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

Diagnostic Pathology

Timo Gaiser⁴  Alexander Marx⁴  Stefan Post³  Jutta Lüttges²  Christian Sauer⁴  Dietmar Dinter¹  Marco Niedergethmann³  Maria Gabriele Ahls⁴ 

[1] Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Mannheim, Germany;Institute of Pathology, Marienhospital of Hamburg, Hamburg, Germany;Department of Surgery, University Medical Center Mannheim, Mannheim, Germany;Institute of Pathology, University Medical Center Mannheim, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

关键词: Pancreatic neoplasm ITPN;    Clear cell morphology;    Intraductal tubulopapillary neoplasm;   

Others  :  803454
DOI  :  10.1186/1746-1596-9-11

 received in 2013-12-27, accepted in 2014-01-10,  发布年份 2014

【 摘 要 】

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

【 授权许可】

   
2014 Ahls et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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