Genetic, immunohistochemical, and clinical features of medullary carcinoma of the pancreas: A newly described and characterized entity.

Authors

• R E Wilentz
• M Goggins
• M Redston
• V A Marcus
• N V Adsay
• T A Sohn
• S S Kadkol
• C J Yeo
• M Choti
• M Zahurak
• K Johnson
• M Tascilar
• G J Offerhaus
• R H Hruban
• S E Kern

Publication/Presentation Date

5-1-2000

Abstract

Medullary carcinomas of the pancreas are a recently described, histologically distinct subset of poorly differentiated adenocarcinomas that may have a unique pathogenesis and clinical course. To further evaluate these neoplasms, we studied genetic, pathological, and clinical features of 13 newly identified medullary carcinomas of the pancreas. Nine (69%) of these had wild-type K-ras genes, and one had microsatellite instability (MSI). This MSI medullary carcinoma, along with three previously reported MSI medullary carcinomas, were examined immunohistochemically for Mlh1 and Msh2 expression, and all four expressed Msh2 but did not express Mlh1. In contrast, all of the medullary carcinomas without MSI expressed both Msh2 and Mlh1. Remarkably, the MSI medullary carcinoma of the pancreas in the present series arose in a patient with a synchronous but histologically distinct cecal carcinoma that also had MSI and did not express Mlh1. The synchronous occurrence of two MSI carcinomas suggests an inherited basis for the development of these carcinomas. Indeed, the medullary phenotype, irrespective of MSI, was highly associated with a family history of cancer in first-degree relatives (P < 0.001). Finally, one medullary carcinoma with lymphoepithelioma-like features contained Epstein-Barr virus-encoded RNA-1 by in situ hybridization. Therefore, because of medullary carcinoma's special genetic, immunohistochemical, and clinical features, recognition of the medullary variant of pancreatic adenocarcinoma is important. Only by classifying medullary carcinoma as special subset of adenocarcinoma can we hope to further elucidate its unique pathogenesis.

Volume

156

Issue

5

First Page

1641

Last Page

1651

ISSN

0002-9440

Published In/Presented At

Wilentz, R. E., Goggins, M., Redston, M., Marcus, V. A., Adsay, N. V., Sohn, T. A., Kadkol, S. S., Yeo, C. J., Choti, M., Zahurak, M., Johnson, K., Tascilar, M., Offerhaus, G. J., Hruban, R. H., & Kern, S. E. (2000). Genetic, immunohistochemical, and clinical features of medullary carcinoma of the pancreas: A newly described and characterized entity. The American journal of pathology, 156(5), 1641–1651. https://doi.org/10.1016/S0002-9440(10)65035-3

Disciplines

Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods

PubMedID

10793075

Department(s)

Administration and Leadership

Document Type

Article