Mucinous cystadenocarcinoma pathophysiology: Difference between revisions

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===Mucinous Cystadenoma of Pancreas===
===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.<ref name="pmid17486047">{{cite journal |vauthors=Klöppel G |title=Chronic pancreatitis, pseudotumors and other tumor-like lesions |journal=Mod. Pathol. |volume=20 Suppl 1 |issue= |pages=S113–31 |date=February 2007 |pmid=17486047 |doi=10.1038/modpathol.3800690 |url=}}</ref>
* Mucinous adenocarcinoma of the pancreas largely .occur in the body or tail of the pancreas, and less commonly in the head of the pancreas.<ref name="pmid17486047">{{cite journal |vauthors=Klöppel G |title=Chronic pancreatitis, pseudotumors and other tumor-like lesions |journal=Mod. Pathol. |volume=20 Suppl 1 |issue= |pages=S113–31 |date=February 2007 |pmid=17486047 |doi=10.1038/modpathol.3800690 |url=}}</ref>
* WHO have classified MCN into three categories depending on the epithelial dysplasia :
* WHO have classified MCN into three categories depending on the epithelial dysplasia :<ref name="pmid26559377">{{cite journal |vauthors=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 |title=A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas |journal=Am. J. Surg. Pathol. |volume=39 |issue=12 |pages=1730–41 |date=December 2015 |pmid=26559377 |pmc=4646710 |doi=10.1097/PAS.0000000000000533 |url=}}</ref>
** Low grade
** Low grade
** Intermediate
** Intermediate

Revision as of 17:06, 5 April 2019

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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.[8]
  • WHO have classified MCN into three categories depending on the epithelial dysplasia :[9]
    • Low grade
    • Intermediate
    • High grade

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[10]

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.[11][12]
  • Rarely, mucinous cystadenocarcinoma can lead to pseudomyxoma peritonei.[4][13][14]
  • 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[15]

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:

Mucinous Cystadenocarcinoma of Ovary

  • Complex glandular structure.[4]
  • Stromal invasion.[16]
  • 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:[15]
    • Columnar epithelium
    • Ovarian-type stroma
    • Luminal mucin
  • Epithelium may forms a single layer or papillary folds.
  • May show mitoses
  • Stroma is cmposed of small spindle-shaped cells

Immunohistochemistry[15]

Stroma is usually positive for:

  • Estrogen and progesterone
  • Inhibin
  • Calretinin

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. 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.
  9. 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.
  10. Ovary Epithelial tumors. Atlasgeneticsoncology (2016).http://atlasgeneticsoncology.org/Tumors/OvaryEpithTumID5230.html Accessed on February 29, 2016
  11. 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.
  12. 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.
  13. 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.
  14. 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.
  15. 15.0 15.1 15.2 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.
  16. 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.
  17. 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.
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  19. 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.
  20. 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.
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