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
Pathogenesis
- Mucinous cystadenocarcinoma of the ovary is a rare malignant ovarian mucinous tumor. This type can account for 5-10% of all ovarian mucinous tumors. It is a type of ovarian epithelial tumor.
- Retrospective studies have suggested that many mucinous carcinomas initially diagnosed as primary to the ovary have in fact metastasised from another site.
Mucinous cystadenoma of pancreas
- It is a large uni/multilocular cystic pancreatic neoplasm lined by columnar mucinous epithelium. While mucinous cyst adenomas very infrequently communicate with the pancreatic duct 13, they can cause partial pancreatic ductal obstruction 11. They are considered premalignant or malignant lesions with usually elevated CEA and CA 19-9 serum levels.
- Largely (~80%) occur in the body or tail of the pancreas, and less commonly in the head of the pancreas (~20%)
Mucinous cystadenoma of Appendix
- Most common tumor of appendix.
- The tumor produces mucous as well as spread to the organs.
- Excess spread of tumor to abdomen is called Peritoneal Mucinous Carcinomatosis (PMCA).
Genetics
Mucinous cystadenoma of Lung
- MCACL has been noted in most cases to show areas of gradual transition wherein cells become more atypical and feature more pronounced characteristics of maignancy as one proceeds from the capsule, or outermost layers of the tumor, toward the center of the mass.[8] Some experts suggest that this tumor often develops after a slow progression from a relatively benign to a frankly malignant phenotype over a period of years to decades.
- The putative cell of origin of this tumor is unknown. Electron microscopic studies in 3 cases[12][13] described intracytoplasmic mucin, convoluted oval nuclei, prominent nucleoli, homogeneous euchromatin with peripheral chromatin condensation, microvilli, junctional complexes, and primitive lumen formation. Their failure to identify lamellar bodies and large dense granules seemed to rule out origination from either Clara cells or Type II pneumocytes.
- In a review of 66 cystic pulmonary mucinous lesions, Gao and colleagues reported that p53 expression and a Ki-67 index exceeding 20% is characteristic of MCACL.
Associated Conditions
- Malignant transformation of a mature cystic teratoma
Gross Pathology
Features:
Multiloculated. Sticky, gelatinous fluid (glycoprotein). +/-Necrosis. Typically unilateral.
- Smooth grey surface was received. Internal surface comprised a multilocular mass with thin walls and mucinous material only, while a small area exhibited solid nodules in the wall
Microscopic Pathology
Features:
Mucinous differentiation. Tall columnar cells in glands with apical mucin. May have an endocervical-like or intestinal-like appearance - see subtypes. Invasive morphology - one of the following: Back-to-back glands/confluent growth pattern. Desmoplastic stromal response. Cribriforming of glands. Malignant characteristics: +/-Nuclear atypia. +/-Necrosis. No cilia. Microscopically, tumor walls were covered with a single line of mucinous cells with small basal nuclei and mucinous cytoplasm. In addition, ovarian-like stroma was identified in the wall. These epithelial areas showed transitions with intraepithelial carcinoma (Figure 3) and areas of borderline mucinous tumor; high-grade adenocarcinoma with dedifferentiation in desmoplastic stroma was identified. High-grade adenocarcinomatous component infiltrated the tumor capsule was seen.