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     

Pancreas divisum, pancreatic pseudocyst, and choledochal cyst in an 8-year-old child

An 8-year-old American Indian girl presented with acute pancreatitis. Ultrasonography and computerized tomography (CT) showed two large cystic masses near the head of the pancreas. During 4 weeks of complete bowel rest and total parenteral nutrition, she had multiple exacerbations of pancreatitis without resolution of the cysts. There were no symptoms of biliary ductal obstruction. Endoscopic cholangiopancreatography (ERCP) showed an 8-cm pancreatic pseudocyst, pancreas divisum, and a 10-cm choledochal cyst. The junction of the common bile duct and the pancreatic duct of Wirsung had a normal relationship. She underwent a Roux-en-Y pseudocyst-jejunostomy and an accessory sphincteroplasty. Three months later, a choledochal cyst excision was performed with biliary reconstruction. The patient is well and asymptomatic 1 year later. This case suggests that pancreatic juice reflux into the common bile duct may not be the only etiology of choledochal cyst formation.     

Ductal branch-oriented minimal pancreatectomy: Two cases of successful treatment

Two patients with intraductal papillary-mucinous adenoma of the pancreas were successfully treated by ductal branch-oriented minimal pancreatectomy. We propose this novel less invasive ductal branch-oriented pancreatectomy, as indicated for benign ductal ectasia of the pancreas. The cystically dilated branch duct is identified by intraoperative ultrasonography, intraoperative balloon pancreatography, and injection of indigocarmine into the cyst. The cystically dilated branch is resected from the surrounding pancreas together with minimal removal of the pancreatic parenchyma. The communicating duct and cutting margins are tightly ligated to prevent pancreatic juice leakage and fistula. A drainage tube is placed in the main pancreatic duct whenever possible. Histopathologic examination of the transected branch duct is necessary to check for mucosal extension of dysplastic epithelium. This ductal branch-oriented minimal pancreatectomy is the least invasive pancreatectomy and a suitable operation for branch-type ductal ectasia of the pancreas, which is usually benign. Received for publication on Jan. 5, 1998; accepted on April 3, 1998     

Pancreatic cyst associated with pancreas divisum treated by laparoscopy-assisted cystgastrostomy in the intragastric approach: a case report and a review of the literature

A 39-year-old Japanese man was admitted to our hospital after experiencing recurrent episodes of pancreatitis over the previous 2 years. On the first episode, he had been admitted to our hospital with elevated serum amylase levels and epigastralgia. Abdominal computed tomography (CT) revealed a diffuse, uncircumscribed area with heterogeneous density in the pancreas. No previous history of pancreatitis, gallstones, drinking, or abdominal injury was elicited. Magnetic resonance cholangiopancreatography (MRCP) demonstrated that the Wirsung duct was unconnected to the Santorini's duct. Endoscopic retrograde cholangiopancreatography through the papilla of Vater and accessory papilla revealed an enlarged ventral pancreatic duct, pancreas divisum, and a cystic lesion in the pancreatic body. On the second and third episodes, endoscopic drainage of the pancreatic pseudocysts through the accessory papilla and ultrasonography-guided transmural drainage were unsuccessful. A follow-up CT and MRCP demonstrated that the pancreatic cyst had enlarged to 9 x 8 cm in diameter. A laparoscopy-assisted cystgastrostomy was performed with an intragastric approach. An anastomosis was performed using an endoscopic linear stapler through the small cystotomy and gastrotomy openings on the posterior wall of the stomach. The postoperative clinical course was uneventful. Over 6 months later, the patient remains well and with a good quality of life. A laparoscopy-assisted cystgastrostomy, using an intragastric surgical technique, offers a safe, less-invasive procedure for cyst drainage by the pancreas divisum.     

Results of cystoduodenostomy for treatment of pancreatic pseudocysts

To evaluate the safety and efficacy of cystoduodenostomy, the cases of 117 patients operated on for pancreatic pseudocysts during the last 14 years have been reviewed. Eleven patients were treated with cystoduodenostomy. They included ten men and one woman whose ages ranged from 26 to 56 years (mean 41 years). The etiology of pancreatitis was alcohol abuse in nine patients, alcohol abuse and gallstones in one, and trauma in one. Three patients had another cyst located within the body or tail of the pancreas which was identified preoperatively by ultrasound. Each patient underwent transduodenal cystoduodenostomy and three had a concomitant cystogastrostomy for a second pseudocyst. There was no operative mortality. Morbidity included postoperative pancreatitis in one patient, a wound infection and pancreatic fistula in one, and excessive bleeding from the cyst in one. There were no injuries to the common bile duct. Upon follow-up, which ranges from 6 months to 8 years, none of the patients has had a persistent or recurrent pseudocyst. This has been confirmed by ultrasound or computerized tomography (CT scan) in nine patients. Transduodenal cystoduodenostomy is a safe, reliable means of internal drainage for mature pseudocysts that are located in the head of the pancreas adjacent to the duodenum. Preoperative evaluation of the pancreas to rule out multiple pseudocysts and intraoperative care to avoid injury to the common bile duct are important factors in obtaining these good results.     

胰腺大囊性浆液性囊腺瘤临床分析

目的 探讨胰腺大囊性浆液性囊腺瘤(MSAP)的诊断与治疗.方法 对1999年10月至2009年10月收治的5例MSAP患者的临床资料进行回顾性分析.男性1例,女性4例.结果 5例MSAP患者中,临床表现为腹痛、腹胀3例,黄疸1例,无明显症状1例.超声和CT检查显示胰腺大囊性占位性病变;其中5例均边界清楚、有分隔、囊壁强化、未见囊壁结节及乳头状突起;4例囊壁光滑、较薄;3例病灶为分叶状,2例病灶为类圆形;2例伴胰管扩张,1例伴胆道扩张.5例MSAP中,肿瘤发生于胰头2例、胰体尾3例,肿瘤直径均大于5.0 cm,平均直径8.8 cm.5例均行手术切除,术后2例发生胰瘘,均治愈.随访8～35个月,未见复发.结论 MSAP缺乏特异性临床表现,超声和CT等影像学特征与胰腺黏液性囊性肿瘤相似,肿瘤逐渐增长并产生压迫症状,手术切除后可治愈.     

Single-branch resection of the pancreas

The treatment of intraductal papillary mucinous tumors (IPMT) of the pancreas is still controversial. In this report we describe a single-branch resection of the pancreas (SBRP), which is a new method for the removal of branch-type IPMT of the head of the pancreas. A multilobular cystic lesion (50 × 40mm) in the head of the pancreas was incidentally detected in an asymptomatic 40-year-old man who underwent a routine ultrasound examination. The tumor was carefully removed along the border of the cyst and the normal parenchyma, with complete preservation of the main pancreatic duct and the common bile duct. A pancreatic fistula developed during the postoperative period, but was well-controlled by endoscopic naso-pancreatic drainage. SBRP is a technically feasible procedure and this operation represents a minimally invasive alternative to any other segmental resection of the pancreas.     

Preoperative endoscopic pancreatic stenting for prophylaxis of pancreatic duct disruption during extirpation of a pancreatic head tumor

BACKGROUND: Pancreatic fistula is a major problem in minimal invasive surgery of the pancreas. To prevent the disruption of the pancreatic duct, the surgeon must recognize the site of the pancreatic duct exactly. METHODS: We reviewed the cases of 7 patients who underwent preoperative endoscopic pancreatic stenting for the prophylaxis of pancreatic fistula development after enucleation of a benign pancreatic head tumor. RESULTS: Preoperative endoscopic pancreatic stenting was successfully performed in all 7 patients. The level of serum amylase increased to 1500 IU/L on postoperative day 1, but levels recovered to normal within 3 days. None of the patients developed a pancreatic fistula. CONCLUSIONS: Preoperative pancreatic duct stenting is a feasible, effective, and safe technique to prevent pancreatic duct disruption during enucleation of a benign tumor of the pancreatic head.     

Enteric duplication cyst of the pancreas with duplicated pancreatic duct

Enteric duplication cyst is one of the rarest forms of cystic lesion of the pancreas. We report a unique case of an enteric duplication cyst of the pancreas that was communicating with a duplicated pancreatic duct. A 7-year-old girl with severe acute abdominal pain was found to have a large cyst that was smoothly communicating with the dilated pancreatic duct in the pancreatic tail. Analysis of cyst fluid showed elevated levels of amylase, carcinoembryonic antigen (CEA) and CA 19-9, and no epithelial cells. Intraoperative cyst pancreatography revealed that the pancreatic duct was duplicated in the tail: 1 duct was communicating with the cyst, and the other was dilated within the pancreatic tail. The patient underwent spleen-preserving distal pancreatectomy and complete cyst excision without complication. Because preoperative diagnosis of duplication cyst of the pancreas is difficult, this condition should be considered during differential diagnosis of atypical cystic lesions of the pancreas. Complete excision is desirable for the management of duplication cyst of the pancreas.     

Mucinous cystadenoma of the pancreas associated with acute pancreatitis and concurrent pancreatic pseudocyst

We report an unusual occurrence of a recurrent pancreatic pseudocyst caused by an underlying mucinous cystadenoma of the distal pancreas. A 54-year old female was admitted for acute pancreatitis. Her only risk factors included the use of hydrochlorothiazide and two or three glasses of wine daily. Abdominal computed tomography (CT) done a week after onset of her symptoms showed a 5-cm cystic lesion in the tail of the pancreas suspected to be a pseudocyst. Her symptoms subsequently resolved. One month later, she had another episode of pancreatitis and an abdominal CT showed an 11 x 16 cm pseudocyst along with the previously mentioned cystic lesion. Approximately 6 weeks after her initial presentation, she was taken to the operating room for an exploratory laparotomy and cyst gastrostomy for a symptomatic pseudocyst. An intraoperative frozen section of the cyst wall showed a fibrous wall with acute and chronic inflammation without an epithelial lining. Six weeks after her cyst gastrostomy, she returned with abdominal pain, early satiety, and anorexia. Abdominal CT showed reaccumulation of fluid within the pseudocyst and endoscopic retrograde cholangiopancreatography (ERCP) revealed a normal caliber pancreatic duct with an abrupt cutoff at the distal duct. She underwent exploratory laparotomy with drainage of 3 L of fluid from the pancreatic pseudocyst. After gaining access to the lesser sac, a 6-cm cystic lesion was identified in the tail of the pancreas. She underwent a distal pancreatectomy and splenectomy. The intraoperative and final pathology confirmed the presence of a benign mucinous cystadenoma. The patient had an uneventful recovery, began to tolerate oral intake, and was discharged 7 days after surgery. The differentiation between a pancreatic pseudocyst and benign cystic neoplasms of the pancreas is crucial to determine treatment options. Cystic neoplasms of the pancreas, whether mucinous or serous, have the potential to harbor malignancy, and resection is recommended.     

Pancreatic abscess due to infected hydatid disease

A 35-year-old man sought treatment for acute epigastric pain, mild jaundice, and high fever. The patient was alcoholic and had been treated for a week in another hospital, at which time the clinical findings and indirect laboratory test indicated an attack of acute pancreatitis. At admission to the surgical intensive care unit of Aretaieon Hospital, the patient was in a septic condition; a computed tomographic scan of the upper abdomen revealed an abscess at the head of the pancreas. Surgical exploration showed an infected hydatid cyst at the head of the pancreas and pancreatic edema. The cyst was evacuated and drained, which resulted in formation of an external pancreatic fistula. Fistulography showed a communication of the residual cavity with the main pancreatic duct. The patient was begun on a regimen of somatostatin infusion, and the fistula closed within 3 days. The patient was discharged on the sixteenth postoperative day and has remained in good health for a year.     

Lymphoepithelial cyst of the pancreas that was difficult to distinguish from branch duct-type intraductal papillary mucinous neoplasm: Report of a case

A 58-year-old woman was admitted to our hospital to optimize the management of her diabetes mellitus. A computed tomography (CT) scan showed a 30-mmdiameter, multilocular cyst in the head of the pancreas. The tumor markers, including DUPAN 2, SPAN-1, and carbohydrate antigen 19-9, were within the normal ranges. A contrast-enhanced CT scan showed a nonenhanced, multilocular cyst. Abdominal magnetic resonance imaging showed a multilocular cyst. Endoscopic retrograde cholangiopancreatography showed that the main pancreatic duct was normal. Based on these findings, we suspected a branch duct type intraductal papillary mucinous neoplasm. A distal pancreatectomy with a splenectomy was performed, since more of the mass was located on the dorsolateral side, inconsistent with the preoperative imaging results. On the resected specimen, a 4-cm-diameter, multilocular cyst containing serous fluid was found. Pathologically, the cyst wall was lined with squamous epithelium surrounded by abundant lymphoid tissue with follicles, consistent with a lymphoepithelial cyst of the pancreas, which is an unusual benign cyst.     

Ventral pancreatic resection for adenoma and low-grade malignancies of the head of the pancreas

The head of the pancreas can be anatomically divided into two sections, one drained by the duct of the Santorini system, and the other drained by the ventral pancreatic duct. This study was undertaken to determine whether independent resection of the ventral pancreas drained by the ventral pancreatic duct could be performed safely and effectively, by employing the following method in four patients. First, the duodenum and pancreas were sufficiently separated preserving the mesoduodenum and the posterior pancreaticoduodenal artery. Next, the main pancreatic duct was divided at the papillary portion, and sectioned at its junction with the duct of Santorini, ensuring preservation of the intrapancreatic bile duct. After the ventral pancreas had been detached from the glistening intrapancreatic bile duct, the ventral pancreas was connected with the dorsal pancreas by only the pancreatic parenchyma. The ventral pancreatic resection was completed following the incision of this border. A pancreatic fistula developed in one patient postoperatively, but this healed within 30 days. The hospital stay after surgery ranged from 35 to 58 days, and a good quality of life was maintained in all four patients. Thus, we conclude that ventral pancreatic resection can be safely performed and is especially valuable for treating the increasingly frequent adenomas and borderline malignancies in the main pancreatic duct system of the head of the pancreas.     

Postnecrotic cysts of the pancreas: possible ways of minimally invasive external and internal drainage

The authors have shown a possibility of minimally invasive interventional surgery in treatment of postnecrotic cysts of the pancreas and their complications. The most optimal accesses for drainage of the cavities located inside the body and the pancreas head are determined. The use of the methods of internal drainage of the cyst cavity connected with the main pancreatic duct allowed avoidance of open operations. The results of antegrade percutaneous or transcavital as well as retrograde endoscopic restoration of the patency of the main pancreatic duct in treatment of the pancreatic cysts connected with the duct system are shown.     

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.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vaters papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vaters papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

胰体尾切除术后并发胰瘘的防治

目的 探讨胰体尾切除术后胰瘘的预防及治疗措施.方法 回顾性分析2000-2009年124例胰体尾部切除病例,包括胰腺恶性肿瘤86例,胰腺假性囊肿及良性肿瘤12例,胃肠肿瘤侵犯胰体尾18例,慢性胰腺炎合并胰体尾囊肿3例,外伤5例.以单纯间断褥式缝合(A组)处理胰腺残端63例;以改良的主胰管缝扎+间断褥式交锁缝合(B组)处理胰腺残端61例.所有患者均放置负压引流,术后均常规应用生长抑素.结果 A组63例中22例术后发生胰瘘,发生率为34.9％,其中高流量胰瘘10例,低流量胰瘘12例;B组61例中9例术后发生胰瘘,发生率为14.7％,无高流量胰瘘.两组胰瘘患者中,A组住院天数明显延长(P＜0.01),术后医疗费用明显增加(P＜0.01).结论 主胰管缝扎+间断褥式交锁缝合的手术方法可减少术后胰瘘的发生,有助于减少住院天数及术后医疗费用.     

Choledochal cyst and benign stenosis of the main pancreatic duct

We report here the first case of choledochal cyst associated with a benign stenosis of the cephalic part of the main pancreatic duct. The pancreatic ductal stenosis was associated with a protein plug located upstream of the stenosis. Preoperatively, it was not possible to rule out a localized intraductal pancreatic tumor, and a pylorus-preserving pancreaticoduodenectomy was performed. This association has not been described previously, and gives new insights into the pathogenesis of acute pancreatitis associated with choledochal cyst. Received: August 8, 2000 / Accepted: October 12, 2000     

Seltene Ursache einer rezidivierenden, nekrotisierenden Pankreatitis

BACKGROUND: Necrotising pancreatitis may develop as a consequence of pancreatic duct obstruction by stones, tumors or in the presence of a pancreas divisum. Alcohol and nicotine are regarded as risk factors for the disease becoming chronic. PATIENT AND COURSE OF THE DISEASE: A 63-year-old female patient with suspected cystadenocarcinoma of the pancreas tail, which was resolved as a pancreatic pseudocyst, was treated for recurrent pancreatitis for 2 years. A tumor in the pancreas head was only detected on a follow-up CT after resection of a complicating liver abscess. In retrospect, progressive pancreatic duct anomalies were visible on previous scans. Partial duodenopancreatectomy confirmed the presence of a pancreas head carcinoma. CONCLUSION: Continuous critical re-evaluation of all potential causes of pancreatitis including rare conditions, such as a tumor, is required particularly if pancreatitis recurs over a long period. Re-evaluation of studies over time and of findings apart from the actual main focus of the complication, in this case pancreatitis of the pancreas tail, may help to detect the underlying disease instead of just treating the consequences.     

Failure of percutaneous catheter drainage of pancreatic pseudocyst

Percutaneous catheter drainage (PCD) of symptomatic pancreatic pseudocysts under CT radiologic guidance is a valuable adjunct or alternative to operative pseudocyst management. PCD failure is characterized by the development of recurrent pseudocysts or external pancreatic fistulas. The purpose of this study is to define the cause and management of PCD failure patients. A retrospective review and analysis of patients with symptomatic pancreatic pseudocysts managed with PCD who required subsequent operative treatment because of PCD failure was undertaken. There were 23 study patients (18 men, 5 women) with a mean age of 44 years identified over a 13-year time period. Pancreatitis etiology was alcohol abuse in 10, gallstones in 7, pancreas divisum in 3, trauma in 2, and sphincter of Oddi dysfunction in 1. Endoscopic retrograde cholangiopancreatography findings were: 13 genu strictures, 4 main pancreatic duct dilations, 2 head strictures, 1 body stricture, 1 stricture in the tail, 1 intact duct, and 1 unknown. Operations used to manage PCD failures were: lateral pancreaticojejunostomy (LPJ) in 9 patients, Roux-en-Y pancreatic fistula jejunostomy in 7, distal pancreatectomy in 3, caudal pancreatectomy in 2, pancreatoduodenectomy in 1, cyst gastrotomy in 1, and caudal pancreatojejunostomy in 1. Follow-up has ranged from 1 to 13 years (mean, 5 years). Five patients who underwent pancreatic fistula jejunostomy developed recurrent pseudocysts or pancreatitis. There have been no recurrent pseudocysts or fistulas in patients managed with LPJ or pancreatic resection. Genu strictures were the cause of PCD failure in the majority of patients. LPJ is the treatment of choice for genu strictures but may not always be possible because of chronic inflammatory changes. Roux-en-Y pancreatic fistula jejunostomy is an acceptable alternative. Recurrent pseudocysts in the head and body are treated with LPJ with cyst incorporation. Pancreatic resection is appropriate for certain strictures of the head, body, and pancreatic tail. Failure of PCD is associated with an underlying ductal disorder that needs to be defined preoperatively with endoscopic retrograde cholangiopancreatography to select the appropriate operation.