Mucinous cystadenocarcinoma pathophysiology

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

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 ovarian epithelium.
• 3 to 4 percent of primary ovarian cancers accounts for mucinous cyst adenocarcinoma.
• Women are affected in their late 40s to early 50s in perimenopausal stage.
• Around 80 percent are mucinous cystadenomas, majority are borderline and rest are malignant tumors.
• Majority of mucinous carcinomas of the ovary are metastasized from another site, often from gastrointestinal tract.
• Retrospective studies have suggested that many mucinous carcinomas initially diagnosed as primary to the ovary have in fact .

Mucinous Cystadenoma of Pancreas

• Mucinous cystadenoma of pancreas is a large uni/multilocular cystic pancreatic neoplasm lined by columnar mucinous epithelium. While mucinous cyst adenomas very infrequently communicate with the pancreatic duct, they can cause partial pancreatic ductal obstruction. They are considered premalignant or malignant lesions with usually elevated CEA and CA 19-9 serum levels.
• 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

Associated Conditions

• Mucinous cystadenocarcinoma is associated with mature cystic teratoma

Gross Pathology

Mucinous Cystadenocarcinoma of Ovary

• 8 to 20 cm in size.
• Cystic or solid.
• Unilateral and confined to the ovary.
• Smooth external surface.
• Intact surface of the ovary without external implants.
• Rarely, mucinous cystadenocarcinoma can lead to pseudomyxoma peritonei.

• 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

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.
• Stromal invasion.
• Expanding pattern of growth(back to back glands with minimal intervening stroma).
• 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.
  • CK7 expression.
• Molecular biology:
  • KRAS mutation in 75 percent of cases.
  • Mucin genes (MUC2, MUC3, and MUC17) positivity.