucinous carcinoma. The K-Ras and p53 genes are involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and tuberculosis peritonitis. The incidence of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility symptoms if left untreated. Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention. On abdominal CT scan, pseudomyxoma peritonei is characterized by low attenuation, loculated ascitic mucinous fluid in the peritoneum, omentum and mesentery with scalloping of visceral surfaces of the liver. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites which is not mobile. Cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion is the optimal way of treatment for pseudomyxoma peritonei.

Historical Perspective

The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.[1]

Pathophysiology

Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes: Disseminated peritoneal adenomucinosis (DPAM), and Peritoneal mucinous carcinomatosis (PMCA).[2]

Causes

Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. The K-Ras and p53 genes may be involved in the oncogenesis.[3]

Differentiating Pseudomyxoma peritonei from other Diseases

Differential diagnosis of pseudomyxoma peritonei include peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, Peritonitis, peritoneal mesothelioma.[4]

Epidemiology and Demographics

The incidence of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years.[5][6]

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. Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.Overall risk of complications after surgery such as urinary tract involvement due to tumor seeding in pelvis is very low.[7][8][9]

History and Symptoms

Symptoms of pseudomyxoma peritonei include: abdominal pain, bloating, abdominal distention, Shortness of breath, Weight change, Menstrual irregularities in women.[10]

Physical Examination

Common physical examination findings of pseudomyxoma peritonei include: Abdominal tenderness, Abdominal distention.[10]

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).[11]

CT

Findings on abdominal CT scan of patients with pseudomyxoma peritonei include: accumulation of fluid throughout the peritoneum, omentum and mesentery seen as low attenuation mucin depositis on CT scan, scalloping of visceral organs.[12]

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.[13]

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.[14][15][16]

Medical Therapy

The predominant therapy for pseudomyxoma peritonei is surgical resection and debulking. 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 intraperitoneal chemoperfusion (HIPEC).[17][18]

Surgery

Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.[11][19]

References

  1. O'Connell JT, Tomlinson JS, Roberts AA, McGonigle KF, Barsky SH (August 2002). "Pseudomyxoma peritonei is a disease of MUC2-expressing goblet cells". Am. J. Pathol. 161 (2): 551–64. doi:10.1016/S0002-9440(10)64211-3. PMC 1850719. PMID 12163380.
  2. Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, Yamashita N, Yamaguchi K, Terakado Y, Ikenoue T, Furukawa Y (December 2015). "Molecular profiles of high-grade and low-grade pseudomyxoma peritonei". Cancer Med. 4 (12): 1809–16. doi:10.1002/cam4.542. PMC 5123786. PMID 26475379.
  3. 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.
  4. 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.
  5. Roberts DL, O'Dwyer ST, Stern PL, Renehan AG (May 2015). "Global gene expression in pseudomyxoma peritonei, with parallel development of two immortalized cell lines". Oncotarget. 6 (13): 10786–800. doi:10.18632/oncotarget.3198. PMC 4484419. PMID 25929336.
  6. 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.
  7. Smeenk RM, Bex A, Verwaal VJ, Horenblas S, Zoetmulder FA (January 2006). "Pseudomyxoma peritonei and the urinary tract: involvement and treatment related complications". J Surg Oncol. 93 (1): 20–3. doi:10.1002/jso.20427. PMID 16353186.
  8. 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.
  9. Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, Baratti D, Deraco M, Elias D, Sardi A, Liauw W, Yan TD, Barrios P, Gómez Portilla A, de Hingh IH, Ceelen WP, Pelz JO, Piso P, González-Moreno S, Van Der Speeten K, Morris DL (July 2012). "Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy". J. Clin. Oncol. 30 (20): 2449–56. doi:10.1200/JCO.2011.39.7166. PMID 22614976.
  10. 10.0 10.1 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.
  11. 11.0 11.1 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.
  12. Lee NK, Kim S, Kim HS, Jeon TY, Kim GH, Kim DU, Park DY, Kim TU, Kang DH (November 2011). "Spectrum of mucin-producing neoplastic conditions of the abdomen and pelvis: cross-sectional imaging evaluation". World J. Gastroenterol. 17 (43): 4757–71. doi:10.3748/wjg.v17.i43.4757. PMC 3229624. PMID 22147976.
  13. 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.
  14. 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.
  15. 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.
  16. 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.
  17. 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.
  18. 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.
  19. Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. PMID 15701175.


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