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Genomic Landscapes of Pancreatic Neoplasia
Laura D. Wood,Ralph H. Hruban 대한병리학회 2015 Journal of Pathology and Translational Medicine Vol.49 No.1
Pancreatic cancer is a deadly disease with a dismal prognosis. However, recent advances in sequencing and bioinformatic technology have led to the systematic characterization of the genomes of all major tumor types in the pancreas. This characterization has revealed the unique genomic landscape of each tumor type. This knowledge will pave the way for improved diagnostic and therapeutic approaches to pancreatic tumors that take advantage of the genetic alterations in these neoplasms.
Acinar Cell Carcinoma of the Pancreas: Clinical and Cytomorphologic Characteristics
Adam D. Toll,Ralph H. Hruban,Syed Z. Ali 대한병리학회 2013 Journal of Pathology and Translational Medicine Vol.47 No.2
Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.
Kim, Joo Young,Brosnan-Cashman, Jacqueline A.,An, Soyeon,Kim, Sung Joo,Song, Ki-Byung,Kim, Min-Sun,Kim, Mi-Ju,Hwang, Dae Wook,Meeker, Alan K.,Yu, Eunsil,Kim, Song Cheol,Hruban, Ralph H.,Heaphy, Christ American Association for Cancer Research 2017 Clinical Cancer Research Vol.23 No.6
<P><B>Purpose:</B> Alternative lengthening of telomeres (ALT), a telomerase-independent telomere maintenance mechanism, is strongly associated with ATRX and DAXX alterations and occurs frequently in pancreatic neuroendocrine tumors (PanNET).</P><P><B>Experimental Design:</B> In a Korean cohort of 269 surgically resected primary PanNETs and 19 sporadic microadenomas, ALT status and nuclear ATRX and DAXX protein expression were assessed and compared with clinicopathologic factors.</P><P><B>Results:</B> In PanNETs, ALT or loss of ATRX/DAXX nuclear expression was observed in 20.8% and 19.3%, respectively, whereas microadenomas were not altered. ALT-positive PanNETs displayed a significantly higher grade, size, and pT classification (all, <I>P</I> < 0.001). ALT also strongly correlated with lymphovascular (<I>P</I> < 0.001) and perineural invasion (<I>P</I> = 0.001) and the presence of lymph node (<I>P</I> < 0.001) and distant metastases (<I>P</I> = 0.002). Furthermore, patients with ALT-positive primary PanNETs had a shorter recurrence-free survival [HR = 3.38; 95% confidence interval (CI), 1.83–6.27; <I>P</I> < 0.001]. Interestingly, when limiting to patients with distant metastases, those with ALT-positive primary tumors had significantly better overall survival (HR = 0.23; 95% CI, 0.08–0.68; <I>P</I> = 0.008). Similarly, tumors with loss of ATRX/DAXX expression were significantly associated with ALT (<I>P</I> < 0.001), aggressive clinical behavior, and reduced recurrence-free survival (<I>P</I> < 0.001). However, similar to ALT, when limiting to patients with distant metastases, loss of ATRX/DAXX expression was associated with better overall survival (<I>P</I> = 0.003).</P><P><B>Conclusions:</B> Both primary ALT-positive and ATRX/DAXX-negative PanNETs are independently associated with aggressive clinicopathologic behavior and displayed reduced recurrence-free survival. In contrast, ALT activation and loss of ATRX/DAXX are both associated with better overall survival in patients with metastases. Therefore, these biomarkers may be used as prognostic markers depending on the context of the disease. <I>Clin Cancer Res; 23(6); 1598–606. ©2016 AACR</I>.</P>