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Presented by Ralph Hruban, M.D. and prepared by Angelique W. Levi, M.D.
Case 3: This 65-year-old woman presented with vague abdominal pain. CT Scan revealed a cystic mass in the tail of the pancreas.
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1. Question
Week 15: Case 3
This 65-year-old woman presented with vague abdominal pain. CT Scan revealed a cystic mass in the tail of the pancreas.
Correct
Answer: Mucinous cystic neoplasm with in situ carcinoma
Histology: This cystic neoplasm is lined by papillary fronds of mucin producing epithelium. At higher magnification, these epithelial cells can be seen to be remarkable for significant pleomorphism. In addition, a dense “ovarian type” stroma is seen.
Discussion: Mucinous cystic neoplasms range from tumors with small cysts lined by a single layer of benign appearing columnar epithelial to neoplasms composed of large cysts with an associating infiltrating carcinoma. These tumors are more common in women than they are in men and the mean age of diagnosis is in the late 5th decade. Patients usually present with vague abdominal pain or discomfort. In contrast to intraductal papillary mucinous neoplasms, the cysts of mucinous cystic neoplasms do not communicate with pancreatic ducts. The cysts in mucinous cystic neoplasms are lined by tall, mucin-producing, columnar cells, and characteristically there is a dense stroma surrounding the epithelial cells. This stroma resembles ovarian stroma. Immunohistochemical staining will demonstrate the expression of carcinoembryonic antigen, carbohydrate antigen 19-9, cytokeratin, and epithelial membrane antigen by the epithelial cells. Focal endocrine differentiation, including the expression of serotonin, gastrin, and somatostatin can be seen in 40% of the tumors. The stromal cells may express inhibin and estrogen receptors, and rare cases have been reported in which the stroma has a sarcomatous appearance. When an infiltrating carcinoma is present, it often produces large quantities of mucin. K-ras gene mutations, p53 immunolabeling, and loss of DPC4 expression are all more common in invasive mucinous cystadeno carcinomas then they are in non-invasive mucinous cystic neoplasms. The behavior of the mucinous cystic neoplasms has long been debated. Thompson et al. recently published a paper in the Am J Surg Pathol in which they contend that histologically benign mucinous cystic tumors can recur and even metastasize and they suggest that all mucinous cystic neoplasms of the pancreas should be called “mucinous cystic neoplasms of low malignant potential”. We recently reviewed the experience at Hopkins, and when completely resected and completely examined histologically, no mucinous cystadenoma, borderline mucinous cystic neoplasm, or mucinous cystic neoplasm with in situ carcinoma recurred or metastasized. We therefore feel it would be inappropriate to label a patient with a mucinous cystic neoplasm that has not invaded as having a carcinoma.
Incorrect
Answer: Mucinous cystic neoplasm with in situ carcinoma
Histology: This cystic neoplasm is lined by papillary fronds of mucin producing epithelium. At higher magnification, these epithelial cells can be seen to be remarkable for significant pleomorphism. In addition, a dense “ovarian type” stroma is seen.
Discussion: Mucinous cystic neoplasms range from tumors with small cysts lined by a single layer of benign appearing columnar epithelial to neoplasms composed of large cysts with an associating infiltrating carcinoma. These tumors are more common in women than they are in men and the mean age of diagnosis is in the late 5th decade. Patients usually present with vague abdominal pain or discomfort. In contrast to intraductal papillary mucinous neoplasms, the cysts of mucinous cystic neoplasms do not communicate with pancreatic ducts. The cysts in mucinous cystic neoplasms are lined by tall, mucin-producing, columnar cells, and characteristically there is a dense stroma surrounding the epithelial cells. This stroma resembles ovarian stroma. Immunohistochemical staining will demonstrate the expression of carcinoembryonic antigen, carbohydrate antigen 19-9, cytokeratin, and epithelial membrane antigen by the epithelial cells. Focal endocrine differentiation, including the expression of serotonin, gastrin, and somatostatin can be seen in 40% of the tumors. The stromal cells may express inhibin and estrogen receptors, and rare cases have been reported in which the stroma has a sarcomatous appearance. When an infiltrating carcinoma is present, it often produces large quantities of mucin. K-ras gene mutations, p53 immunolabeling, and loss of DPC4 expression are all more common in invasive mucinous cystadeno carcinomas then they are in non-invasive mucinous cystic neoplasms. The behavior of the mucinous cystic neoplasms has long been debated. Thompson et al. recently published a paper in the Am J Surg Pathol in which they contend that histologically benign mucinous cystic tumors can recur and even metastasize and they suggest that all mucinous cystic neoplasms of the pancreas should be called “mucinous cystic neoplasms of low malignant potential”. We recently reviewed the experience at Hopkins, and when completely resected and completely examined histologically, no mucinous cystadenoma, borderline mucinous cystic neoplasm, or mucinous cystic neoplasm with in situ carcinoma recurred or metastasized. We therefore feel it would be inappropriate to label a patient with a mucinous cystic neoplasm that has not invaded as having a carcinoma.
