Intraductal papillary mucinous tumor of the pancreas associated with autosomal dominant polycystic kidney disease

A 43-year-old male with a history of autosomal dominant polycystic kidney disease (ADPKD) was admitted to our center with severe abdominal pain and was diagnosed with acute pancreatitis. CT showed multiple cysts in the liver and both kidneys along with ADPKD and a cystic mass, 4 cm in diameter, in the pancreatic head. The main pancreatic duct was dilated to 1 cm in diameter. The patient was diagnosed with acute pancreatitis due to intraductal papillary mucinous tumor (IPMT), and pancreatoduodenectomy was performed. Histologic examination revealed a multiloculated cystic tumor filled with mucin in the head of the pancreas. Microscopically, the tumor was diagnosed as adenocarcinoma and was found to have invaded the main pancreatic duct. Although, in addition to our case, only seven cases with association between ADPKD and malignant neoplasms have been reported, five of these cases had neoplasms arising from the pancreas. Therefore, we suggest that some genetic interactions may exist between ADPKD and pancreatic carcinogenesis.     

慢性胰腺炎伴胰管结石外科治疗体会

目的 探讨慢性胰腺炎伴胰管结石外科治疗的术式选择.方法 对1991年6月至2006年6月收治的17例慢性胰腺炎伴胰管结石手术治疗的患者进行回顾性分析,总结不同类型的胰管结石的手术方式及结果.结果 本组17例中胰头部胰管结石13例,胰体尾部胰管结石4例,合并胆石症6例,其中6例行胰管切开取石胰管空肠吻合术(Partington法);4例行胰管胃吻合术(Warren法);3例行保留十二指肠胰头次全切除术(Beger法);3例行胰尾切除胰腺空肠吻合术(Duval法);1例行胰尾、脾切除胰腺空肠吻合术.17例临床治愈,其中上腹部顽固性疼痛完全缓解15例,血糖控制2例,胰漏2例,1例术后11个月死于胰腺癌.结论 针对慢性胰腺炎合并胰管结石患者的不同状况采取的手术方式应高度个体化,有主胰管扩张者采取引流术,无胰管扩张及局部胰腺病变者采取胰腺部分切除联合内引流术,同时注意尽量保存胰腺组织功能,可明显改善患者生活质量.     

Clinical and experimental studies on serum and urine amylase levels in carcinoma of the pancreas and periampullary region

Serum and urinary amylase level are different between in cases with carcinoma of the pancreas head and in those with carcinoma of body or tail of the pancreas. In this study the relationship between elevation in serum and urinary amylase level and the portion of obstructed pancreatic duct by tumor was analysed in cases with pancreatic carcinoma was analysed and also this was investigated in experimental model of pancreatic duct ligated dog. In patients with carcinoma of the pancreas and periampullary region, the site of obstruction of the main pancreatic duct was estimated by ERP and serum and urinary amylase level were measured. The values of serum amylase level were different according to the site of obstruction of the pancreatic duct by the tumor and in cases with highly elevated serum amylase levels the main pancreatic duct was obstructed within 5cm from the duodenal papilla. Pathology of these cases revealed pancreatic fibrosis derived from pancreatitis accompanied by tumor was closely related to serum and urinary amylase level. In pancreatic ligated dogs similar findings were observed. These data suggested that elevated serum amylase level is due to the pancreatic duct obstruction in cases with carcinoma of the periampullary duodenum.     

Invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion but with changes in the pancreatic duct on pancreatography

We present a case of invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion in a 65-year-old man with a 45-year history of alcohol abuse and a 2-year follow-up of chronic pancreatitis. Two years previously, in May 1998, he was admitted for investigation of abdominal pain. Computed tomography (CT) showed diffuse dilation of the main pancreatic duct with atrophy of the pancreatic parenchyma. Endoscopic retrograde pancreatography (ERP) showed a diffusely dilated main pancreatic duct with irregular side branches in the head of the pancreas. Chronic alcoholic pancreatitis was diagnosed on the basis of the pancreatography findings. The patient was readmitted for investigation of progressive weight loss in August 2000. Serum CA19-9 levels were markedly elevated (750 U/ml) and CT showed enlargement of the head and body of the pancreas. ERP showed irregularity of the main pancreatic duct in the head of the pancreas, and the distal main pancreatic duct (which was dilated on initial ERP examination) was interrupted in the body of the pancreas. Suspected pancreatic carcinoma was diagnosed, and pylorus-preserving pancreatoduodenectomy was performed. Frozen section examination of the cut end of the pancreas revealed ductal carcinoma, and total pancreatoduodenectomy with portal vein resection was performed. Histologically, the resected tumor was diagnosed as an invasive carcinoma derived from intraductal papillary adenocarcinoma without mucin hypersecretion. We recommend observing changes in the pancreatic duct on pancreatography to diagnose invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma in a resectable state. Received: February 6, 2002 / Accepted: June 10, 2002 Offprint requests to: S. Ariizumi     

A novel technique for multiple pancreatectomies: removal of unicinate process of the pancreas combined with medial pancreatectomy

Preservation of normal pancreatic tissue in the surgical treatment of benign tumors of the pancreas offers advantages over more extended pancreatic resections. Removal of the uncinate process of the pancreas with the preservation of Wirsung's duct allows resection of a localized tumor within the uncinate process of the pancreas, maintains the flow of pancreatic juice into the duodenum, and preserves the dorsal part of the head of the gland. A pancreatic duct stent is particularly useful to identify the pancreatic duct (Wirsung's duct) intraoperatively to avoid injury which causes postoperative pancreatic leak. We have developed and employed a novel technique whereby tumors are completely excised, in combination with medial pancreatectomy, for the management of multiple mucin-producing tumors of the pancreas localized in the uncinate process and in the body of the pancreas. The cut end of the head of the pancreas is closed by interrupted sutures. Reconstruction for the distal pancreas is effected with a Roux-en-Y pancreatico-jejunostomy to the tail of the pancreas. Recovery was uncomplicated in our patient, with no endocrine or exocrine pancreatic insufficiency after 2-year follow-up. Received for publication on July 1, 1999; accepted on Aug. 17, 1999     

Studies of the growth and infiltration of experimental tumor of the pancreas in rabbits

An experimentally transplanted pancreatic cancer was induced by means of an intraductal injection of V × 2 carcinoma supernatant into the pancreatic duct of rabbits. The presence of ductal obstruction appeared to assist the nidation and growth of injected cancer cells in the pancreas. Tumors induced in the head of the pancreas grew by extension to the body and tail where they also produced interspersed, nodular tumor masses. Tumors induced in the body and tail region, on the other hand, grew by direct expansion towards the head, but this did not produce the interspersed nodular tumors in the head portion. These observations indicate that cancer of the head of the pancreas associated with the stenosis or obstruction of the pancreatic duct resulted in ductal dissemination and extensive lymphatic infiltration in the body and tail, a wider cancer invasion than recognizable at the gross cancer border. This extension was noncontinuous and interspersed, so that radical curative surgery for such cancer of the head of the pancreas would indicate total pancreatectomy.     

Surgery and chronic pancreatitis

It is hoped that, in this millennium, chronic pancreatitis will be diagnosed earlier in the course of the disease process. Improved axial imaging of the pancreatic duct and pancreatic parenchyma will diminish the need for other invasive tests. Surgical procedures are directed at pancreatic duct decompression or resection of the pancreas (head, body or tail) or, infrequently, total pancreatectomy. Pain relief in 75% to 90% is the general rule, with diabetes developing subsequently in as many as 33% of patients. Surgery for chronic pancreatitis is effective in correcting sequelae of pancreatic fibrosis. Endoscopic stenting of the pancreatic and bile duct is used more frequently today. Until their place is ascertained, careful performance of surgery will continue to be a mainstay of treatment.     

INTRAPANCREATIC PSEUDOCYST FORMATION: A COMPLICATION OF ACRYLATE PANCREATIC DUCT OBLITERATION

The case of a young woman is presented who, at the age of 33 years, had surgical occlusion of the pancreatic duct by instillation of acrylate glue for chronic relapsing pancreatitis. She presented in mid-1979, after a period of increasing frequency and severity of pain, with intractable narcotic dependency, and was shown to have a pseudocyst in the head of the pancreas. Distal pancreatic resection and cystoenterostomy have resulted in loss of narcotic dependency and a return to reasonably good health, in spite of a late development of insulin-dependent diabetes mellitus which is well controlled. Histological examination of the excised body and tail of the pancreas showed that exocrine atrophy, though advanced, was incomplete. It is suggested that in chronic pancreatitis, complete obliteration of the duct system may not be possible because of preexisting duct lesions. This may allow persistence of exocrine pancreatic tissue and lead to the complications of continuing pancreatitis and pseudocyst formation.     

Repeating regional acute pancreatitis in the head of the pancreas caused by intraductal papillary mucinous neoplasms in the tail: report of a case

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a distinct entity characterized by papillary proliferations of mucin-producing epithelial cells with excessive mucin production and cystic dilatation of the pancreatic ducts. The clinical presentation often involves recurrent episodes of pancreatitis associated with the temporal obstruction of the main pancreatic duct caused by the hypersecretion of mucin. We herein describe a case in which the patient repeatedly experienced the occurrence of idiopathic acute pancreatitis in the head of the pancreas over a 9-year period, and who was ultimately was cured by distal pancreatectomy for IPMNs in the pancreatic tail. This case illustrates the potential pitfalls in the diagnosis of IPMNs owing to a discrepancy between the site of pancreatitis and that of the IPMN. The possible mechanisms linking acute pancreatitis with the formation of IPMNs are also reviewed.     

The surgical treatment of pancreatic disease

Periampullary carcinoma and chronic pancreatitis are the most frequent indications for operations on the pancreas. Exploration and resection by pancreaticoduodenectomy is the preferred method of treatment for carcinoma of the periampullary region when the malignancy is localized to this area and when it does not invade the superior mesenteric vein. A direct anastomosis of the remaining pancreatic duct to the side of the jejunum is performed when possible. Total pancreatectomy has been utilized for extensive carcinomas of the pancreas and for early lesions with an essentially normal pancreatic parenchyma to avoid the hazards of the pancreaticojejunal anastomosis. Chronic pancreatitis has been treated by various operative procedures. Duct stenosis and calculi, fibrosis and inflammation, and occasional pseudocyst formation commonly occur in the head of the gland. This area seems to act as a site of origin and perpetuation of the disease process. Proximal pancreatic resection by pancreaticoduodenectomy is being performed more frequently with anastomosis of five to 15 per cent of the pancreatic tail to the jejunum. Diabetes may be prevented and some external pancreatic enzyme function may thereby be preserved. In any pancreaticoduodenectomy, preservation of the entire stomach and first portion of the duodenum and intact pylorus should be considered. Preliminary observations suggest that the presence of an intact stomach and a functioning pylorus tend to lessen the digestive disturbances following this resection. After total pancreatectomy, further efforts should be made to extract and regraft the patient's viable islet of Langerhans cells from the excised pancreas. A reliable method of restoring insulin production would extend the use of total pancreatectomy for both malignant and benign pancreatic disease.     

Coexistence of mucinous cystic neoplasm occurring in the head of the pancreas with annular pancreas: Report of a case

Mucinous cystic neoplasms (MCN) of the pancreas are rare tumors that are almost exclusively located in the body or the tail of the pancreas. A 60-year-old woman with no history of pancreatic disease was referred to our hospital with a chief complaint of dull pain in the upper abdomen. Abdominal computed tomography showed a multilocular cystic mass of 7.0 cm in the head of the pancreas, and endoscopic retrograde cholangiopancreatography showed no communication between the cystic mass and the main pancreatic duct. A pancreatoduodenectomy was performed for the complete resection of the tumor, and an annular pancreas was discovered by accident. The pathological examination of the tumor led to a definitive diagnosis of MCN with ovarian-type stroma. To our knowledge this is the first documented case of MCN occurring in the head of the pancreas and associated with annular pancreas.     

A duodenum-preserving and bile duct-preserving total pancreatic head resection with associated pancreatic duct-to-duct anastomosis

A duodenum-preserving pancreatic head resection technique was first reported in 1980, but the indications have been limited to benign pancreatic disease as it involves a subtotal pancreatic head resection. In 1988 we detailed a duodenum-preserving total pancreatic head resection (DPTPHR) technique. This procedure involved a total pancreatic head resection and as such expanded the indications for this approach to include tumorigenic masses. The original method involved closure of the proximal pancreatic duct and an anastomosis of the pancreatic duct of the distal pancreas to a newly created small hole in the duodenum (we termed this a "pancreatoduodenostomy"). Our current technique involves a duct-to-duct anastomosis of the proximal pancreatic duct and the distal pancreas to better preserve anatomic structure. DPTPHR was performed in 26 patients from 1988 to 2002, including 12 cases of DPTPHR with pancreatoduodenostomy and 14 cases of DPTPHR with pancreatic duct-to-duct anastomosis. No differences were observed between the two methods with respect to operative time or blood loss during surgery. Postoperatively, there was one case of cholecystitis and one case of pancreatitis in a patient who underwent a pancreatoduodenostomy; both of these patients were treated conservatively with curative intent. No complications were observed in the group undergoing duct-to-duct anastomosis. The advantage of duct-to-duct anastomosis is that the pancreatic head is totally resected, thus allowing removal of neoplastic disease such as an intraductal papillary mucinous tumor and also therapy for chronic pancreatitis. A key benefit of this procedure is that sphincter function of the duodenal papilla is preserved permitting drainage of pancreatic/bile juice into the duodenum, preserving a more physiologic state than is the case after a pancreatoduodenostomy. Supported in part by a Grant-in-Aid for Scientific Research (63480311) from the Ministries of Education, Science, and Culture of Japan.     

Malignant islet cell tumor projecting into the main pancreatic duct

We report herein a rare case of islet cell tumor showing a unique growth pattern in a patient who developed repeated acute pancreatitis as the tumor's initial symptom. Preoperative imaging examinations showed dilatation of the main pancreatic duct (MPD) and cysts around the pancreatic tail. A distal pancreatectomy with splenectomy was performed because the pancreatitis was localized in the distal pancreas and was not controlled by various drug therapies. Grossly, the tumor consisted of two component parts: a markedly infiltrative part in the pancreatic parenchyma, and a papillary elevated part in the MPD. The MPD was obstructed by the tumor spreading widely along the distal MPD. Microscopically, the tumor was composed entirely of islet cell tumors (nonfunctioning), with several foci of venous and lymphatic involvement. Based on its growth behavior, we assumed that the tumor may have arisen from the MPD or from islet cells closely adjacent to the MPD. The patient's postoperative course was uneventful and he is doing well 2 years after the operation. We discuss the growth pattern of the tumor and the cause of the pancreatitis. Received: March 30, 2000 / Accepted: July 12, 2000     

Pancreatic mucin-producing adenocarcinoma associated with a pancreatic stone: Report of a case

We present an autopsy case of an 83-year-old Japanese man with a mucin-producing adenocarcinoma accompanied by pancreatolithiasis in the head of the pancreas. He suffered from obstructive jaundice and died of disseminated intravascular coagulation. He did not normally drink alcohol and had no history of chronic pancreatitis. The autopsy findings revealed a mucinous cystic tumor, composed of multiple dilatated branches, in the head of the pancreas. Histological examinations showed papillary adenocarcinoma, which scirrhously infiltrated the distal common bile duct with perineural invasion and lymph node involvement. He was thus diagnosed to have mucin-producing branch-type cancer in the head of the pancreas. The main pancreatic duct was dilated, and the residual pancreatic tissue showed moderate fibrosis and parenchymal atrophy. A stone was observed in a dilated branch of the primary lesion. To the best of our knowledge, there have only been five previously reported cases of mucin-producing tumor associated with pancreatolithiasis. Intraductal calcification is a major characteristic of chronic pancreatitis, but it is clinically important not to misdiagnose cancers associated with pancreatolithiasis such as chronic pancreatitis.     

Intraoperative removal of pancreatic duct calculus with a rigid ureterorenoscope and ultrasonic lithotripsy

A 51-year-old man with chronic relapsing pancreatitis and recurrent episodes of acute attacks had an impacted ductal stone deep in the head of the pancreas. A staghorn calculus, 1 cm in diameter, was successfully disintegrated and removed intraoperatively with use of a visual endourologic technique and ultrasonic lithotripsy. Because of a dilated pancreatic duct and pseudocyst formation near the tail of the pancreas, distal pancreatic resection and longitudinal pancreaticojejunostomy were performed. Postoperatively, no pancreas irritation has been verified, and the patient has remained without symptoms.     

胰管结石的诊断与外科治疗：附43例报告

目的:探讨胰管结石的诊断及手术治疗方式。方法:回顾性分析6年间进行外科手术的43例胰管结石患者的临床资料。结果:患者男女比例约为2:1,主要症状为上腹痛,部分伴腰背痛。全组均采用影像学检查方法确诊。行胰管切开取石、胰管空肠侧侧Roux-en-Y吻合术34例(其中同时切除胰体尾2例),保留十二指肠的胰头切除术5例,保留幽门的胰十二指肠切除1例,胰十二指肠切除术1例,胰体尾、脾切除1例,探查发现恶变无法切除1例。随访5个月至6年;43例患者中23例腹痛症状完全缓解,18例有不同程度的缓解。结论:影像学检查是确诊胰管结石的主要方法。对胰管结石的手术应制定个体化方案。胰管切开取石,胰管-空肠Roux-en-Y吻合术为胰管结石的主要术式,其他可根据情况采用保留十二指肠的胰头切除术或胰体尾切除等术式。     

Carcinoma masquerading as a pancreatic pseudocyst on ultrasound

Ultrasound has proven invaluable in detecting and evaluating pancreatic pseudocysts, and it is now a standard test to rule out complications of pancreatitis. In reviewing the authors' experience with 122 patients treated surgically for a pancreatic pseudocyst, five patients were identified in whom an ultrasound demonstrated a pseudocyst that was associated with an unexpected cancer at the time of operation. A sixth patient, with a pseudocyst documented by ultrasound, died prior to surgery and was found at autopsy to have metastatic common bile duct carcinoma. There was little difference in presenting symptoms, age, frequency of alcoholism, or physical findings compared with patients with pseudocysts secondary to pancreatitis. In two patients, pseudocysts were found in the tail of the pancreas at operation, in addition to carcinoma. In the other three patients, no pseudocyst was found; however, a subcapsular splenic hematoma was present in one. Five patients had metastatic disease, three from pancreatic adenocarcinoma, one from islet cell carcinoma, and one from a common bile duct carcinoma. One patient with a pancreatic adenocarcinoma confined to the head underwent a Whipple procedure and has no evidence of disease 6 months later. Malignancy may cause or coexist with pancreatic pseudocysts. Ultrasound is often not helpful in distinguishing pseudocysts associated with malignancy from those associated with pancreatitis. Biopsy should be performed to rule out malignancy when operating for pancreatic pseudocysts.     

Isolated lymphoplasmacytic sclerosing pancreatitis involving the pancreatic tail

We present an interesting case of a 62-year-old woman with a 3-month history of vague, left-sided abdominal pain. CT imaging revealed a hypodense lesion in the tail of the pancreas. The patient had no history of pancreatitis or autoimmune diseases. Laboratory testing revealed a normal CA19-9 (33 U/mL) and an elevated IgG4 (133 mg/dL). Due to concerns of pancreatic malignancy, she underwent operation. We found a dense, inflammatory mass in the tail of the pancreas, which was removed via an open distal pancreatectomy with splenectomy. Histologic analysis revealed a pancreas with sclerotic ducts and surrounding lymphoplasmacytic inflammation most consistent with lymphoplasmacytic sclerosing pancreatitis (LPSP). LPSP, also termed autoimmune pancreatitis, is a benign disease of the pancreas, which can mimic pancreatic adenocarcinoma. It is the most common benign finding diagnosed on pathology after pancreatic resection for presumed malignancy. LPSP most commonly involves the head and, more uncommonly, the tail of the pancreas. It can be successfully treated with steroids obviating the need for resection. IgG4 levels may assist in recognition of this disease. As our experience with utilization of IgG4 testing and knowledge of the systemic nature of LPSP increase, patients with this disease may be spared unnecessary resection.     

Pancreatic Duct Strictures: Identifying Risk of Malignancy

Background This study aimed to define PDS characteristics that predict malignancy and would thus invoke further diagnostic evaluation or aggressive treatment.Methods 355 cases of PDS were diagnosed by ERCP during a 7-year period at a single institution. A retrospective review identified clinical/demographic patient data and ERCP results.Results 218 (61%) patients with a PDS were found to have an isolated PDS. Twelve percent of isolated PDS and 79% of CBD stricture-associated PDS were malignant. The sensitivity and specificity for the double duct sign for malignancy were 77% and 80% respectively, and the positive predictive value was 65%. Predictors of malignancy were statistically similar for both isolated PDS and those associated with a CBD stricture. Univariate predictors of malignancy included stricture location in the pancreatic head/neck, jaundice, and patient age. Predictors of benign disease included a history of pancreatitis, the presence of multiple strictures, pancreatic duct stones, pseudocyst, pancreas divisum anatomy, irregular side branches, and irregular pancreatic duct morphology. Less than 1% of patients with either pancreas divisum anatomy, pancreatic duct stones, or pancreatic pseudocyst had malignancy. Using malignancy as the dependent variable, multivariate factors included in the final prognostic equation were history of pancreatitis (odds ratio 0.009 with history of pancreatitis), stricture location in the head or neck (odds ratio 42) and irregular pancreatic duct side branches (odds ratio 0.05 with irregular branches).Conclusions This study demonstrates that certain characteristics of PDS can predict the subset of patients who have an increased risk of cancer.     

Cancer of the pancreas with hyperamylasemia

A 66-yr-old woman had several attacks resembling relapsing acute pancreatitis with elevation of serum amylase for about three months. Obstruction of the main pancreatic duct was detected with endoscopic retrograde pancreatoductography, though no abnormality was revealed on celiac and mesenteric arteriograms. At that time she had no jaundice. Laparotomy revealed a walnut-sized tumor in the head of the pancreas compressing the main pancreatic duct. Pancreaticoduodenectomy was performed. The pathological diagnosis of the tumor was well-differentiated papillary cysto-adenocarcinoma of the pancreas. She had no attack of hyperamylasemia since the operation, and is well 2 years after the surgery. Close attention to persistent elevation of pancreatic amylase levels in serum and urine would enhance the chance of detection of small pancreatic cancers before the development of jaundice.