Mucinous cystadenocarcinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2], Ammu Susheela, M.D. [3]

Overview

Mucinous cystadenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous carcinomas. The organs involved in pathogenesis of mucinous cystadenoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth gray surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous cystadenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous cystadenocarcinoma.

Pathogenesis

  • Mucinous adenocarcinoma is one of the most aggressive forms of cancer.

Mucinous Cystadenocarcinoma of Ovary

  • Mucinous cystadenocarcinoma of the ovary is a rare malignant ovarian mucinous tumor that originates from the ovarian epithelium.
  • 3 to 4 percent of primary ovarian cancers account for mucinous cystadenocarcinoma.[1] [2][3]
  • Women are affected in their late 40s to early 50s in the perimenopausal stage.[4]
  • Around 80 percent are mucinous cystadenomas, the majority are borderline and rest are malignant tumors.[1][5][6][7]
  • Majority of mucinous carcinomas of the ovary are metastasized from another site, often from the gastrointestinal tract.[2]
  • Retrospective studies have suggested that many mucinous carcinomas initially diagnosed as primary to the ovary have in fact metastasized from another sites.
  • Primary ovarian mucinous carcinomas usually evolve from mucinous borderline neoplasms of the ovary.[1][4][6]
  • KRAS mutations are found in mucinous carcinomas[8]

Mucinous Cystadenocarcinoma of Pancreas

  • Mucinous adenocarcinoma of the pancreas largely .occur in the body or tail of the pancreas, and less commonly in the head of the pancreas.[9]
  • WHO have classified MCN into three categories depending on the epithelial dysplasia :[10][11]
    • Low grade
    • Intermediate
    • High grade[12]
      • Non-invasive
      • Invasive[13]

Mucinous Cystadenocarcinoma of Appendix

  • Most common tumor of appendix.
  • The tumor produces mucous as well as spread to the organs.
  • Excess spread of the tumor to the abdomen is called Peritoneal Mucinous Carcinomatosis (PMCA).[14][15]
  • There are two types:[16]
    • Non-neoplastic appendiceal mucinous lesions
    • Neoplastic appendiceal mucinous lesions
  • Chronic obstruction can lead to mucinous cysts known as retention cysts.
  • Neoplastic appendiceal mucinous lesions contain serrated polyps.
  • Cysts are formed due to obstruction of the lumen by a fecalith, endometriosis involving the appendix, local process leading to focal scarring.

Associated Conditions

  • Mucinous cystadenocarcinoma is associated with mature cystic teratoma.

Gross Pathology

Mucinous Cystadenocarcinoma of Ovary

  • 8 to 20 cm in size.[3]
  • Cystic or solid.
  • Unilateral and confined to the ovary.
  • Smooth external surface.
  • Intact surface of the ovary without external implants.[1]
  • Inovolvement of surface of ovary in case of metstized tumor.[17][18]
  • Rarely, mucinous cystadenocarcinoma can lead to pseudomyxoma peritonei.[4][19][20]
  • Pseudomyxoma peritonei has following features:
  • Multiloculated
  • Sticky, gelatinous fluid (glycoprotein)
  • Necrosis
  • Typically unilateral
  • Smooth gray surface
  • Internal surface comprised of multilocular mass with thin walls
  • Mucinous material and solid nodules on the wall
Multiple cysts in the ovary, typical of mucinous cystadenocarcinoma of the ovary. Source: Wikimedia commons

Mucinous Cystadenoma of Pancreas

  • Mucin-producing intraductal neoplasm
  • Sharply demarcated
  • Cystic masses with a thick fibrous covering
  • No contact with the pancreatic duct system[21]

Mucinous Cystadenoma of Appendix

  • Non-neoplastic appendiceal mucinous lesions: retention cysts or mucoceles.[22][23]
  • Neoplastic appendiceal mucinous lesions: polypoid lesions

Microscopic Pathology

Microscopic features:

Following features are common to mucinous cystadenocarcinoma of all regions:

  • Mucinous differentiation
  • Tall columnar cells with apical mucin
  • Endocervical or intestinal-like appearance
  • Back-to-back cribriform glands with confluent growth pattern
  • Invasive morphology
  • Desmoplastic stromal response
  • Infiltration of the tumor capsule

Malignant characteristics on microscopy:

Mucinous Cystadenocarcinoma of Ovary

  • Complex glandular structure.[4]
  • Stromal invasion.[24]
  • Expanding pattern of growth(back to back glands with minimal intervening stroma). [3]
  • Infiltration of stroma in the form clusters of glands and nest.
  • Columnar epithelium of glands with the eosinophilic lake of mucin inside.
  • Desmoplastic stromal reaction.

Immunophenotype:

Molecular biology:

Mucinous Cystadenoma of Pancreas

  • Tumor is composed of:[21]
    • Columnar epithelium
    • Ovarian-type stroma
    • Luminal mucin
  • Epithelium may form a single layer or papillary folds.
  • May show mitoses
  • Stroma is composed of small spindle-shaped cells

Immunohistochemistry[21]

Stroma is usually positive for:

  • Estrogen and progesterone
  • Inhibin
  • Calretinin

Mucinous Cystadenoma of Appendix

  • Can be non-neoplastic or neoplastic depending on the features.
  • Non-neoplastic appendiceal mucinous lesions:
    • Mucin filled simple mucoceles
    • Epithelial flattening
    • Mucin expulsion
  • Neoplastic appendiceal mucinous lesions:[37][38][39][40][41]
    • Mixed histopathological characteristics
    • Serrated polypoid form
    • Dysplastic lesions
    • Hyperplasia
    • Proteoglycan-rich extracellular matrix
    • Desmoplastic reaction
    • stromal invasion

Reference

  1. ↑ 1.0 1.1 1.2 1.3 Hart WR, Norris HJ (May 1973). "Borderline and malignant mucinous tumors of the ovary. Histologic criteria and clinical behavior". Cancer. 31 (5): 1031–45. PMID 4735836.
  2. ↑ 2.0 2.1 Riopel MA, Ronnett BM, Kurman RJ (June 1999). "Evaluation of diagnostic criteria and behavior of ovarian intestinal-type mucinous tumors: atypical proliferative (borderline) tumors and intraepithelial, microinvasive, invasive, and metastatic carcinomas". Am. J. Surg. Pathol. 23 (6): 617–35. PMID 10366144.
  3. ↑ 3.0 3.1 3.2 Hoerl HD, Hart WR (December 1998). "Primary ovarian mucinous cystadenocarcinomas: a clinicopathologic study of 49 cases with long-term follow-up". Am. J. Surg. Pathol. 22 (12): 1449–62. PMID 9850171.
  4. ↑ 4.0 4.1 4.2 4.3 Lee KR, Scully RE (November 2000). "Mucinous tumors of the ovary: a clinicopathologic study of 196 borderline tumors (of intestinal type) and carcinomas, including an evaluation of 11 cases with 'pseudomyxoma peritonei'". Am. J. Surg. Pathol. 24 (11): 1447–64. PMID 11075847.
  5. ↑ Bladt O, De Man R, Aerts R (2004). "Mucinous cystadenoma of the ovary". JBR-BTR. 87 (3): 118–9. PMID 15293671.
  6. ↑ 6.0 6.1 de Nictolis M, Montironi R, Tommasoni S, Valli M, Pisani E, Fabris G, Prat J (January 1994). "Benign, borderline, and well-differentiated malignant intestinal mucinous tumors of the ovary: a clinicopathologic, histochemical, immunohistochemical, and nuclear quantitative study of 57 cases". Int. J. Gynecol. Pathol. 13 (1): 10–21. PMID 8112952.
  7. ↑ Hart WR (January 2005). "Mucinous tumors of the ovary: a review". Int. J. Gynecol. Pathol. 24 (1): 4–25. PMID 15626914.
  8. ↑ Ovary Epithelial tumors. Atlasgeneticsoncology (2016).http://atlasgeneticsoncology.org/Tumors/OvaryEpithTumID5230.html Accessed on February 29, 2016
  9. ↑ Klöppel G (February 2007). "Chronic pancreatitis, pseudotumors and other tumor-like lesions". Mod. Pathol. 20 Suppl 1: S113–31. doi:10.1038/modpathol.3800690. PMID 17486047.
  10. ↑ Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T (December 2015). "A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas". Am. J. Surg. Pathol. 39 (12): 1730–41. doi:10.1097/PAS.0000000000000533. PMC 4646710. PMID 26559377.
  11. ↑ Bernard P, Scoazec JY, Joubert M, Kahn X, Le Borgne J, Berger F, Partensky C (November 2002). "Intraductal papillary-mucinous tumors of the pancreas: predictive criteria of malignancy according to pathological examination of 53 cases". Arch Surg. 137 (11): 1274–8. PMID 12413317.
  12. ↑ Crippa S, Salvia R, Warshaw AL, Domínguez I, Bassi C, Falconi M, Thayer SP, Zamboni G, Lauwers GY, Mino-Kenudson M, Capelli P, Pederzoli P, Castillo CF (April 2008). "Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients". Ann. Surg. 247 (4): 571–9. doi:10.1097/SLA.0b013e31811f4449. PMC 3806104. PMID 18362619.
  13. ↑ Baker ML, Seeley ES, Pai R, Suriawinata AA, Mino-Kenudson M, Zamboni G, Klöppel G, Longnecker DS (December 2012). "Invasive mucinous cystic neoplasms of the pancreas". Exp. Mol. Pathol. 93 (3): 345–9. doi:10.1016/j.yexmp.2012.07.005. PMID 22902940.
  14. ↑ Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ (January 2016). "A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process". Am. J. Surg. Pathol. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. PMID 26492181.
  15. ↑ Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ (January 2016). "A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process". Am. J. Surg. Pathol. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. PMID 26492181.
  16. ↑ 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.
  17. ↑ Prayson RA, Hart WR, Petras RE (June 1994). "Pseudomyxoma peritonei. A clinicopathologic study of 19 cases with emphasis on site of origin and nature of associated ovarian tumors". Am. J. Surg. Pathol. 18 (6): 591–603. PMID 8179074.
  18. ↑ Young RH, Gilks CB, Scully RE (May 1991). "Mucinous tumors of the appendix associated with mucinous tumors of the ovary and pseudomyxoma peritonei. A clinicopathological analysis of 22 cases supporting an origin in the appendix". Am. J. Surg. Pathol. 15 (5): 415–29. PMID 2035736.
  19. ↑ McKenney JK, Soslow RA, Longacre TA (May 2008). "Ovarian mature teratomas with mucinous epithelial neoplasms: morphologic heterogeneity and association with pseudomyxoma peritonei". Am. J. Surg. Pathol. 32 (5): 645–55. doi:10.1097/PAS.0b013e31815b486d. PMID 18344868.
  20. ↑ Ronnett BM, Seidman JD (May 2003). "Mucinous tumors arising in ovarian mature cystic teratomas: relationship to the clinical syndrome of pseudomyxoma peritonei". Am. J. Surg. Pathol. 27 (5): 650–7. PMID 12717249.
  21. ↑ 21.0 21.1 21.2 Masia R, Mino-Kenudson M, Warshaw AL, Pitman MB, Misdraji J (February 2011). "Pancreatic mucinous cystic neoplasm of the main pancreatic duct". Arch. Pathol. Lab. Med. 135 (2): 264–7. doi:10.1043/1543-2165-135.2.264. PMID 21284448.
  22. ↑ Raijman I, Leong S, Hassaram S, Marcon NE (March 1994). "Appendiceal mucocele: endoscopic appearance". Endoscopy. 26 (3): 326–8. doi:10.1055/s-2007-1008979. PMID 8076556.
  23. ↑ Mizuma N, Kabemura T, Akahoshi K, Yasuda D, Okabe H, Chijiiwa Y, Nawata H, Matsui N (December 1997). "Endosonographic features of mucocele of the appendix: report of a case". Gastrointest. Endosc. 46 (6): 549–52. PMID 9434225.
  24. ↑ Rodríguez IM, Prat J (February 2002). "Mucinous tumors of the ovary: a clinicopathologic analysis of 75 borderline tumors (of intestinal type) and carcinomas". Am. J. Surg. Pathol. 26 (2): 139–52. PMID 11812936.
  25. ↑ Vang R, Gown AM, Barry TS, Wheeler DT, Yemelyanova A, Seidman JD, Ronnett BM (September 2006). "Cytokeratins 7 and 20 in primary and secondary mucinous tumors of the ovary: analysis of coordinate immunohistochemical expression profiles and staining distribution in 179 cases". Am. J. Surg. Pathol. 30 (9): 1130–9. doi:10.1097/01.pas.0000213281.43036.bb. PMID 16931958.
  26. ↑ Baker PM, Oliva E (January 2005). "Immunohistochemistry as a tool in the differential diagnosis of ovarian tumors: an update". Int. J. Gynecol. Pathol. 24 (1): 39–55. PMID 15626916.
  27. ↑ McCluggage WG (April 2006). "Immunohistochemical and functional biomarkers of value in female genital tract lesions". Int. J. Gynecol. Pathol. 25 (2): 101–20. doi:10.1097/01.pgp.0000192269.14666.68. PMID 16633059.
  28. ↑ Vang R, Gown AM, Barry TS, Wheeler DT, Ronnett BM (January 2006). "Immunohistochemistry for estrogen and progesterone receptors in the distinction of primary and metastatic mucinous tumors in the ovary: an analysis of 124 cases". Mod. Pathol. 19 (1): 97–105. doi:10.1038/modpathol.3800510. PMID 16294196.
  29. ↑ Vang R, Gown AM, Wu LS, Barry TS, Wheeler DT, Yemelyanova A, Seidman JD, Ronnett BM (November 2006). "Immunohistochemical expression of CDX2 in primary ovarian mucinous tumors and metastatic mucinous carcinomas involving the ovary: comparison with CK20 and correlation with coordinate expression of CK7". Mod. Pathol. 19 (11): 1421–8. doi:10.1038/modpathol.3800698. PMID 16980943.
  30. ↑ Vang R, Gown AM, Farinola M, Barry TS, Wheeler DT, Yemelyanova A, Seidman JD, Judson K, Ronnett BM (May 2007). "p16 expression in primary ovarian mucinous and endometrioid tumors and metastatic adenocarcinomas in the ovary: utility for identification of metastatic HPV-related endocervical adenocarcinomas". Am. J. Surg. Pathol. 31 (5): 653–63. doi:10.1097/01.pas.0000213369.71676.25. PMID 17460447.
  31. ↑ Gemignani ML, Schlaerth AC, Bogomolniy F, Barakat RR, Lin O, Soslow R, Venkatraman E, Boyd J (August 2003). "Role of KRAS and BRAF gene mutations in mucinous ovarian carcinoma". Gynecol. Oncol. 90 (2): 378–81. PMID 12893203.
  32. ↑ Mayr D, Hirschmann A, Löhrs U, Diebold J (December 2006). "KRAS and BRAF mutations in ovarian tumors: a comprehensive study of invasive carcinomas, borderline tumors and extraovarian implants". Gynecol. Oncol. 103 (3): 883–7. doi:10.1016/j.ygyno.2006.05.029. PMID 16806438.
  33. ↑ Cuatrecasas M, Villanueva A, Matias-Guiu X, Prat J (April 1997). "K-ras mutations in mucinous ovarian tumors: a clinicopathologic and molecular study of 95 cases". Cancer. 79 (8): 1581–6. PMID 9118042.
  34. ↑ Kuo KT, Guan B, Feng Y, Mao TL, Chen X, Jinawath N, Wang Y, Kurman RJ, Shih I, Wang TL (May 2009). "Analysis of DNA copy number alterations in ovarian serous tumors identifies new molecular genetic changes in low-grade and high-grade carcinomas". Cancer Res. 69 (9): 4036–42. doi:10.1158/0008-5472.CAN-08-3913. PMC 2782554. PMID 19383911. Vancouver style error: initials (help)
  35. ↑ Shi H, Wang MX, Caldwell CW (September 2007). "CpG islands: their potential as biomarkers for cancer". Expert Rev. Mol. Diagn. 7 (5): 519–31. doi:10.1586/14737159.7.5.519. PMID 17892361.
  36. ↑ Kurman RJ, Shih I (April 2008). "Pathogenesis of ovarian cancer: lessons from morphology and molecular biology and their clinical implications". Int. J. Gynecol. Pathol. 27 (2): 151–60. doi:10.1097/PGP.0b013e318161e4f5. PMC 2794425. PMID 18317228. Vancouver style error: initials (help)
  37. ↑ Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ (January 2016). "A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process". Am. J. Surg. Pathol. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. PMID 26492181.
  38. ↑ 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.
  39. ↑ Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ (January 2016). "A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process". Am. J. Surg. Pathol. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. PMID 26492181.
  40. ↑ Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ (January 2016). "A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process". Am. J. Surg. Pathol. 40 (1): 14–26. doi:10.1097/PAS.0000000000000535. PMID 26492181.
  41. ↑ Aho AJ, Heinonen R, Laurén P (1973). "Benign and malignant mucocele of the appendix. Histological types and prognosis". Acta Chir Scand. 139 (4): 392–400. PMID 4718184.

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