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 adenocarcinoma 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 adenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.
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]
Mucinous Cystadenoma 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.
Mucinous Cystadenoma 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).
Mucinous Cystadenoma of Colon and Rectum
- Most common type of colorectal cancer
Genetics
Mucinous Cystadenoma of Ovary
- KRAS mutations are found in mucinous carcinomas[8]
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.[9][10]
- Rarely, mucinous cystadenocarcinoma can lead to pseudomyxoma peritonei.[4][11][12]
- Pseudomyxoma peritonei has following features:
- Multiloculated
- Sticky, gelatinous fluid (glycoprotein)
- Necrosis
- Typically unilateral
- Smooth gray surface
- Internal surface comprised a multilocular mass with thin walls and mucinous material only, while a small area exhibited solid nodules on the wall
Mucinous Cystadenoma of Pancreas
- Mucin-producing intraductal neoplasm
- Sharply demarcated
- Cystic masses with a thick fibrous covering
- No contact with the pancreatic duct system[13]
Microscopic Pathology
Microscopic features:
Following features are common to mucinous cystadenocarcinoma of all regions:
- Mucinous differentiation
- Tall columnar cells in glands with apical mucin
- May has an endocervical-like or intestinal-like appearance
- Invasive morphology
- Back-to-back glands/confluent growth pattern
- Desmoplastic stromal response
- Cribriform of glands
- Infiltration the tumor capsule
Malignant characteristics on microscopy:
- Nuclear atypia
- Necrosis
- Absent cilia
Mucinous Cystadenocarcinoma of Ovary
- Complex glandular structure.[4]
- Stromal invasion.[14]
- 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:
- Gastrointestinal markers CK20 and CDX2 positive.[15]
- CK7 expression.[16][17][18][19]
- p16 expression.[20]
Molecular biology:
- KRAS mutation in 75 percent of cases.[21][22][23]
- Mucin genes (MUC2, MUC3, and MUC17) positivity.[24][25][26]
Mucinous Cystadenoma of Pancreas
Tumor is composed of two components:[13]
- Columnar epithelium
- Ovarian-type stroma
Immunohistochemistry[13]
Stroma is usually positive for:
- Estrogen and progesterone
- Inhibin
- Calretinin
Reference
- ↑ 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.
- ↑ 2.0 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.
- ↑ 3.0 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.
- ↑ 4.0 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.
- ↑ Bladt O, De Man R, Aerts R (2004). "Mucinous cystadenoma of the ovary". JBR-BTR. 87 (3): 118–9. PMID 15293671.
- ↑ 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.
- ↑ Hart WR (January 2005). "Mucinous tumors of the ovary: a review". Int. J. Gynecol. Pathol. 24 (1): 4–25. PMID 15626914.
- ↑ Ovary Epithelial tumors. Atlasgeneticsoncology (2016).http://atlasgeneticsoncology.org/Tumors/OvaryEpithTumID5230.html Accessed on February 29, 2016
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 13.0 13.1 13.2 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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)
- ↑ 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.
- ↑ 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)