Table 2.
Histopathologic features of epithelial mucinous tumors of appendiceal, colonic, and small bowel origin are designated as disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA).
Features | DPAM | PMCA |
Primary site | Appendix | Appendix, colon, small intestine |
Primary diagnosis | Mucinous adenoma usually in a mucocoele | Mucinous adenocarcinoma |
Surgical appearance | Mucinous tumors and mucinous ascites with redistribution | Carcinomatosis with variable amounts of mucinous ascites, redistribution is prominent with large volume of ascites |
Peritoneal tumor | ||
• Cellularity | Scant | Moderate to abundant |
• Morphology | Abundant extracellular mucin containing simple to focally proliferative mucinous epithelium. There is a single layer of cells | Moderate to abundant extracellular mucin containing extensively proliferative mucinous epithelium or mucinous glands, clusters of cells, or individual cells consistent with carcinoma |
• Cytologic atypia | Minimal | Moderate to marked |
• Mitotic activity | Rare | Infrequent to frequent |
Lymph node involvement | Almost never | Moderate |
Liver metastases | Almost never | Very infrequent |
Parenchymal organ invasion | Rare (except ovary) | Frequent |
Hybrid type tumors show less than 5% of PMCA within DPAM. Mucinous carcinomas are divided into three grades by maintenance or loss of glandular architecture.