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Open Access Case Report

Case report: intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype

Maria Gabriele Ahls1, Marco Niedergethmann2, Dietmar Dinter3, Christian Sauer1, Jutta Lüttges4, Stefan Post2, Alexander Marx1 and Timo Gaiser1*

Author Affiliations

1 Institute of Pathology, University Medical Center Mannheim, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

2 Department of Surgery, University Medical Center Mannheim, Mannheim, Germany

3 Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Mannheim, Germany

4 Institute of Pathology, Marienhospital of Hamburg, Hamburg, Germany

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Diagnostic Pathology 2014, 9:11  doi:10.1186/1746-1596-9-11

Published: 20 January 2014

Abstract

Intraductal tubulopapillary neoplasms of the pancreas are very rare tumors characterized by intraductal tubulopapillary growth, ductal differentiation, scant intracellular mucin production and cellular dysplasia. Here, we report the first case of an intraductal tubulopapillary neoplasm of the pancreas with clear cell morphology. The tumor was detected during the diagnostic work-up of acute pancreatitis in a 43- year old female. Histological examination revealed a tumor with the typical architecture of an intraductal tubulopapillary neoplasm of the pancreas with tumor cells showing abundant clear cytoplasm and Di-PAS negativity. Immunohistochemistry revealed positivity for Pan-CK, CK7, CK8/18, MUC1, MUC6, carbonic anhydrase IX, CD10, EMA, β-catenin and e-cadherin. Sanger sequencing did not detect mutations for β-catenin, BRAF, KRAS, PIK3CA and GNAS. Altogether, histology, immunohistochemical expression profile (MUC1+, MUC6+, MUC2-, MUC5AC-, thrypsin-, chymotrypsin-, CDX2-) and sequencing results led to the diagnosis of intraductal tubulopapillary neoplasm. However, the neoplasm consisted of cells showing abundant clear cytoplasm, a morphological pattern not being described so far in the current classification of pancreatic intraductal neoplasms. Potential differential diagnosis and the molecular basis of clear cell morphology are discussed. In conclusion, we consider this tumor as intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype. After surgery and without adjuvant therapy, the patient’s clinical course has been uneventful for over two years now.

Virtual slides

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1051828790117127 webcite

Keywords:
Intraductal tubulopapillary neoplasm; Clear cell morphology; Pancreatic neoplasm ITPN