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Multiple endocrine neoplasm, type 1. Gastrinomas, pancreatic neoplasms, microcarcinoids, the Zollinger-Ellison syndrome, lymph nodes, and hepatic metastases.

OBJECTIVE: We reviewed the age of presentation, malignant potential, and outcome of gastrinomas and pancreatic tumors in patients with multiple endocrine neoplasm, type 1. DESIGN: Screening of one very large and one smaller, possibly related family on an island, including serum gastrin estimations and, when indicated, pancreatic ultrasound. SETTING AND PATIENTS: Over 2000 family members and their family physicians were advised on screening procedures. INTERVENTION: Data were collected and reviewed retrospectively and prospectively for all medical records, investigations, surgical procedures, and available tissue samples. OUTCOME MEASUREMENTS: Criteria for diagnosis were established for radiological, biochemical, and histological studies. RESULTS: Sixty-two patients had evidence of gastrinoma or pancreatic neoplasm. In 19 patients the diagnosis was based on demonstration of a tumor. In 21 patients the diagnosis was based on elevated serum gastrin concentration in the absence of demonstrable tumor. None of these patients required gastric surgery if they first underwent parathyroidectomy. In 18 patients the diagnosis was based on the combination of demonstrated pancreatic tumor plus elevated glucagon (two patients), gastrin (11 patients), or insulin (five patients) concentration. Peptic ulcer was difficult to control in seven of the 11 patients with elevated gastrin concentrations plus demonstrated tumor. Four patients had liver metastases that appeared to be secondary to the pancreatic gastrinoma. In patients with insulinomas, the first symptoms occurred before age 20 years. Elevated serum gastrin concentrations were not seen before age 24 years and were observed to occur for the first time in two patients after age 50 years.

Adolescent↗

Difficulty with diagnosis of malignant pancreatic neoplasms coexisting with chronic pancreatitis.

Chronic pancreatitis is a relatively common disease. We encountered two different cases of belatedly demonstrated pancreatic carcinoma featuring underlying chronic pancreatitis. The first case was one that was highly suspected as that of a malignancy based upon imaging study, but unfortunately, it could not be confirmed by intra-operative cytology at that time. Following this, the surgeon elected to perform only conservative bypass surgery for obstructive biliary complication. Peritoneal carcinomatosis was later noted and the patient finally died. The second case, a malignant mucinous neoplasm, was falsely diagnosed as a pseudocyst, based upon the lesion's sonographic appearance and associated elevated serum amylase levels. After suffering repeated hemoptysis, the patient was found to exhibit lung metastasis and peritoneal seeding. We reviewed some of the literature, including those studies discussing chronic pancreatitis predisposing to a malignant change. These two case analyses illustrate clearly that the diagnosis for such conditions, which is simply based upon imagery or pathological considerations may end up being one of a mistaken malignancy. Some of our suggestions for the treatment of such malignancies as revealed herein include, total pancreatomy for univocal mass lesion, and needle aspiration of lesion-contained tissue for amylase, CA199 and CEA levels for a suspicious cystic pancreatic mass.

Chronic Disease↗

Outcomes analysis of laparoscopic resection of pancreatic neoplasms.

BACKGROUND: Experience with laparoscopic resection of pancreatic neoplasms remains limited. The purpose of this study is to critically analyze the indications for and outcomes after laparoscopic resection of pancreatic neoplasms. METHODS: The medical records of all patients undergoing laparoscopic resection of pancreatic neoplasms from July 2000 to February 2006 were reviewed. Data are expressed as mean +/- standard deviation. RESULTS: Laparoscopic pancreatic resection was performed in 22 patients (M:F, 8:14) with a mean age of 56.3 +/- 15.1 years and mean body mass index (BMI) of 26.3 +/- 4.5 kg/m2. Nine patients had undergone previous intra-abdominal surgery. Indications for pancreatic resection were cyst (1), glucagonoma (1), gastrinoma (2), insulinoma (3), metastatic tumor (2), IPMT (4), nonfunctioning neuroendocrine tumor (3), and mucinous/serous cystadenoma (6). Mean tumor size was 2.4 +/- 1.6 cm. Laparoscopic distal pancreatectomy was attempted in 18 patients and completed in 17, and enucleation was performed in 4 patients. Laparoscopic ultrasound (n = 10) and a hand-assisted technique (n = 4) were utilized selectively. Mean operative time was 236 +/- 60 min and mean blood loss was 244 +/- 516 ml. There was one conversion to an open procedure because of bleeding from the splenic vein. The mean postoperative LOS was 4.5 +/- 2.0 days. Seven patients experienced a total of ten postoperative complications, including a urinary tract infection (UTI) (1), lower-extremity deep venous thrombosis (DVT) and pulmonary embolus (1), infected peripancreatic fluid collection (1), pancreatic pseudocyst (1), and pancreatic fistula (6). Five pancreatic fistulas were managed by percutaneous drainage. The reoperation rate was 4.5% and the overall pancreatic-related complication rate was 36.4%. One patient developed pancreatitis and a pseudocyst 5 months postoperatively, which was managed successfully with a pancreatic duct stent. There was no 30-day mortality. CONCLUSIONS: Laparoscopic pancreatic resection is safe and feasible in selected patients with pancreatic neoplasms. With a pancreatic duct leak rate of 27%, this problem remains an area of development for the minimally invasive technique.

Adult↗

Cystic pancreatic neoplasms: 12-year surgical experience.

Cystic pancreatic neoplasms have been increasingly diagnosed in the last years. Resection is recommended in most cases, but their management has not been standardized since an accurate nonoperative differentiation is often difficult. A retrospective review of 30 patients undergoing surgical resection for cystic pancreatic neoplasms between 1993 and 2005 was performed. Median age of the patients was 63 years and 63.5% were female. Twelve patients (40%) were asymptomatic. Twenty-nine had curative resections. Pathologic analysis revealed 13 serous cystadenomas, 9 mucinous cystadenomas, 3 mucinous cystadenocarcinomas, 4 intraductal papillary mucinous neoplasms and 1 solid pseudopapillary neoplasm. Overall mortality was 6.5% (2 patients). Postoperative complications occurred in 12 patients (41%). Pancreatic fistula occurred in 7 cases (24%). Reoperation was required in 2 patients (6.5%). Two patients operated for mucinous cystadenocarcinoma and invasive intraductal papillary mucinous neoplasms died of recurrence at 24 and 7 months postoperatively. Excluding another patient died from other cause, all others are currently alive with no evidence of disease. Diagnostic accuracy for cystic pancreatic neoplasms is still limited. Considering the good prognosis and acceptable morbidity and minimal mortality after surgical treatment in specialized centers, resection seems still justified in most cases.

Adult↗

Pancreatic neoplasms: MR imaging and pathologic correlation.

Magnetic resonance (MR) imaging can aid in the detection and characterization of many pancreatic neoplasms. The MR imaging appearances of common pancreatic neoplasms such as ductal adenocarcinoma are well-known. However, MR imaging features of more unusual pancreatic neoplasms are not well understood. Such tumors include mucin-hyper-secreting carcinoma, serous microcystic neoplasm, mucinous macrocystic neoplasm, solid and papillary epithelial neoplasm, multiple cysts associated with von Hippel-Lindau disease, acinar cell carcinoma, pancreaticoblastoma, and endocrine neoplasms (eg, nonfunctioning islet cell tumors, insulinoma, and gastrinoma). In general, pancreatic neoplasms demonstrate high signal intensity on T2-weighted images; the signal intensity on T1-weighted images is more variable but is often intermediate or low. Gadolinium enhancement is often helpful in further characterizing pancreatic neoplasms. The gross and histologic features of pancreatic neoplasms are also not well-known. Correlation with the underlying pathologic features enhances understanding of the MR imaging characteristics of both common and unusual pancreatic neoplasms.

Adenocarcinoma, Mucinous↗

Thin-section contrast-enhanced computed tomography accurately predicts the resectability of malignant pancreatic neoplasms.

A prospective diagnostic study was designed to determine the ability of thin-section contrast-enhanced computed tomography (CT) to predict the resectability of malignant neoplasms of the pancreatic head. Patients with a presumed resectable pancreatic neoplasm referred during a 21-month period were studied with abdominal CT performed at 1.5-mm section thickness and 5-mm slice interval during the bolus phase of intravenous contrast enhancement. CT criteria for resectability included the absence of extrapancreatic disease, no evidence of arterial encasement, and a patent superior mesenteric-portal venous confluence. Of 145 patients evaluated, 42 were considered to have resectable tumors by CT criteria, and 37 (88%) underwent potentially curative pancreaticoduodenectomy. Six patients were found to have a microscopically positive retroperitoneal resection margin; no patient had a grossly positive resection margin. Five (12%) of 42 patients were found at laparotomy to have unresectable, locally advanced or metastatic tumors. Thin-section contrast-enhanced CT is an essential component of the preoperative evaluation for pancreaticoduodenectomy and can prevent needles laparotomy in most patients with locally advanced or metastatic disease.

Adenocarcinoma↗

Large cystic pancreatic neoplasms: pathology, resectability, and outcome.

BACKGROUND: Cystic pancreatic neoplasms may be benign, premalignant, or malignant. These lesions may remain asymptomatic for long periods and can be quite large at the time of presentation. METHODS: A retrospective analysis was used to determine whether preoperative evaluation can predict pathology and determine resectability and outcome. RESULTS: Over 12 years, 145 cystic pancreatic neoplasms, of which 24 (17%) were larger than 10 cm, were managed at the Johns Hopkins Hospital. Those 24 large tumors included 9 of 73 cystadenomas (12%), 7 of 27 cystadenocarcinomas (26%), 2 of 35 adenocarcinomas producing mucin or associated with a cyst (6%), 5 of 9 Hamoudi tumors (55%), and 1 dermoid cyst. Clinical symptoms, liver function tests, and computed tomographic scans did not distinguish benign from malignant pathology. On 18 angiograms, 2 malignant and 4 benign neoplasms demonstrated encasement or occlusion; however, 3 of these 6 tumors were resectable. Twenty of 22 patients (91%) who were explored underwent resection with no hospital mortality. For the entire series, 5-year survival for those with cystadenomas, cystadenocarcinomas, and cystic adenocarcinomas was 97%, 38%, and 9%, respectively. Three-year survival for those 7 with cystadenocarcinomas larger than 10 cm was 54%, compared with 51% for those 20 with smaller cystadenocarcinomas. CONCLUSIONS: Preoperative evaluation usually does not predict pathology, resectability, or outcome. Moreover, resectability is high and morbidity is low, irrespective of size. Large cystic pancreatic tumors should be explored to determine pathology, attempt resection, and provide an opportunity for long-term survival.

Adult↗

Cystic pancreatic neoplasms: enucleate or resect?

Asymptomatic cystic pancreatic neoplasms are being detected by abdominal imaging with increasing frequency. Enucleation of small cystic neoplasms can be performed without recurrence but has been associated with a higher incidence of pancreatic fistula. Thus the procedure has been modified to include intraoperative ultrasound imaging and closure of the pancreatic defect. This analysis was performed to determine whether these modifications have improved operative outcome. Thirty patients with mucinous cystic neoplasms (n=16), serous cystadenomas (n=10), and cystic islet cell tumors (n=4) were studied. Enucleation was performed in 11 patients (7 with mucinous cystic neoplasms, 2 with serous cystadenomas and 2 with islet cell tumors), whereas 19 underwent resection of cystic tumors (pancreatoduodenectomy in 8 and distal pancreatectomy in 11). The mean groups did not differ with regard to age (57 years), gender (73% female), presentation (63% incidental), or site (43% head, neck, or uncinate). Patients undergoing enucleation had smaller tumors (2.2 vs. 4.7 cm, P<0.01) that were less likely to be in the tail (9% vs. 42%). Operative time was significantly shorter in the enucleation group (199 vs. 298 minutes, P<0.01). Blood loss also was significantly reduced in the enucleation group (114 vs. 450 ml, P<0.001). Pancreatic fistula rates (27% vs. 26%) and length of hospital stay (12.6 vs. 15.7 days) were similar in the two groups. Enucleation of benign cystic pancreatic neoplasms reduces operative time and blood loss without increasing postoperative complications or length of stay. Therefore enucleation should be the standard operation for small benign cystic neoplasms in the uncinate, head, neck, and body of the pancreas.

Adenoma, Islet Cell↗

Surgical concepts for therapy of pancreatic neoplasms.

Surgical treatment of pancreatic malignomas has improved dramatically over the past decades, which is illustrated by the decrease in perioperative mortality to 1%-2%. This has made it possible to widen the indication for pancreatic resection and the extent of the surgical intervention. Experience with pancreatic resection for neoplasms at our center using standard surgical techniques during the last decade is reviewed, with special focus on the cases with partial resection of the portal vein. Perioperative morbidity has decreased below 5% and 5-year survival rates reach 60% for stage I tumors and 15%-25% for stage II and III tumors. The authors conclude that surgical therapy for pancreatic neoplasms is safe and yields a considerably higher quality of life for the patient and therefore should be considered in all patients with this disease entity.

Adult↗

Exocrine pancreatic neoplasms induced by methylazoxymethanol acetate in the guppy Poecilia reticulata.

Exocrine pancreatic neoplasms developed in the guppy Poecilia reticulata following a single brief exposure to methylazoxymethanol acetate [(MAM-Ac) CAS: 592-62-1]. Fish 6-10 days old were exposed to concentrations of MAM-Ac up to 100 mg/liter for 2 hours. Exposed specimens were transferred to carcinogen-free water and sampled periodically for tumor development. Pancreatic neoplasms occurred in approximately 9% of histologically examined individuals exposed to 10 mg MAM-Ac/liter or less. Neoplastic lesions were not found in 122 control specimens. The neoplasms included 6 cases diagnosed as adenoma, 7 cases diagnosed as acinar cell carcinoma, and 2 cases diagnosed as adenocarcinoma. Adenomas consisted mainly of well-differentiated acinar cells that were filled with zymogen granules. Two adenomas also contained foci of atypical, less-differentiated acinar cells possessing basophilic, fibrillar cytoplasm. Acinar cell carcinomas occurred in several cellular patterns that ranged from well-differentiated to more anaplastic lesions; however, none exhibited areas of ductular proliferation. Adenocarcinomas, on the other hand, exhibited a glandular growth pattern and contained numerous ductlike structures. Both types of carcinomas appear to arise from primary acinar cells. Thus lesions probably progress from adenomas to acinar cell carcinomas and adenocarcinomas. The findings of carcinogen-induced pancreatic neoplasms in guppies further strengthen the usefulness of small fish species in carcinogen testing and provide an additional model for pancreatic tumors.

Adenocarcinoma↗

Exocrine pancreatic neoplasms in the mummichog (Fundulus heteroclitus) from a creosote-contaminated site.

A high prevalence of exocrine pancreatic neoplasms occurred in mummichog, Fundulus heteroclitus, from a creosote-contaminated site in the Elizabeth River, Virginia. A total of 20 neoplasms were found in a group of about 1,300 fish obtained at this site over a 2-yr period. Of 240 fish collected during October 1991, 3.3% had pancreatic neoplasms. Adjusted total lesion prevalence for large adult fish (Size Class III: total length = 75-85 mm; Size Class IV: total length > 85 mm) was 6.7%. Pancreatic neoplasms were not observed in 234 fish collected at this site during May 1991, nor were they found in 420 fish obtained during fall 1991 from 1 uncontaminated and 6 moderately contaminated localities. Lesions involved both mesenteric and intrahepatic exocrine pancreas and ranged from well-differentiated acinar cell adenomas to poorly differentiated acinar cell carcinomas. One fish had an atypical acinar cell focus. All specimens with pancreatic neoplasms also had hepatocellular lesions. This epizootic of exocrine pancreatic neoplasia is the first to be reported in a wild fish population. Based on chemical characterization of the site and limited experimental data on chemically induced pancreatic carcinogenesis in other small fish species, the neoplasms were probably caused by exposure of the mummichog to chemical carcinogens in their environment.

Adenoma↗

Accuracy of cytologic interpretation of pancreatic neoplasms by fine needle aspiration and pancreatic duct brushings.

OBJECTIVE: To investigate the accuracy of fine needle aspiration (FNA) specimens and pancreatic duct brushings in the detection of pancreatic lesions and to compare the results with follow-up biopsy and/or surgical interpretation. STUDY DESIGN: We reviewed a total of 57 specimens (37/20), 37 FNA specimens and 20 pancreatic duct brushings, from 45 patients treated at Froedtert Memorial Lutheran Hospital, affiliated with the Medical College of Wisconsin, Milwaukee, over a 4-year period. The FNA and brushing samples were categorized as follows: positive for malignancy (21/3 = 24), suspicious for malignancy (8/7 = 15) and atypical (8/10 = 18). The results were then correlated with the tissue diagnosis. RESULTS: The 24 cytologic samples positive for malignancy included 23 (20/3) pancreatic ductal carcinoma (CA) and 1 (1/0) neuroendocrine CA; in the suspicious category, 11 (6/5) were pancreatic ductal CA; 2 (0/2) mucinous neoplasms and (2/0) neuroendocrine neoplasms; in the atypical category; 2 (2/0) suggestive of mucinous neoplasia, 1 (1/0) suggestive of serous neoplasia and 9 (2/7) favor reactive; and 6 (3/3) without further categorization. Tissue diagnoses were available in 26 cases: 12 (10/2) cases positive for malignancy, 8 (5/3) suspicious for malignancy and 6 (5/1) atypical. The 12 cytologically positive cases confirmed by histology showed 10 ductal CA, 1 neuroendocrine CA and 1 negative. All 8 cases (100%) suspicious for malignancy revealed positive results, including 5 ductal CA, 1 neuroendocrine neoplasm, 1 mucinous cystic neoplasm and 1 lymphoma. Of the 6 atypical lesions, 1 showed ductal CA, 2 mucinous cystic neoplasm and 3 chronic pancreatitis. CONCLUSION: Pancreatic FNA and duct brushings [table: see text] are accurate methods in identifying pancreatic lesions, particularly ductal CA. Accuracy can be improved in the case of mucinous and other lesions with adequate cellularity of the smear and recognizing the limitations of brush samples in the case of mucinous cystic lesions. False negative results may occur in cases of poor representation of malignant cells or poor sampling.

Adenocarcinoma↗