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review of literature on clinical pancreatology - The Pancreapedia

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adenocarcinoma must be addressed at follow-up evaluati<strong>on</strong>. A follow-up managementalgorithm also was also proposed in the <str<strong>on</strong>g>review</str<strong>on</strong>g> [578]Risk <str<strong>on</strong>g>of</str<strong>on</strong>g> cancerAlthough branch duct intraductal papillary mucinous neoplasms (BD-IPMNs) are slowgrowingtumors with a favorable prognosis, the synchr<strong>on</strong>ous occurrence <str<strong>on</strong>g>of</str<strong>on</strong>g> pancreatic ductaladenocarcinomas (PDAs) in patients with BD-IPMNs has been reported. One study wasaimed to elucidate the development <str<strong>on</strong>g>of</str<strong>on</strong>g> PDAs in l<strong>on</strong>g-term follow-up patients with BD-IPMNs.It was investigated 89 BD-IPMN patients who had no mural nodules and followed them upc<strong>on</strong>servatively at least 2 years (median follow-up, 64 m<strong>on</strong>ths; range, 25-158 m<strong>on</strong>ths). Allsubjects underwent examinati<strong>on</strong>s by imaging modalities including endoscopic retrogradepancreatography. It was calculated the standardized incidence ratio (SIR) from vital statistics.Am<strong>on</strong>g the 89 patients, 4 cases <str<strong>on</strong>g>of</str<strong>on</strong>g> PDAs distant from BD-IPMN were observed in 552patient-years <str<strong>on</strong>g>of</str<strong>on</strong>g> follow-up (7.2 per 1000 patient-years). <strong>The</strong> expected number was 0.25, andthe SIR <str<strong>on</strong>g>of</str<strong>on</strong>g> PDAs was 15.8 (95 % c<strong>on</strong>fidence interval 4.3 to 40.4). Subgroup analysesshowed that the incidence <str<strong>on</strong>g>of</str<strong>on</strong>g> PDAs was significantly increased in patients 70 years or older(SIR 16.7; 95 % c<strong>on</strong>fidence interval 3.4 to 48.7) and in women (SIR 22.5; 95 % c<strong>on</strong>fidenceinterval 2.7 to 81.1). It was c<strong>on</strong>cluded that patients with BD-IPMNs are at a high risk forpancreatic adenocarcinomas. During the follow-up, careful examinati<strong>on</strong> is required to detectthe development <str<strong>on</strong>g>of</str<strong>on</strong>g> PDAs in patients with BD-IPMNs [579].<strong>The</strong> biologic and <strong>clinical</strong> behavior <str<strong>on</strong>g>of</str<strong>on</strong>g> intraductal papillary mucinous neoplasms <str<strong>on</strong>g>of</str<strong>on</strong>g> thepancreas (IPMNs) and IPMN-associated adenocarcinomas is different from ductal pancreaticcancer in having a less aggressive tumor growth and significantly improved survival. Up todate, the molecular mechanisms underlying the <strong>clinical</strong> behavior <str<strong>on</strong>g>of</str<strong>on</strong>g> IPMNs are incompletelyunderstood. Now 128 cystic pancreatic lesi<strong>on</strong>s were prospectively identified during thecourse <str<strong>on</strong>g>of</str<strong>on</strong>g> 2 years. From the corresp<strong>on</strong>ding surgical specimens, 57 IPMNs were separatedand subdivided by histologic criteria into those with low-grade dysplasia, moderate dysplasia,high-grade dysplasia, and invasive cancer. Twenty specimens were suitable for DNAisolati<strong>on</strong> and subsequent performance <str<strong>on</strong>g>of</str<strong>on</strong>g> array CGH. While n<strong>on</strong>e <str<strong>on</strong>g>of</str<strong>on</strong>g> the IPMNs with lowgradedysplasia displayed detectable chromosomal aberrati<strong>on</strong>s, IPMNs with moderate andhigh-grade dysplasia showed frequent copy number alterati<strong>on</strong>s. Comm<strong>on</strong>ly lost regi<strong>on</strong>s werelocated <strong>on</strong> chromosome 5q, 6q, 10q, 11q, 13q, 18q, and 22q. <strong>The</strong> incidence <str<strong>on</strong>g>of</str<strong>on</strong>g> loss <str<strong>on</strong>g>of</str<strong>on</strong>g>chromosome 5q, 6q, and 11q was significantly higher in IPMNs with high-grade dysplasia orinvasi<strong>on</strong> compared with PDAC. Ten <str<strong>on</strong>g>of</str<strong>on</strong>g> 13 IPMNs with moderate dysplasia or malignancy hadloss <str<strong>on</strong>g>of</str<strong>on</strong>g> part or all <str<strong>on</strong>g>of</str<strong>on</strong>g> chromosome 6q, with a minimal deleted regi<strong>on</strong> between linear positi<strong>on</strong>s78.0 and 130.0. This study is the first to use array CGH to characterize IPMNs. Recurrentcytogenetic alterati<strong>on</strong>s were identified and were different than those described in PDAC.Array CGH may help distinguish between these 2 entities and give insight into thedifferences in their biology and prognosis [580].A 52-year-old man with a history <str<strong>on</strong>g>of</str<strong>on</strong>g> distal gastrectomy for gastric cancer was admittedbecause <str<strong>on</strong>g>of</str<strong>on</strong>g> jaundice. CT scan revealed double tumors in the pancreatic head and bodyc<strong>on</strong>comitant with multicystic lesi<strong>on</strong>s <str<strong>on</strong>g>of</str<strong>on</strong>g> the pancreas. Total pancreatectomy with splenectomyand remnant gastrectomy was performed. Final histological diagnosis was double invasiveductal carcinomas <str<strong>on</strong>g>of</str<strong>on</strong>g> the pancreas head and tail with multifocal branch duct intraductalpapillary mucinous adenomas <str<strong>on</strong>g>of</str<strong>on</strong>g> the pancreas. <strong>The</strong> present case suggests that entirepancreas might have malignant potential in patients with intraductal papillary mucinousneoplasms [581].A c<strong>on</strong>sensus c<strong>on</strong>ference has recommended close observati<strong>on</strong> <str<strong>on</strong>g>of</str<strong>on</strong>g> branch duct intraductalpapillary mucinous neoplasms (BD-IPMNs) smaller than 30 mm, without symptoms or muralnodules. One study investigated whether these recommendati<strong>on</strong>s could be validated in a

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