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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with hereditary pancreatitis have a 40% lifetime risk of developing pancreatic ductal adenocarcinoma. Existing methods of diagnosing pancreatic cancer such as tumor markers, endoscopy, and radiological imaging lack the sensitivity and specificity for early diagnosis, particularly in a background of chronic pancreatitis. Molecular based strategies offer new avenues of screening for pancreatic ductal adenocarcinoma in these high-risk patients, which may allow the development of highly sensitive and specific diagnostic tests for the early detection of cancer.
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PMID:Screening for early pancreatic ductal adenocarcinoma in hereditary pancreatitis. 1087 28

Adenocarcinoma of the pancreas is the fifth most common cause of cancer death in the United States. It affects men and women fairly equally and is most frequently diagnosed in the eighth decade of life. It may occur as part of hereditary/familial pancreatitis with an identified genetic mutation, and smokers are at increased risk. Cancer most often occurs in the pancreatic head and often leads to biliary obstruction with a clinical presentation of painless jaundice. The principal diagnostic modality is dedicated pancreatic computed tomography (CT) scanning, although other imaging techniques have a role. Endoscopic retrograde cholangiopancreatography (ERCP) is generally reserved for obtaining tissue, for which it is insensitive, or for palliative stenting. Surgery with the Whipple procedure offers the only chance of cure. Patients are staged as resectable if there are no distant metastases to lymph nodes or organs and there is no major vessel involvement. The 5-year survival rate for resectable patients is about 10% with a median survival of 12 to 18 months. Unresectable patients live about 6 months. Adjuvant chemotherapy with 5-fluorouracil (5-FU) or gemcitabine provides modest benefits. Palliative biliary decompression, pain control, and maintenance of gastric drainage are the usual forms of therapy.
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PMID:New developments in pancreatic cancer. 1095 Apr 64

Recurring complaints of unknown origin in the upper abdomen during childhood are not frequent. Choledochoceles, or type III choledochal cysts, are rare congenital abnormalities of unclear aetiology. They are rarely considered in the differential diagnosis of upper abdominal symptoms in childhood. Only 130 cases of choledochoceles have been reported in world literature to date. Only 20% of these patients were children or adolescents. The symptoms do not specifically suggest a choledochocele, and are usually attributed to other disorders of the upper intestinal tract, frequently resulting in a delayed diagnosis. We present the case of a 10-year old girl in whom a congenital choledochocele was diagnosed by gastro-intestinal studies and endoscopic retrograde cholangiopancreatography (ERCP) following a 2-years history of recurrent pancreatitis. Various types of choledochoceles are presented in the literature, classified according to morphology and histology. Most authors consider them to be a form of choledochal cyst. Their embryonic development is related to that of congenital duodenal diverticula. Although congenital intraluminal duodenal dilatations are of known oncogenic significance, only two cases of adenocarcinoma arising from a choledochocele have been described to date. Established treatment of large choledochoceles in childhood is transduodenal marsupialization.
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PMID:Choledochocele--a rare cause of necrotising pancreatitis in childhood. 1103 17

Acute pancreatitis (AP) has been recognized as a presentation of patients with pancreatic carcinoma (PC). However, the natural history of patients with PC who present with AP as the first manifestation is largely unknown. The aim of this study was to determine the time between the presentation of AP and diagnosis of PC and what factors should alert the clinician to suspect underlying PC in patients with AP. Nineteen physicians completed the survey forms that encompassed 45 patients with a diagnosis of AP preceding a diagnosis of PC. Information included the patient's age, gender, race, conditions that could account for the AP, criteria for diagnosis of AP, severity of AP, criteria for diagnosis of PC, time between the diagnosis of AP and PC, pathology of the carcinoma, extension of the disease, treatment of PC, and survival after the diagnosis of PC. The study population consisted of 45 patients, 27 (60%) men and 18 (40%) women whose average age was 58 years (range, 32-89). Thirty-eight patients were Caucasian, five were black, one was Japanese, and one Arabian. The number of AP episodes before PC diagnosis ranged between one and 15 (mean + 2 SD). AP was mild in 40 (89%) and severe in five (11%). The time between the onset of AP and the diagnosis of PC averaged 34 weeks (range, 1-52). Symptoms on presentation of AP included abdominal pain 45 (100%), weight loss 15 (33%), and jaundice 3 (7%). CA 19-9 was available in 13 patients, eight of whom had levels >100 at the time AP was diagnosed. Abnormal imaging suggestive of PC was detected by ultrasonography in 17 patients, by computed tomography in 30, endoscopic retrograde cholangiopancreatography in 20, and endoscopic ultrasonography in three. Tissue diagnosis was obtained in 43 of 45 (96%) patients; by surgery in 25 patients, needle aspiration in 14, laparoscopy in one, autopsy in two, and lymph node in one. Pathology revealed adenocarcinoma in 37 patients, squamous cell carcinoma in two, undifferentiated carcinoma in two, islet cell in one, and cystadenocarcinoma in one. Surgical findings in 26 patients included 19 with a nonresectable lesion or metastasis and seven patients with resectable lesion for cure. Thirteen patients (28%) were alive 1 year after the diagnosis of PC. The patients had a mean of two (range, one to 15) episodes of AP before the diagnosis of PC, and this was associated with a delay of 34 weeks from AP to diagnosis of PC. Patients with PC who presented with AP were generally older than 50 years of age and the severity of the pancreatitis was mild. The survival rate of patients with PC who presented initially with AP was >25% at 1 year compared with 20% 1 year overall survival of patients with PC. AP seems to be an early presentation of PC and should be sought in patients with idiopathic pancreatitis.
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PMID:Acute pancreatitis secondary to pancreatic carcinoma. Study Group Participants. 1107 85

Hereditary pancreatitis is an uncommon cause of chronic pancreatitis in Western society. It should be suspected when chronic pancreatitis presents in young adults. The diagnosis is made when chronic pancreatitis is present in several members of the same family who are determined not to have other risk factors for chronic pancreatitis. Molecular research focusing on mutations in the trypsinogen gene has uncovered the genetic defects associated with hereditary pancreatitis, and this knowledge has suggested the possible pathophysiologic mechanism of this disease. Because patients with hereditary pancreatitis develop their disease early in life they are very likely to require treatment for complications. As in patients with chronic pancreatitis of other etiologies those with hereditary pancreatitis should be treated medically for acute exacerbations. When complications occur or when the disease causes intractable pain surgery is recommended. Surgical therapy is tailored to the patient's pancreatic anatomy based on endoscopic retrograde cholangiopancreatography or CT scan. The two patients described in this report underwent successful longitudinal pancreaticojejunostomy (Puestow procedure) with good results. Finally it has been shown that patients with hereditary pancreatitis are at increased risk for developing pancreatic adenocarcinoma. Although not widely used pancreatic cancer screening programs have been suggested for surveillance of these patients.
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PMID:Hereditary chronic pancreatitis: implications for surgical treatment and follow-up. 1124 47

Adenoma and adenocarcinoma of the ampulla of Vater are uncommon neoplasms of the gastrointestinal tract. Only one report has analyzed the relationship between ampullary adenocarcinoma and pancreatic intraductal neoplasia (PanIN), the precursor lesion of pancreatic adenocarcinoma. An association between PanIN and ampullary adenoma has not been reported previously. Case reports have documented the progression of PanIN to invasive pancreatic adenocarcinoma. We reviewed five resected ampullary adenoma and 17 ampullary adenocarcinoma cases and evaluated the pancreas for PanIN. Pancreatic sections from 35 autopsies were reviewed as a control group. Immunohistochemistry for overexpression of p53 and COX-2 proteins was performed in selected cases, as was PCR analysis for K-ras mutations. Follow-up clinical data were obtained. All 22 ampullary neoplasms were associated with PanIN, which was high grade in two (40%) adenoma cases and seven (41%) adenocarcinoma cases. In 16 (73%) evaluable cases, PanIN extended to the pancreatic resection margin; two of which had high grade PanIN. Among the autopsy controls eight (23%) had low-grade PanIN. Seven of the 22 ampullary cases but none of the autopsy controls had coexistent pancreatitis. A smoking history was present in two of four autopsy cases in which this history was available. Overexpression of the p53 and COX-2 proteins was present in only one case of high-grade PanIN. K-ras mutations were present in four of four of the PanIN lesions evaluated, including one autopsy case. Clinical follow-up revealed no progression of PanIN to invasive carcinoma in the remnant pancreas, although the follow-up period was too short to adequately assess that risk (an average of 3.8 y for adenoma cases and 2.5 y for adenocarcinoma cases). We conclude that adenomas and carcinomas of the ampulla are associated with PanIN, and often high-grade PanIN. Although its malignant potential has not been fully established, PanIN is underreported and often unrecognized. PanIN may be analogous to colorectal adenoma in that both are prevalent in the older adult population, but few progress to carcinoma.
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PMID:Neoplasms of the ampulla of vater with concurrent pancreatic intraductal neoplasia: a histological and molecular study. 1126 17

Pancreatic cancer is a disease with a very poor prognosis and its etiology is still largely elusive. The only consistent environmental risk factor is cigarette smoking. A previous history of pancreatitis or diabetes mellitus is also considered to be a risk factor. Epidemiological studies have confirmed that relatives of those with pancreatic cancer have an increased risk of this malignancy, and it has been evaluated that 3-5% of all pancreatic cancer cases are caused by genetic predisposition to the disease. Usually this occurs in the setting of a known inherited cancer syndrome caused by mutations in genes such as BRCA1/2 and CDKN2A. Whether or not a true site-specific pancreatic adenocarcinoma syndrome exists is not known. The real challenge for the management of high risk patients is to develop new screening methods than can identify pre-neoplastic or early neoplastic lesions in a timely manner.
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PMID:The role of genetic factors in the etiology of pancreatic adenocarcinoma: an update. 1129 63

A transgenic mouse line carrying ornithine decarboxylase cDNA as the transgene under the control of a mouse mammary tumor virus long terminal repeat (MMTV LTR) promoter was generated in order to study whether ornithine decarboxylase transgene expression will have any physiological or pathological effect during the entire life of a transgenic mouse. The high frequency of infertile animals and the loss of pups made the breeding of homozygous mice unsuccessful. However, a colony of heterozygous transgenic mice was followed for 2 years. In adult heterozygous transgenic mice, ornithine decarboxylase activity was significantly increased in the testis, seminal vesicle and preputial gland when compared to non-transgenic controls. In contrast, ornithine decarboxylase activity was decreased in the kidney and prostate of transgenic mice. No significant changes in ornithine decarboxylase activity were found in the ovary and mammary gland and only moderate changes in ornithine decarboxylase activity were detected in the heart, brain, pancreas and lung. The most common abnormalities found in adult animals (12 males and 20 females) of the transgenic line were inflammatory processes, including pancreatitis, hepatitis, sialoadenitis and pyelonephritis. Spontaneous tumors were observed in eight animals, including two benign tumors (one dermatofibroma, one liver hemangioma) and six malignant tumors (one lymphoma, one intestinal and three mammary adenocarcinomas and one adenocarcinoma in the lung). No significant pathological changes were found in 17 nontransgenic controls.
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PMID:Abnormal ornithine decarboxylase activity in transgenic mice increases tumor formation and infertility. 1133 Dec 6

This article presents current MR imaging techniques for the pancreas, and review a spectrum of MR imaging features of various pancreatic diseases. These include: 1) congenital anomalies such as anomalous union of pancreatobiliary ducts, divisum, and annular pancreas, 2) inflammatory diseases, including acute or chronic pancreatitis with complications, groove pancreatitis, and autoimmune pancreatitis, tumor-forming pancreatitis, 3) pancreatic neoplasms, including adenocarcinoma, islet cell tumors, and cystic neoplasms (microcystic adenoma, mucinous cystic neoplasms, and intraductal mucin-producing pancreatic tumor). Particular attention is paid to technical advances in MR imaging of the pancreas such as fat-suppression, MR pancreatography (single- or multi-slice HASTE), and thin-section 3D multiphasic contrast-enhanced dynamic sequences. Imaging characteristics that may lead to a specific diagnosis or narrow the differential diagnosis are also discussed.
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PMID:MR imaging of pancreatic diseases. 1133 90

Pancreatic intraepithelial neoplasia (PanIN) is thought to be a precursor lesion of infiltrating pancreatic ductal adenocarcinoma (IPA). DPC4 is a tumor-suppressor gene on chromosome 18q21.1 and is inactivated in approximately 55% of IPAs. Recently, immunohistochemical labeling using a monoclonal antibody to the Dpc4 protein has been shown to mirror DPC4 genetic status in invasive adenocarcinomas of the pancreas. In the present study, we examined the role of Dpc4 loss in neoplastic progression and recurrence. Two cases in which a PanIN clinically progressed to an invasive adenocarcinoma and a third of a patient with IPA of the head of the pancreas who later developed invasive adenocarcinoma in the tail of the pancreas were studied using Dpc4 immunolabeling. The first patient underwent pancreatic resection, which revealed PanIN-3 that lacked Dpc4 expression, and the patient developed an invasive pancreatic ductal carcinoma 10 years later that shared this loss of expression. The second patient had a pancreaticoduodenectomy for recurrent pancreatitis, and the resected pancreas contained PanIN-3 with intact Dpc4 expression. Seventeen months later, the patient developed an invasive adenocarcinoma of the distal pancreas that also had intact Dpc4 expression. In the third case, the patient underwent pancreaticoduodenectomy for an invasive ductal adenocarcinoma with negative margins. This carcinoma lacked Dpc4 expression. Three years later, resection of the pancreatic tail showed a second invasive adenocarcinoma. The cancer in the tail of the gland showed intact Dpc4 expression, suggesting it represented a second primary tumor, not a recurrence. We conclude that Dpc4 expression in PanIN can be predictive of Dpc4 expression in the subsequent invasive ductal adenocarcinoma. Additionally, Dpc4 expression can be used to differentiate recurrent or persistent adenocarcinoma from a second primary adenocarcinoma.
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PMID:Pancreatic intraepithelial neoplasia and infiltrating adenocarcinoma: analysis of progression and recurrence by DPC4 immunohistochemical labeling. 1143 19


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