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All of the following inherited familial syndromes are associated with an increased incidence of p
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ancreatic endocrine neoplasms except:
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A. von Hippel-Lindau (VHL) syndrome.
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B. tuberous sclerosis (TSC).
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C. multiple endocrine neoplasia type 1 (MEN-1).
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D. Peutz-Jeghers syndrome (PJS).
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E. neurofibromatosis (NF-1). - ANSWER Peutz-Jeghers syndrome (PJS).
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Correct.
The majority of pancreatic endocrine neoplasms are sporadic. Some of them, however, occur as
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part of inherited familial syndromes such as MEN-1, VHL syndrome, NF-1, and TSC. MEN-
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1 is associated with gastrinoma, while NF-
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1 is associated with somatostatinomas. VHL and TSC are both associated with increased pancrea
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tic endocrine neoplasms. PJS is characterized by the development of benign hamartomatous pol
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yps in the gastrointestinal tract. It is associated with an increased risk of developing carcinomas
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of the pancreas, liver, lungs, breast, ovaries, uterus, testicles, and other organs.
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Which of the following statements is true regarding pseudocysts in chronic pancreatitis?
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A. Pseudocysts, although common following an episode of acute pancreatitis, rarely occur in pat
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ients with chronic pancreatitis.
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B. Recurrence of a pancreatic pseudocyst after aspiration implies an ongoing communication wit
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h the pancreatic duct.
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C. In patients with chronic pancreatitis and a pseudocyst, adequate drainage of the pseudocyst
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will usually result in significant pain relief and decreased narcotic requirements.
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D. Percutaneous drainage successfully treats pancreatic pseudocysts in nearly all cases. -
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OANSWER Recurrence of a pancreatic pseudocyst after aspiration implies an ongoing communica
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tion with the pancreatic duct.
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,Correct.
Pancreatic pseudocysts are a common complication of chronic pancreatitis. The underlying duct
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al abnormalities in patients with chronic pancreatitis make pseudocysts less likely to regress spo
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ntaneously in these patients. Pseudocysts can be an important additional cause of pain in patien
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ts with chronic pancreatitis, but treatment of the pseudocyst rarely leads to complete pain relief
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. Ideally, the pancreatic ductal anatomy should be defined prior to any attempt at treatment. A p
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seudocyst that recurs after percutaneous drainage has by definition an ongoing communication
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with the pancreatic ductal system and is best treated by operative internal drainage. This proced
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ure can be combined with a ductal drainage procedure if indicated to address the patient's chro
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nic pancreatitis.
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A 35-year-
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old woman is evaluated for a seizure disorder, mental obtundation, and personality change. The
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findings on physical examination are normal. The fasting serum glucose is 44 mg/dL. Other seru
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m values are normal. An insulin-to-
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glucose (I:G) ratio of 0.5 was documented after 28 hours of fasting. Symptoms of mental obtund
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ation developed concurrently and were reversed by means of oral administration of glucose. En
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doscopic ultrasonography showed a 1.2- O O O O
cm mass in the head of the pancreas. Appropriate management consists of which of the followi
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ng?
A. Long-term administration of octreotide
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B. Surgical enucleation of the tumor
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C. Pancreaticoduodenectomy
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D. Total pancreatectomy - ANSWER Surgical enucleation of the tumor
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Correct.
Management of an insulinoma is surgical in nearly all cases. Insulinomas are evenly distributed i
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n the pancreas; approximately one third are located in the head and uncinate process, one third
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in the body of the gland, and one third in the tail of the gland. Ninety percent of patients have a
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benign solitary adenoma amenable to surgical cure. Small benign insulinomas not close to the p
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ancreatic duct can be removed by means of enucleation, independent of location within the gla
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nd. In the body and tail of the pancreas, insulinomas larger than 2 cm in diameter and those clos
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e to the pancreatic duct are most commonly excised by means of a distal pancreatectomy. Large
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, insulinomas deep in the head of pancreas or uncinate process of the pancreas may not be amen
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able to local excision and may necessitate pancreaticoduodenectomy.
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Which of the following genetic alterations is found in high frequency (50% or greater) in patients
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with pancreatic cancer?
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A. O
Disorders of DNA mismatch-repair genes O O O O
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B. O
Absence of the tumor-suppressor gene DPC4
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O
C. O
Point mutation of the oncogene p53
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O
D. O
Activation of the oncogene p16 - ANSWER Absence of the tumor-suppressor gene DPC4
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Correct.
The genes involved in the pathogenesis of pancreatic cancer can be divided into three categorie
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s: (a) tumor-suppressor genes, (b) oncogenes, and (c) DNA mismatch-repair genes. Tumor-
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suppressor genes normally function to control cellular proliferation. When these genes are inact
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ivated by genomic events such as mutation, deletion, chromosome rearrangements, or mitotic r
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ecombination, their function as growth suppressors can be lost, and abnormal growth regulatio
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n is the result. The function of p53 appears to be inactivated in up to 75% of all pancreatic cance
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rs. The p53 gene product is the DNA-
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binding protein that acts as a cell cycle checkpoint and an inducer of apoptosis. The DPC4 is a tu
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mor-
suppressor gene that has been identified on chromosome 18q. The chromosome has been show
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n to be missing in approximately 90% of pancreatic cancers. The DPC4 gene is inactive in approxi
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mately 50% of pancreatic carcinomas. DPC4 mutations are more specific than p53 or p16 mutati
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ons for pancreatic cancers. The p16 gene encodes a protein that binds cyclin to cyclin D-
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Cdk4 complexes. When the p16 gene product binds to these complexes, it inhibits the phosphor
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