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 slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1051828790117127
Highlights
The term “Intraductal tubulopapillary neoplasm (ITPN)” was introduced by Yamaguchi and colleagues in 2009
The neoplasm appeared homogenous (“de novo-like appearance”) with tubulopapillary glands lined by pseudostratified cells showing abundant clear cytoplasm (Figure 2A, B)
A few mucin droplets in the cytoplasm of the clear cells were detectable with histochemistry; Di-PAS and alcian blue (Figure 2C, D)
Summary
The term “Intraductal tubulopapillary neoplasm (ITPN)” was introduced by Yamaguchi and colleagues in 2009. With less than 0.9% of all pancreatic exocrine neoplasms, ITPN is a rarity within pancreatic tumors [1]. ITPN has been included into the WHO classification of 2010 and belongs to the group of intraductal neoplasms of the pancreas [2]. The tumor shows tubulopapillary growth with scanty cytoplasmic mucin, often combined with areas of necrosis [1]. The neoplastic cells, Case presentation A 43-year old female visited the outpatient clinic of the University Medical Center Mannheim with epigastric pain for approximately 5 days prior to presentation. A subsequent MRI scan revealed dilatation of the main pancreatic duct with a minor contrast enhancing intraductal tumor of approximately 3.0 cm. The tumor was partially duct-obstructing and suggestive for the diagnosis of intraductal neoplasms of the pancreas (Figure 1). The postoperative course has been uneventful for over two years
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