Diagnostic challenge: Intraductal neoplasms of the pancreatobiliary system
ABSTRACT To help pathologists avoid misdiagnosis of intraductal neoplasms arising from the pancreatobiliary system, we report two cases that illustrate diagnostic pitfalls. The first is of a 66-year-old man who complained of appetite loss. An early examination led to a diagnosis of intraductal papillary mucinous neoplasm. Macroscopically, a multilocular cyst without visible mucin was identified. Histologically, the compartments consisted of complex fusion of tubular glands surrounded by dilated pancreatic duct. The neoplasm resembled an acinar cell cystadenocarcinoma. However, the neoplastic cells were negative for trypsin. Thus, the final histopathologic diagnosis was an unusual cystic variant of intraductal tubulopapillary neoplasm (ITPN) of the pancreas. The second case is of a 71-year-old man who complained of right upper quadrant pain. Although bile duct stone was suspected, a polypoid nodule was extracted. Histologically, the nodule was composed of tubular glands, with some complex fusion and focal dysplasia, consistent with carcinoma. In addition, lack of MUC-5AC expression led to an initial impression of ITPN of the bile duct. However, the neoplasm showed dysplastic cells based on the columnar cells resembling pyloric glands, indicating the sequential progression. Thus, the final histopathological diagnosis was intraductal papillary neoplasm of the bile duct with high-grade intraepithelial neoplasia. Because phenotypic variants of intraductal neoplasms of the pancreatobiliary system exist, ITPN and ITPN-mimicking tumor must be carefully differentiated from other intraductal neoplasms.
SourceAvailable from: Huasong Cai[Show abstract] [Hide abstract]
ABSTRACT: Background To describe the Gd-EOB-DTPA-enhanced MRI appearances of cholangiocarcinoma, and evaluate the relative signal intensities(RSIs) changes of major abdominal organs, and investigate the effect of total bilirubin(TB) levels on the RSI.Methods25 patients with pathologically-proven cholangiocarcinoma underwent Gd-EOB-DTPA-enhanced MRI. The visualization of the biliary system during biliary phase (BP) was observed. RSIs of the abdominal aorta(A), portal vein(V), liver(L), and spleen(S) were measured.ResultsOn hepatocellular phase(HP), exophytic tumors(n =10) and infiltrative tumors(n =10) were hypointense, polypoid tumors(n¿=¿2) were hypointense, and combined type tumors(n¿=¿3) had mixed appearances. While patients with normal TB levels (¿22¿mol/L, n¿=¿12) had clear visualization of the biliary tree during BP, those with elevated TB levels (>22 ¿mol/L, n¿=¿13) had obscured or no visualization. In addition, patients with normal TB levels had higher RSIA, RSIV and RSIS than those with elevated TB levels on all dynamic phases(P <0.001), and lower RSIA, RSIV and RSIS on HP and BP(P <0.001). Patients with normal TB levels had higher RSIL than those with elevated TB levels on all phases(P <0.001).ConclusionsRSIs of major abdominal organs reflected underlying biliary function. Cholangiocarcinoma patients with elevated TB levels had delayed excretion of Gd-EOB-DTPA compared with patients with normal TB levels.BMC Cancer 02/2015; 15(1):38. DOI:10.1186/s12885-015-1039-x · 3.32 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Background: Long non-coding RNAs (lncRNAs) have been recently observed in various human cancers. However, the role of lncRNAs in pancreatic duct adenocarcinoma (PDAC) remains unclarified. The aim of this study was to detect the expression of lncRNA MALAT1 in PDAC formalin-fixed, paraffin embedded (FFPE) tissues and to investigate the clinical significance of the MALAT1 level. Methods: The expression of MALAT1 was examined in 45 PDAC and 25 adjacent non-cancerous FFPE tissues, as well as in five PDAC cell lines and a normal pancreatic epithelium cell line HPDE6c-7, using qRT-PCR. The relationship between MALAT1 level and clinicopathological parameters of PDAC was analyzed with the Kaplan-Meier method and Cox proportional hazards model. Results: The relative level of MALAT1 was significantly higher in PDAC compared to the adjacent normal pancreatic tissues (p=0.009). When comparing the MALAT1 level in the cultured cell lines, remarkably higher expression of MALAT1 was found in aspc-1 PDAC cells compared with the immortal pancreatic duct epithelial cell line HPDE6c-7 (q=7.573, p<0.05). Furthermore, MALAT1 expression level showed significant correlation with tumor size (r=0.35, p=0.018), tumor stage (r=0.439, p=0.003) and depth of invasion (r=0.334, p=0.025). Kaplan-Meier analysis revealed that patients with higher MALAT1 expression had a poorer disease free survival (p=0.043). Additionally, multivariate analysis indicated that overexpression of MALAT1, as well as the tumor location and nerve invasion, was an independent predictor of disease-specific survival of PDAC. Conclusion: MALAT1 might be considered as a potential prognostic indicator and may be a target for diagnosis and gene therapy for PDAC.Asian Pacific journal of cancer prevention: APJCP 04/2014; 15(7):2971-7. DOI:10.7314/APJCP.2014.15.7.2971 · 1.50 Impact Factor
[Show abstract] [Hide abstract]
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, beta-catenin and e-cadherin. Sanger sequencing did not detect mutations for beta-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.Diagnostic Pathology 01/2014; 9(1):11. DOI:10.1186/1746-1596-9-11 · 2.41 Impact Factor