Saturday, May 19, 2007

Pathology wk 13-16: Pseudomyxoma Peritonei, mucocele








Pseudomyxoma Peritonei
Adenomucinosis, mucinous adenocarcinoma
Mucin goes around nodes
originates in order of appendix, ovary and gallbladder








Pseudomyxoma peritonei (PMP) is an uncommon tumor known for its production of mucus in the abdominal cavity.[1] The tumor is not harmful by itself, but the mucus has no place to go inside the abdominal cavity. If left untreated, it will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc.
Unlike most cancers, PMP does not spread through the lymphatic system or through the bloodstream. PMP is most commonly associated with cancer of the appendix; mucinous tumors of the ovary have also been implicated.

The disorder combines the presence of an ovarian tumor with extensive mucinous ascites, cystic epithelial implants on the peritoneal surfaces, and adhesions. Pseudoyxoma peritonei, if extensive, may result in intestinal obstruction and death. Recent evidence strongly supports an extraovarian (usually appendiceal) primary mucinous tumor in many cases, with secondary ovarian and peritoneal spread.
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A mucocele, also referred to as mucus extravasation phenomenon, is a swelling of connective tissue consisting of collected mucin from a ruptured salivary gland duct, which is usually caused by local trauma. The mucocele is a bluish translucent color, and is more commonly found in children and young adults.















Figure 30A cross section of the dilated appendix in a patient with large volume pseudomyxoma peritonei. The lumen of the appendix contains areas that show adenomatous change. The distal portion of the appendix contains mucinous material and multiple adenomatous epithelial cells. The presence of adenomatous epithelial cells outside of the intestinal lumen establishes the diagnosis of pseudomyxoma peritonei. (H&E x 10).