Pseudomyxoma peritonei differential diagnosis

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

Differential Diagnosis

Pseudomyxoma peritonei must be differentiated from:[1][2]

Differentiating pseudomyxoma peritonei from other diseases

  • On the basis of findings on CT scan such as visceral scalloping and histopathology, pseudomyxoma peritonei must be differentiated from other similar rare diseases such as Peritoneal carcinomatosis without muscinous ascites, Tubercluosis peritonitis, Sarcomatosis peritonei.
Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Signs
Lab Findings Imaging Histopathology
Symptom Constitutional symptoms Physical exam Other lab values Immunohistochemistry CT Scan Ultrasound
Pseudomyxoma peritonei[3][4]
  • Bloating
  • Abdominal distention
  • Negative
  • Non specific
  • CK 20, CDX2 and MUC2,MUC5AC
  • Low-attenuation
  • Scalloping of the visceral surfaces differentiates pseudomyxoma from other causes of peritnoitis.
  • Typically does not invade visceral organs or spread by lymphatic or hematogenous routes unlike mucinous carcinomatosis
pseudomyxoma peritonei CT [source:Case courtesy of Dr Maxime St-Amant, Radiopaedia.org, rID: 18623
  • The echoes within pseudomyxoma peritonei are not mobile
  • Echogenic septations within the gelatinous ascites are frequently observed.
  • Scalloping of the hepatic and splenic margins
  • Gelatinous ascites in peritoneum and visceral organs,usually right hemidiaphragm, liver.
  • Diagnostic laparascopy/laparatomy
Peritoneal carcinomatosis without mucinous ascites[5]
  • Abdominal pain
  • Abdominal distention
  • Negative
  • Abdominal distention
  • Abdominal pain
  • Non specific
  • Pleural effusion and/or masses in chest
  • Mesenteric or retroperitoneal lymphadenopathy
  • Omental caking, and invasion into parenchymal organs
CT of Peritoneal carcinomatosis with peritoneal thickening and omental deposits source:Subhaschandra Singh, Y. Sobita Devi, Shweta Bhalothia and Veeraraghavan Gunasekaran (2016). "Peritoneal Carcinomatosis: Pictorial Review of Computed Tomography Findings.". International Journal of Advanced Research 4 (7): 735–748. DOI:10.21474/IJAR01/936. ISSN 23205407. CC-BY 4.0
  • Echoes are mobile
  • No scalloping of visceral organs.
Peritoneal sarcomatosis
  • Abdominal pain
  • Abdominal distention
  • Bloating
  • Nausea
  • Negative
  • Abdominal mass
  • Non specific
  • Heterogeneous bulky masses
  • Hypervascularity with or without
  • Hemoperitoneum Variable presence of ascites.
  • CT-guided percutaneous tissue biopsy
Lymphomatosis
  • Abdominal pain
  • Bloating
  • Weight loss
  • CD20,
  • CD79a,
  • CD10
  • Mostly non-specific
  • Aneurysmal dilatation of the bowel loop with wall thickening may differentiate lymphomatosis from other peritoneal diseases.
  • Atypical lymphoid cells
  • Biopsy and histology
  • Rare manifestation of lymphoma, seen most frequently with non-Hodgkin lymphoma
  • Associated with Herpes virus 8 (HHV-8), which is also associated with Kaposi sarcoma, and Epstein-Barr virus
Tuberclousis Peritonitis[6][6]
  • Abdominal pain
  • Ascitic fluid will have an elevated white blood cell count with a lymphocytic predominance
  • Miliary microabscesses in the liver or spleen
  • lymph node calcification
  • Inflammatory thickening of the terminal ileum and cecum
  • low-attenuation lymphadenopathy
  • Caseating granuloma with central area of necrotic acellular debris surrounded by histiocytes
  • Laparoscopy and peritoneal biopsy
  • Tuberclousis
Endometriosis[7][8][9]
  • Menstrual cramps
  • Painful bowel movements
  • Painful urination during menstrual periods
  • Heavy menstrual periods
  • Pain during or after sex
  • Negative
  • Immobile uterine
  • Fixed and retroverted uterus
  • Tender adnexal mass
  • Tender uterosacral nodularity
  • Thomsen-Friedenreich (T) antigen (Gal beta1-3GalNAc)
  • Hypoechogenic linear thickening or nodules/masses
  • Endometrial-type glands and stroma, as well as hemosiderin-laden macrophages
  • Hemorrhagic, reddish brown or blue nodules or cysts on the peritoneal surfaces
  • Visual inspection with laparascopy
Sclerosing encapsulating peritonitis[10][11][12]
  • Fever,
  • Weight loss,
  • Loss of appetite
  • Altered bowel movement
  • Abdominal pain
  • Negative
  • Abdominal mass
  • Abdominal tenderness
  • Diffusely thickened peritoneum and ascites
  • Small bowel may be matted within loculated fluid collections
  • Encapsulated small bowel
  • Thin echogenic strands can be seen within ascitic fluid;
  • Tethered small bowel
  • Dense,opaque peritoneum
  • Progressive collagen formation with an associated chronic inflammatory infiltrate
  • Most commonly in patients who undergo continuous ambulatory peritoneal dialysis. It may also be idiopathic, associated with ventriculoperitoneal shunts, liver transplantation
Gliomatosis Peritonei[13][14]
  • Abdominal pain
  • Bloating
  • Negative
  • SOX2+/OCT4-/NANOG- immunophenotype
  • Mature glial tissue in the peritoneal cavity
  • Micronodular growth pattern
  • Association with solid or immature ovarian teratomas, but it has also been reported to occur in association with ventriculoperitoneal shunts
Osseous metaplasia
  • Abdominal pain
  • Nausea
  • Vomiting
  • Bloating
  • Negative
  • Multiple, high-attenuation, linear-branching structures within the mesentery that extend to the peritoneal surfaces
Splenosis
  • Increase in abdominal girth
  • Negative
  • Abdominal mass
  • Abdominal tenderness
  • Abdominal rigidity and guarding due to spleen rupture in some cases and retroperitoneal bleeding.
  • Lesions are typically multiple, small, reddish-brown nodules that range in size from a few millimeters to 7 cm
Melanosis[15][16][17][18]
  • Majority of cases are asymptomatic
  • Increase in abdominal girth
  • Negative
  • Pigmentation within the dermoid, omentum, and peritoneal cavity
  • Pigment-laden macrophages within the mucosa on PAS staining
  • Biopsy and histology
  • Enteric duplication cyst
  • Associated with ovarian cystic teratomas
  • Reported case of gastric triplication and its association with melanosis peritonei.
Inflammatory Pseudotumor[19]
  • Weight loss
  • Growth retardation
  • Symptoms of Anemia
  • Symptoms related to mass effect,
  • Negative
  • Polyclonal hypergammaglobulinemia
  • Stain positive for ALK with immunohistochemistry
  • IgG4 positive plasma cell
  • CT findings are non specific ascites, masses may be seen in peritoneum,mimic neoplasm.
  • Loose myxoid areas with interspersed spindle cells,
  • Variable vascularity
  • Areas of dense collagen, and dense spindled myofibroblasts admixed with inflammatory cells to include plasma cells and lymphocytes.
  • Chromosomal translocations involving the ALK gene

References

  1. ↑ Harmon RL, Sugarbaker PH (February 2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. doi:10.1186/1477-7800-2-3. PMC 549516. PMID 15701175.
  2. ↑ Carr NJ, Bibeau F, Bradley RF, Dartigues P, Feakins RM, Geisinger KR, Gui X, Isaac S, Milione M, Misdraji J, Pai RK, Rodriguez-Justo M, Sobin LH, van Velthuysen MF, Yantiss RK (December 2017). "The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei". Histopathology. 71 (6): 847–858. doi:10.1111/his.13324. PMID 28746986.
  3. ↑ 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.
  4. ↑ 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.
  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. ↑ 6.0 6.1 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ 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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