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Pseudomyxoma peritonei

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]

Synonyms and keywords: PMP; jelly belly; colloid carcinoma; disseminated peritoneal adenomucinosis (DPAM); malignant appendiceal tumor; malignant large bowel cystadenocarcinoma; malignant large bowel peritoneal carcinomatosis; malignant large bowel tumor; mucinous cyst adenocarcinoma; mucinous cystadenoma; peritoneal carcinomatosis; peritoneal mucinous carcinomatosis (PMCA); pseudomyxoma ovarii

Overview

Overview

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.

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).

Causes

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

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 0.1 per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years.

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.

History and Symptoms

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

Physical Examination

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

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).

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.

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.

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.

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).

Surgery

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


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Historical Perspective

Historical Perspective

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 an uncommon tumor known for its production of abundant mucinous tumor spreading in the peritoneal cavity, the tumor origin is mainly appendiceal neoplasm, however it may arise from ovarian or colon cancer as well. The mainstay of therapy is cytoreduction surgery with chemotherapy infusion. On 1884, Werth described the term pseudomyxoma peritonei and its association with the ovarian mucinous tumor.

Historical Perspective

  • On 1841, Carl F. Rokitansky described the first case of pseudomyxoma peritonei.[1]
  • On 1901, Frankel reported the first case of appendiceal tumor.[2]

References

  1. ↑ Pandey A, Mishra AK (May 2011). “Pseudomyxoma peritonei: disseminated peritoneal adenomucinosis variant”. BMJ Case Rep. 2011. doi:10.1136/bcr.07.2010.3181. PMC 3089927. PMID 22696701.
  2. ↑ Kotani Y, Shiota M, Umemoto M, Tobiume T, Koike E, Shimaoka M, Takahashi Y, Kanemura K, Ishizu A, Hoshiai H (2009). “Laparoscopic mucin removal in patients with pseudomyxoma peritonei”. JSLS. 13 (2): 203–6. PMC 3015933. PMID 19660216.


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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Pseudomyxoma peritonei (PMP) is a rare tumor known for its production of abundant mucinous ascites in the abdominal cavity. It can have a mass impact on vital organs such as spleen, pancreas, and kidney. Pseudomyxoma peritonei may be divided into two pathological subtypes: peritoneal adenomucinosis and peritoneal mucinous carcinoma.

Pathogenesis

The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.

Pathology

Pseudomyxoma peritonei may be divided into two pathological subtypes:[2]

Immunohistology

Immunohistochemical markers can help identify the organ of origin.

  • The tumor is positive for cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125.
  • Studies have shown that mucin MUC2 and MUC5AC is extensively positive in pseudomyxoma peritonei patients. [3]

References

  1. ↑ Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM (June 1999). “Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women”. Am. J. Pathol. 154 (6): 1849–55. doi:10.1016/S0002-9440(10)65442-9. PMC 1866622. PMID 10362811.
  2. ↑ 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.
  3. ↑ Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M (October 2006). “CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases”. Histopathology. 49 (4): 381–7. doi:10.1111/j.1365-2559.2006.02512.x. PMID 16978201.


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Causes

Causes

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 caused by a pre-existing intraperitoneal mucinous neoplasm, accumulation of fluids in abdominal cavity causes mass impact on vital organs such as the spleen, kidney, and pancreas. The pathology and causes of pseudomyxoma peritonei are poorly understood. It is shown in some studies that the K-Ras and p53 genes may be involved in the oncogenesis.

Causes

References

  1. ↑ Carr NJ, Finch J, Ilesley IC, Chandrakumaran K, Mohamed F, Mirnezami A, Cecil T, Moran B (October 2012). “Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases”. J. Clin. Pathol. 65 (10): 919–23. doi:10.1136/jclinpath-2012-200843. PMID 22718846.
  2. ↑ 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. PMID 12163380.
  3. ↑ Shetty S, Thomas P, Ramanan B, Sharma P, Govindarajan V, Loggie B (March 2013). “Kras mutations and p53 overexpression in pseudomyxoma peritonei: association with phenotype and prognosis”. J. Surg. Res. 180 (1): 97–103. doi:10.1016/j.jss.2012.10.053. PMID 23199549.


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Differentiating Pseudomyxoma peritonei from other Diseases

Differentiating Pseudomyxoma peritonei from other Diseases


To view the full chapter, click here.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and tuberculosis peritonitis.

Differential Diagnosis

Differentiating pseudomyxoma peritonei from other diseases

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Signs
Lab Findings Imaging Histopathology
Symptom Constitutional symptoms Physical exam Other lab values CT Scan Ultrasound
Pseudomyxoma peritonei[1][2][3][4] –
  • Non specific
  • Low-attenuation
  • The echoes within pseudomyxoma peritonei are not mobile.
  • Echogenic septations within the gelatinous ascites.
  • Scalloping of the hepatic and splenic margins
  • Depending on WHO classification, whether it’s low or high grade with cellular atypia or acellular mucin. ( DPAM, PMCA)
Peritoneal carcinomatosis without mucinous ascites[5][6][7] –
  • Echoes are mobile
Peritoneal sarcomatosis[8][9] –
  • Heterogeneous bulky masses
  • Hypervascularity with or without hemoperitoneum
  • Variable presence of ascites.
Lymphomatosis[10][11][12][8] +
    Tuberculosis Peritonitis[13][14] +
      Endometriosis[15][16][17][18][19]
      • Menstrual cramps
      • Heavy menstrual periods
      • Pain during or after sex
      		 –
      • Thomsen-Friedenreich (T) antigen (Gal beta1-3GalNAc)
        Sclerosing encapsulating peritonitis[20][21][22][23] –
        • Increased CRP level
        • Fibrin-fibrin degradation product
        Gliomatosis Peritonei[24][25][26][27][28][29] –
        Osseous metaplasia[8][30] –
        Splenosis[8][31][32][33][34] –
        • Lesions are typically multiple, small, reddish-brown nodules that range in size from a few millimeters to 7 cm
        Melanosis[35][36][37][38][39]
        • Majority of cases are asymptomatic
        		 –
        • Majority have normal lab values
        Inflammatory Pseudotumor[40] –

        References

        1. ↑ Carranza-Martínez I, Cornejo-López G, Monroy-Argumedo M, Villanueva-Sáenz E (2014). “[Pseudomyxoma peritonei. Two-case-report]”. Cir Cir (in Spanish; Castilian). 82 (2): 206–11. PMID 25312322.
        2. ↑ 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.
        3. ↑ Carr, Norman J.; Cecil, Thomas D.; Mohamed, Faheez; Sobin, Leslie H.; Sugarbaker, Paul H.; González-Moreno, Santiago; Taflampas, Panos; Chapman, Sara; Moran, Brendan J. (2016). “A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia”. The American Journal of Surgical Pathology. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. ISSN 0147-5185.
        4. ↑ 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.
        5. ↑ Winder T, Lenz HJ (2010). “Mucinous adenocarcinomas with intra-abdominal dissemination: a review of current therapy”. Oncologist. 15 (8): 836–44. doi:10.1634/theoncologist.2010-0052. PMC 3228029. PMID 20656916.
        6. ↑ Kusamura S, Baratti D, Zaffaroni N, Villa R, Laterza B, Balestra MR, Deraco M (January 2010). “Pathophysiology and biology of peritoneal carcinomatosis”. World J Gastrointest Oncol. 2 (1): 12–8. doi:10.4251/wjgo.v2.i1.12. PMC 2999153. PMID 21160812.
        7. ↑ Coccolini F, Gheza F, Lotti M, Virzì S, Iusco D, Ghermandi C, Melotti R, Baiocchi G, Giulini SM, Ansaloni L, Catena F (November 2013). “Peritoneal carcinomatosis”. World J. Gastroenterol. 19 (41): 6979–94. doi:10.3748/wjg.v19.i41.6979. PMC 3819534. PMID 24222942.
        8. ↑ 8.0 8.1 8.2 8.3 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.
        9. ↑ Oei TN, Jagannathan JP, Ramaiya N, Ros PR (September 2010). “Peritoneal sarcomatosis versus peritoneal carcinomatosis: imaging findings at MDCT”. AJR Am J Roentgenol. 195 (3): W229–35. doi:10.2214/AJR.09.3907. PMID 20729420.
        10. ↑ Weng SC, Wu CY (December 2008). “Lymphoma presenting as peritoneal lymphomatosis with ascites”. J Chin Med Assoc. 71 (12): 646–50. doi:10.1016/S1726-4901(09)70009-7. PMID 19114331.
        11. ↑ Horger M, Müller-Schimpfle M, Yirkin I, Wehrmann M, Claussen CD (January 2004). “Extensive peritoneal and omental lymphomatosis with raised CA 125 mimicking carcinomatosis: CT and intraoperative findings”. Br J Radiol. 77 (913): 71–3. doi:10.1259/bjr/35139284. PMID 14988144.
        12. ↑ Cunningham N, Ffrench-Constant S, Planche K, Gillmore R (February 2015). “Peritoneal lymphomatosis: a rare presentation of follicular lymphoma mimicking peritoneal carcinomatosis”. BMJ Case Rep. 2015. doi:10.1136/bcr-2014-207136. PMC 4336898. PMID 25694630.
        13. ↑ Kaya M, Kaplan MA, Isikdogan A, Celik Y (2011). “Differentiation of tuberculous peritonitis from peritonitis carcinomatosa without surgical intervention”. Saudi J Gastroenterol. 17 (5): 312–7. doi:10.4103/1319-3767.84484. PMC 3178918. PMID 21912057.
        14. ↑ Yapar, E. G.; Ekici, E.; Karasahin, E.; Gökmen, O. (1995). “Sonographic features of tuberculous peritonitis with female genital tract tuberculosis”. Ultrasound in Obstetrics and Gynecology. 6 (2): 121–125. doi:10.1046/j.1469-0705.1995.06020121.x. ISSN 0960-7692.
        15. ↑ DiVasta AD, Vitonis AF, Laufer MR, Missmer SA (March 2018). “Spectrum of symptoms in women diagnosed with endometriosis during adolescence vs adulthood”. Am. J. Obstet. Gynecol. 218 (3): 324.e1–324.e11. doi:10.1016/j.ajog.2017.12.007. PMID 29247637.
        16. ↑ Yeaman GR, Collins JE, Lang GA (March 2002). “Autoantibody responses to carbohydrate epitopes in endometriosis”. Ann. N. Y. Acad. Sci. 955: 174–82, discussion 199–200, 396–406. PMID 11949946.
        17. ↑ Kennedy S, Bergqvist A, Chapron C, D’Hooghe T, Dunselman G, Greb R, Hummelshoj L, Prentice A, Saridogan E (October 2005). “ESHRE guideline for the diagnosis and treatment of endometriosis”. Hum. Reprod. 20 (10): 2698–704. doi:10.1093/humrep/dei135. PMID 15980014.
        18. ↑ Hsu AL, Khachikyan I, Stratton P (June 2010). “Invasive and noninvasive methods for the diagnosis of endometriosis”. Clin Obstet Gynecol. 53 (2): 413–9. doi:10.1097/GRF.0b013e3181db7ce8. PMC 2880548. PMID 20436318.
        19. ↑ Bloski, Terri; Pierson, Roger (2008). “Endometriosis and Chronic Pelvic Pain: Unraveling the Mystery Behind this Complex Condition”. Nursing for Women’s Health. 12 (5): 382–395. doi:10.1111/j.1751-486X.2008.00362.x. ISSN 1751-4851.
        20. ↑ Suh WN, Lee SK, Chang H, Hwang HJ, Hyung WJ, Park YN, Kim TI (June 2007). “Sclerosing encapsulating peritonitis (abdominal cocoon) after abdominal hysterectomy”. Korean J. Intern. Med. 22 (2): 125–9. PMC 2687622. PMID 17616031.
        21. ↑ Al Ani AH, Al Zayani N, Najmeddine M, Jacob S, Nair S (2014). “Idiopathic sclerosing encapsulating peritonitis (abdominal cocoon) in adult male. A case report”. Int J Surg Case Rep. 5 (10): 735–8. doi:10.1016/j.ijscr.2014.07.017. PMC 4189066. PMID 25217877.
        22. ↑ Sharma D, Nair RP, Dani T, Shetty P (2013). “Abdominal cocoon-A rare cause of intestinal obstruction”. Int J Surg Case Rep. 4 (11): 955–7. doi:10.1016/j.ijscr.2013.08.004. PMC 3825929. PMID 24055916.
        23. ↑ Machado NO (May 2016). “Sclerosing Encapsulating Peritonitis: Review”. Sultan Qaboos Univ Med J. 16 (2): e142–51. doi:10.18295/squmj.2016.16.02.003. PMC 4868512. PMID 27226904.
        24. ↑ Liang L, Zhang Y, Malpica A, Ramalingam P, Euscher ED, Fuller GN, Liu J (December 2015). “Gliomatosis peritonei: a clinicopathologic and immunohistochemical study of 21 cases”. Mod. Pathol. 28 (12): 1613–20. doi:10.1038/modpathol.2015.116. PMC 4682736. PMID 26564007.
        25. ↑ Lovell MA, Ross GW, Cooper PH (April 1989). “Gliomatosis peritonei associated with a ventriculoperitoneal shunt”. Am. J. Clin. Pathol. 91 (4): 485–7. PMID 2648802.
        26. ↑ Nielsen SN, Scheithauer BW, Gaffey TA (November 1985). “Gliomatosis peritonei”. Cancer. 56 (10): 2499–503. PMID 2412689.
        27. ↑ Ohara T, Yamanoi K, Inayama Y, Ogura J, Sakai M, Suzuki H, Hirayama T, Yasumoto K, Suginami K (July 2018). “Gliomatosis peritonei with 18F-fluorodeoxyglucose accumulation and contrast enhancement secondary to immature teratoma: A case report”. Mol Clin Oncol. 9 (1): 40–43. doi:10.3892/mco.2018.1618. PMC 6031035. PMID 29977538.
        28. ↑ Menéndez-Sánchez P, Villarejo-Campos P, Padilla-Valverde D, Murillo-Lázaro C, Martín-Fernández J (2011). “Gliomatosis peritonei: recurrence, treatment and surveillance”. Cir Cir. 79 (3): 256–9, 278–81. PMID 22380998.
        29. ↑ Wang, Dan; Jia, Cong-wei; Feng, Rui-e; Shi, Hong-hui; Sun, Juan (2016). “Gliomatosis peritonei: a series of eight cases and review of the literature”. Journal of Ovarian Research. 9 (1). doi:10.1186/s13048-016-0256-5. ISSN 1757-2215.
        30. ↑ 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.
        31. ↑ Moon C, Choi YJ, Kim EY, Lee IS, Kim SB, Jung SM, Kim SK, Chang J, Jung JY (March 2013). “Combined intrathoracic and intraperitoneal splenosis after splenic injury: case report and review of the literature”. Tuberc Respir Dis (Seoul). 74 (3): 134–9. doi:10.4046/trd.2013.74.3.134. PMC 3617134. PMID 23579787.
        32. ↑ Kim KA, Park CM, Kim CH, Choi SY, Park SW, Kang EY, Seol HY, Cha IH (December 2003). “An interesting hepatic mass: splenosis mimicking a hepatocellular carcinoma (2003:9b)”. Eur Radiol. 13 (12): 2713–5. PMID 14705605.
        33. ↑ Kim KA, Park CM, Kim CH, Choi SY, Park SW, Kang EY, Seol HY, Cha IH (December 2003). “An interesting hepatic mass: splenosis mimicking a hepatocellular carcinoma (2003:9b)”. Eur Radiol. 13 (12): 2713–5. PMID 14705605.
        34. ↑ Garaci, Francesco Giuseppe; Grande, Michele; Villa, Massimo; Mancino, Stefano; Konda, Daniel; Attinà, Grazia Maria; Galatà, Gabriele; Simonetti, Giovanni (2009). “What is a reliable CT scan for diagnosing splenosis under emergency conditions?”. World Journal of Gastroenterology. 15 (29): 3684. doi:10.3748/wjg.15.3684. ISSN 1007-9327.
        35. ↑ Chang ES, Bachul P, Szura M, Szpor J, Okoń K, Walocha JA (September 2015). “Peritoneal “melanosis““. Pol J Pathol. 66 (3): 330–3. PMID 26619112.
        36. ↑ Gao R, Liu NF, Sheng XG (April 2010). “Malignant ovarian melanoma with extensive pelvic and peritoneal metastasis: a case report and literature review”. Chin J Cancer. 29 (4): 460–2. PMID 20346227.
        37. ↑ Kim SS, Nam JH, Kim SM, Choi YD, Lee JH (March 2010). “Peritoneal melanosis associated with mucinous cystadenoma of the ovary and adenocarcinoma of the colon”. Int. J. Gynecol. Pathol. 29 (2): 113–6. doi:10.1097/PGP.0b013e3181bb4182. PMID 20173496.
        38. ↑ De la Torre Mondragón L, Daza DC, Bustamante AP, Fascinetto GV (December 1997). “Gastric triplication and peritoneal melanosis”. J. Pediatr. Surg. 32 (12): 1773–5. PMID 9434025.
        39. ↑ . doi:10.5114/pjp.2015.549. Missing or empty |title= (help)
        40. ↑ Maves, C K; Johnson, J F; Bove, K; Malott, R L (1989). “Gastric inflammatory pseudotumor in children”. Radiology. 173 (2): 381–383. doi:10.1148/radiology.173.2.2678252. ISSN 0033-8419.

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        References


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        Epidemiology and Demographics

        Epidemiology and Demographics

        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, it is more common in females and usually arises from an appendiceal tumor, but it can originate from ovary or colon as well. It has an incident rate of 0.1 cases per 100,000 people per year.

        Epidemiology and Demographics

        • The overall incidence of pseudomyxoma peritonei is 0.1 cases per 100,000 people per year. [1][2]
        • Pseudomyxoma peritonei is slightly more common in females than males.
        • The median age at presentation is typically about 50 years of age.

        References

        1. ↑ Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA (February 2008). “Appendiceal neoplasms and pseudomyxoma peritonei: a population based study”. Eur J Surg Oncol. 34 (2): 196–201. doi:10.1016/j.ejso.2007.04.002. PMID 17524597.
        2. ↑ Baratti D, Kusamura S, Nonaka D, Langer M, Andreola S, Favaro M, Gavazzi C, Laterza B, Deraco M (February 2008). “Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC)”. Ann. Surg. Oncol. 15 (2): 526–34. doi:10.1245/s10434-007-9691-2. PMID 18043976.


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        Risk Factors

        Risk Factors

        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 an uncommon tumor known for its production of abundant mucus in the abdominal cavity. It is 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 will eventually build up to the point where it compresses vital organs, including the colon, the liver, kidneys, pancreas. There is no known risk factors for pseudomyxoma peritonei.

        Risk Factors

        There are no established risk factors for pseudomyxoma peritonei.

        References


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        Natural History, Complications and Prognosis

        Natural History, Complications and Prognosis

        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

        Prognosis

        References

        1. ↑ 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.
        2. ↑ 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.
        3. ↑ 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.
        4. ↑ 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.
        5. ↑ 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.
        6. ↑ 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.
        7. ↑ 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.


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