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Presented by Ralph Hruban, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 6: This elderly white female noted a gradually increasing abdominal girth.
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1. Question
Week 224: Case 6
This elderly white female noted a gradually increasing abdominal girth. An appendectomy was performed.images/may 23 2005 case 6 1.jpg
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images/may 23 2005 case 6 5.jpgCorrect
Answer: Disseminated peritoneal adenomucinosis
Histology: The appendix is remarkable for a ruptured mucinous adenoma. Associated with this are dissecting pools of relatively acellular mucin. Occassional neoplastic mucin-producing cells are present within the mucin. These lack significant architectural and cytologic atypia.
Discussion: Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by disseminated intraperitoneal mucinous tumors, often with mucinous ascites (see reference below). Pseudomyxoma peritonei is an historical term used for both benign and malignant mucinous neoplasms that produce abundant extracellular mucin. Because pseudomyxoma peritonei mixes histologically bland neoplasms with high-grade carcinomas, patients with pseudomyxoma peritonei have a variable and poorly predictable prognosis. A recent study reported a pathologic classification that can be used to separate patients into prognostically distinct groups. Those with histologically benign epithelium are designated disseminated peritoneal adenomucinosis, while those with histologically malignant features (significant architectural and cytologic atypia) are designated peritoneal mucinous carcinomatosis.
Reference(s):
– Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis.Cancer. Jul 1;92(1):85-91, 2001.Incorrect
Answer: Disseminated peritoneal adenomucinosis
Histology: The appendix is remarkable for a ruptured mucinous adenoma. Associated with this are dissecting pools of relatively acellular mucin. Occassional neoplastic mucin-producing cells are present within the mucin. These lack significant architectural and cytologic atypia.
Discussion: Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by disseminated intraperitoneal mucinous tumors, often with mucinous ascites (see reference below). Pseudomyxoma peritonei is an historical term used for both benign and malignant mucinous neoplasms that produce abundant extracellular mucin. Because pseudomyxoma peritonei mixes histologically bland neoplasms with high-grade carcinomas, patients with pseudomyxoma peritonei have a variable and poorly predictable prognosis. A recent study reported a pathologic classification that can be used to separate patients into prognostically distinct groups. Those with histologically benign epithelium are designated disseminated peritoneal adenomucinosis, while those with histologically malignant features (significant architectural and cytologic atypia) are designated peritoneal mucinous carcinomatosis.
Reference(s):
– Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis.Cancer. Jul 1;92(1):85-91, 2001.
