Digestive and Liver Disease 47 (2015) 256–259
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Correspondence Pancreatic neuroendocrine tumour simulating an intraductal papillary mucinous neoplasm Dear Editor, The authors present the case of a 50 year-old woman with no relevant medical history that was sent to our hospital presenting with periumbilical abdominal pain and nausea lasting for several weeks and an abdominal ultrasound that identiﬁed an 18 mm cystic lesion with regular wall localized in the body–tail transition of the pancreas. Biochemical analysis, including tumour markers (carcinoembryonic antigen, CEA and cancer antigen 19.9), showed no changes. For cystic evaluation, it was decided to perform a magnetic resonance imaging (MRI) and endoscopic ultrasonography. MRI (Fig. 1) reported a cystic lesion in apparent communication with the Wirsung duct, without changing its calibre, probably representing a side-branch intraductal papillary mucinous neoplasm (IPMN). Endoscopic ultrasonography revealed similar morphological characteristics, and the cyst content was aspirated to be analyzed. Biochemical analysis of the sample showed no increased tumour markers levels (CEA 1 ng/mL), however the cytological analysis revealed the presence of cells with adenocarcinoma features. Thus, it was proposed to perform the surgical resection of the lesion with the patient agreement. When the surgical piece was analyzed, it was found that the lesion expressed features consistent with a well-differentiated neuroendocrine tumour pT1 that was conﬁrmed by immunohistochemistry (positive to cromogranin and synaptophysin). Pancreatic neuroendocrine tumours (PNETs) are rare neoplasms, with an annual incidence of 1:100,000–1:1,000,000 population , representing 1–2% of all pancreatic neoplasms, with less than 100 cases reported to this date. Most cases occur in middle age individuals, with a slight female preponderance . The cystic degeneration of these tumours is a much less frequent phenomenon, occurring in 2–10% of all cases. The PNETs are usually benign and nonfunctioning, unless they present in the context of
paraneoplastic syndromes. However, there is a low but real probability of progression to malignancy. The preoperative diagnosis of these tumours is usually difﬁcult to do and may be impossible to differentiate them from other cystic neoplasms, in particular due to the lack of speciﬁc imaging signals and serum markers . More recently, studies with positron emission tomography scans revealed that they may be useful in some situations, although these data require more evidence . Management options for these types of lesions are evolving, but radical surgery remains as the ﬁrst-line treatment whenever possible. In our case, the imaging characteristics of the lesion suggested that an IPMN was the most likely diagnosis. However, cytological analysis revealed the presence of cells with malignant characteristics, and the histological examination following surgical resection conﬁrmed its neuroendocrine nature. In conclusion, although rare, PNETs should be included in the differential diagnosis of pancreatic cystic lesions, mainly due to its malignant potential. In this context, the cytological and biochemical evaluation of the cystic content is critical to the exclusion of such tumours. Conﬂict of interest None declared. References  Deshmukh SD, Gulati HK, Gaopande V, et al. Incidental cystic endocrine tumor of the pancreas: A case report with immunohistochemical study. J Cancer Res Ther 2012;8:289–91.  Ballarin R, Masetti M, Losi L, et al. Cystic pancreatic neuroendocrine neoplasms with uncertain malignant potential: report of two cases. Surg Today 2009;39:162–7.  Fisher WE, Hodges SE, Yagnik V, et al. Accuracy of CT in predicting malignant potential of cystic pancreatic neoplasms. HPB 2008;10:483–90.  Brugge WR, Lauwers GY, Sahani D, et al. Cystic neoplasms of the pancreas. N Engl J Med 2004;351:1218–26.
Armando Peixoto ∗ Pedro Pereira Susana Lopes Guilherme Macedo Gastroenterology Department, Centro Hospitalar de São João, Porto, Portugal ∗ Corresponding
author at: Gastroenterology Department, Centro Hospitalar de São João, Alameda Prof. Hernâni Monteiro, 4200–319 Porto, Portugal. Tel.: +351 225 512 100; fax: +351 225 025 766. E-mail address: [email protected]
(A. Peixoto) Fig. 1. Cystic lesion in apparent communication with the Wirsung duct compatible with a side-branch intraductal papillary mucinous neoplasm.
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