胰管内乳头状黏液瘤的临床研究

目的 通过探讨胰管内乳头状黏液瘤（IPMT）的临床、逆行胰胆管造影（ERCP）表现及病理特征,提高对IPMT的认识。方法 对9例IPMT患者的临床、影像学表现及病理资料作回顾性分析。结果 9例中男女之比为2：1,年龄为37-76岁,平均68.4岁。上腹痛为最常见症状。肿瘤主要位于胰头部。ERCP可见十二指肠乳头肿大开口扩大,见大量黏液流出及主胰管扩张。病理表现多样性,可表现为腺瘤和腺癌。结论 IMPT是胰腺肿瘤的一种,其预后较一般的胰腺癌好,临床上应将其与其他胰腺肿瘤加以区别。     

Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum

A 34-year-old man was admitted to our hospital with the chief complaints of back pain and epigastralgia. The physical examinations on admission disclosed no abdominal tumor. The serum concentration of total bilirubin was 1.4?mg/dl. The serum elastase-1 level was elevated to 526?ng/dl. Computed tomography showed a cystic lesion, 1?cm in diameter, in the head of the pancreas, without dilatation of the main pancreatic duct. Endoscopic retrograde cholangiopancreatography via the papilla of Vater and the accessory papilla revealed an enlarged ventral pancreatic duct and pancreas divisum. The preoperative diagnosis was mucin-producing pancreatic tumor in the ventral pancreas of a patient with pancreas divisum. A pylorus-preserving pancreatoduodenectomy was performed. The gross findings of the cut surface of the resected specimen disclosed mural nodules in the dilated duct of the ventral pancreas. A histological examination of the mural nodules in the ventral pancreas revealed mucin and intraductal papillary adenoma. Benign tumors associated with pancreas divisum are rare; to the best of our knowledge, only three cases have been reported. Although in these three patients the tumor developed in the dorsal pancreas, the tumor developed in the ventral pancreas in our patient.     

Intraductal carcinoma in a surgically resected pancreas with chronic pancreatitis

A 59-yr-old Japanese male presented with epigastralgia. Endoscopic retrograde cholangiopancreatography (ERCP) revealed narrowing of the inferior common bile duct and protein plugs in the main pancreatic duct. He was diagnosed as suffering from chronic pancreatitis with suspicion of a pancreatic head tumor, and a pancreatoduodenectomy was performed. Histologically, a diffuse chronic pancreatitis was evident in the resected pancreas. Although no tumors were seen in the head portion of the pancreas around the inferior common bile duct, an intraductal carcinoma was found in the second branch of Santorini’s duct. Precancerous alteration of the duct epithelium, presenting papillary hyperplasia, and atypical hyperplasia were observed in areas continuous with the intraductal carcinoma. Immunohistochemically, carcinoembronic antigen (CEA) was specifically expressed in atypical hyperplasia and intraductal carcinoma, but not in papillary hyperplasia.     

"Duodenal intussusception" due to adenoma of the papilla of Vater

The case of a 55-year-old woman with a pedunculate adenoma of the papilla of Vater is presented. Diagnostic imaging modalities including ultrasonography, CT scan, magnetic resonance of cholangiopancreatography, simultaneous duodenography and cholangiography, and angiography showed a giant tumor protruding intraluminally and moving forward in the duodenum by peristalsis. It had a duodenal intussusception-like appearance, with remarkable left-lower deviation of the common bile duct and major pancreatic duct in the papilla of Vater as far as the left side of the aorta. Episodes of jaundice or ileus were absent, probably because the tumor was mobile in the duodenum. As biopsy specimens showed no malignancy and intraductal ultrasonography in the common bile duct revealed no intraductal invasion of the tumor in the papilla of Vater, the patient underwent transduodenal papillectomy with papilloplasty with pancreatic ductoplasty. Pathological diagnosis of consecutive specimens was a papillary adenoma with moderate atypia and occasional tubular structure. There seems to be an exceptional subtype of the tumor in the papilla of Vater, like this case, demonstrating the duodenal intussusception-like appearance without prominent clinical symptoms.     

Case of intraductal papillary mucinous tumor (noninvasive adenocarcinoma) of the pancreas resected 27 years after onset

Summary A case of intraductal papillary mucinous tumor (IPMT) of the pancreas resected 27 yr after onset is presented. In March of 1997, a 71-yr-old man was admitted to our hospital with a complaint of severe epigastric pain. He had initially undergone endoscopic retrograde pancreatography (ERP) in April 1971 in our hospital and the patient had been followed up for pancreatografic changes for 26 yr. Dilatation of the main pancreatic duct gradually progressed during follow-up, and the filing defect owing to the tumor became demonstrable. On admission, ERP revealed diffuse dilatation of the main pancreatic duct, which was 20 mm in diameter, and the filling defect of 35 mm in diameter. We diagnosed this patient as having an IPMT of the pancreas. Considering his general condition, pancreatic segmentectomy was carried out, and the postoperative course was favorable. Histological findings were compatible with those of noninvasive papillary adenocarcinoma. This is a precious case for studying the natural history of intraductal papillary tumor of the pancreas and to evaluate the application of surgery, because the biologic behavior of this tumor is much less aggressive than that of pancreatic ductal cell carcinoma.     

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 × 40 mm) 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.     

Intraductal papillary mucinous tumor of the pancreas: computerized tomography and magnetic resonance imaging features

The aim of this study was to analyze the computerized tomography (CT) and magnetic resonance imaging (MRI) features of intraductal papillary mucinous tumor (IPMT) of the pancreas. The cases of eight patients with pathologically proven IPMT (1 papillary hyperplasia, 7 adenocarcinoma) of the pancreas were retrospectively reviewed. There were five men and three women with ages ranging from 42 to 82 years. Imaging studies included six thin-section dynamic CT scans, seven MRI scans, one MR cholangiopancreatography scan, and two endoscopic retrograde cholangiopancreatography scans. There was only one benign IPMT, which presented as a unilocular cyst in the pancreatic body with no mural nodules and no dilatation of the main pancreatic duct (MPD). All seven patients with malignant IPMT had multilocular cysts with papillary projections in the pancreatic head and/or uncinate process accompanied by dilated MPD (5 diffuse, 2 segmental). Communication between the cystic lesions and the MPD were evident in all seven patients. One patient had small mural nodules in the branch ducts of the pancreatic body and five had a bulging papilla with a patulous orifice. A mass effect resulting in biliary obstruction was shown in one patient. One patient had a ruptured cyst with mucin leakage into the right anterior pararenal space following sono-guided aspiration. In conclusion, the main imaging feature of IPMT in our patients was a multilocular cyst with papillary projections located in the pancreatic head and uncinate process. Although CT and MRI cannot differentiate mucin content from pancreatic juice, communication between the cystic lesion and the dilated MPD and a bulging papilla with a patulous orifice are characteristics of IPMT.     

Case of intraductal papillary mucinous tumor (noninvasive adenocarcinoma) of the pancreas resected 27 years after onset.

A case of intraductal papillary mucinous tumor (IPMT) of the pancreas resected 27 yr after onset is presented. In March of 1997, a 71-yr-old man was admitted to our hospital with a complaint of severe epigastric pain. He had initially undergone endoscopic retrograde pancreatography (ERP) in April 1971 in our hospital and the patient had been followed up for pancreatografic changes for 26 yr. Dilatation of the main pancreatic duct gradually progressed during follow-up, and the filling defect owing to the tumor became demonstrable. On admission, ERP revealed diffuse dilatation of the main pancreatic duct, which was 20 mm in diameter, and the filling defect of 35 mm in diameter. We diagnosed this patient as having an IPMT of the pancreas. Considering his general condition, pancreatic segmentectomy was carried out, and the postoperative course was favorable. Histological findings were compatible with those of noninvasive papillary adenocarcinoma. This is a precious case for studying the natural history of intraductal papillary tumor of the pancreas and to evaluate the application of surgery, because the biologic behavior of this tumor is much less aggressive than that of pancreatic ductal cell carcinoma.     

Local resection of a gangliocytic paraganglioma near the papilla of Vater

A 69-year-old man was admitted to our hospital with epigastric discomfort. Upper gastrointestinal endoscopy showed a submucosal tumor near the papilla of Vater. Abdominal CT and MRI showed a small, well-enhanced tumor. Endoscopic tumor biopsy was performed before the operation, but pathologic findings showed normal duodenal musosa. Nevertheless, since malignancy could not be ruled out, we resected the tumor with the sphincter of the papilla of Vater, followed by plasty of the orifice for the common bile duct and main pancreatic duct. We identified 3 parts with tumor cells; epithelioid cells, spindle cells, and ganglion-like cells. The tumor was diagnosed as gangliocytic paraganglioma of the duodenum. Treatment by resecting the tumor with the sphincter of the papilla of Vater, followed by the plasty of the orifice for the common bile duct and main pancreatic duct, was selected considering the patient's safety and to achieve radical cure.     

Elevated pressure in the dorsal part of pancreas divisum: the cause of chronic pancreatitis?

In 6 patients with upper abdominal pain of unknown origin presenting with pancreas divisum, the pressure in the pancreatic duct was measured via the minor papilla into which in these patients the main part of the pancreatic duct system drains. For comparison intraductal manometry via the major papilla (papilla of Vater) was performed in 8 patients with normal pancreatic duct system. The pressure in the pancreatic duct of the control group was 10.5 +/- 0.9 mm Hg, whereas in the patients with pancreas divisum it was 23.7 +/- 1.3 mm Hg. The results demonstrate that in patients with pancreas divisum, intraductal pressure may be largely increased even in the fasting state.     

Signet-ring-cell carcinoma of the ampulla of Vater: a case report

We encountered a rare variant of carcinoma of the ampulla of Vater in a 68-year-old man with postprandial abdominal pain and nausea. Ultrasonography and computed tomography showed a dilated common bile duct and main pancreatic duct. At duodenoscopy, the papilla of Vater was enlarged, and biopsy specimens taken from the papilla revealed signet-ring-cell carcinoma. Endoscopic ultrasonography showed a hypoechoic tumor without pancreatic invasion. Pylorus-preserving pancreatoduodenectomy was performed. Histologic examination of resected specimens indicated lymphatic and vascular invasion, but surgical margins were tumor-free. The patient is alive and disease-free 10 months after the operation.     

内镜诊治胰腺导管内乳头状粘液性肿瘤的应用

目的探讨胰腺导管内乳头状粘液性肿瘤(IPMN)的诊断、内镜下治疗,以提高对该疾病的认识及内镜对其诊断及治疗的意义。方法回顾性分析经内镜诊治的IPMN患者12例,总结并分析他们的一般情况、临床症状、影像学检查、实验室检查、内镜下治疗等方面资料。结果 12例IPMN患者以老年男性为主,无特异性临床表现,主要发生部位为胰头或钩突部,CT主要表现为囊实性低密度影,其内可有壁结节,胰管扩张伴或不伴胆管扩张,2例侵及胰腺实质。磁共振胰胆管造影(MRCP)主要表现为胰管扩张,胰腺萎缩,胰头部圆形高信号影。内镜下逆行胰胆管造影(ERCP)显示十二指肠乳头膨大,开口扩张,可见胶冻样粘液流出,胰管造影显示胰管全程扩张或胰头部囊状扩张,3例伴胆总管、肝内胆管扩张。9例行ERCP治疗,清除粘液栓并置入胰管支架,3例同时置入胆管支架。结论 IPMN是一种特殊类型的胰腺囊性疾病,影像学检查有其独特的表现,ERCP对其诊断及治疗有重要意义。     

A case of intraductal papillary mucinous neoplasm of the pancreas arising from pancreas divisum without ventral pancreatic duct of Wirsung]

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.     

Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is characterised by the development of numerous colorectal adenomatous polyps. Other extracolonic benign or malignant lesions have been reported previously in association with FAP but precancerous lesions in the pancreas have never been described. We report the first case of intraductal papillary and mucinous pancreatic tumour (IPMT) in a patient with FAP. A 48 year old man with a well documented past history of FAP was admitted for epigastric pain, weight loss, and new onset diabetes mellitus. Spiral computed tomography scan revealed a large tumour in the pancreatic head with upstream main pancreatic duct dilatation. Endoscopic ultrasonography confirmed these data. Mucous secretion was seen at duodenoscopy and a lesion in the main pancreatic duct was confirmed by retrograde pancreatography. The patient underwent a pancreaticoduodenectomy for suspected IPMT. Histological examination of the resected specimen confirmed an IPMT with in situ carcinoma. Twelve months after resection, the patient remained free of tumour relapse. Genetic analysis showed loss of the wild allele of the adenomatous polyposis coli gene in IPMT, causing inactivation of both alleles and demonstrating that IPMT was not incidental in this patient. IPMT should be included in the extracolonic localisation of FAP.     

Intraductal papillary adenocarcinoma with mucin hypersecretion and coexistent invasive ductal carcinoma of the pancreas with apparent topographic separation

We report a 66-year-old man who had a cystic intraductal papillary adenocarcinoma containing a papillary adenoma, in the head of the pancreas and a coexistent invasive, well differentiated solid tubular adenocarcinoma in the tail of the pancreas. He was hospitalized with acute epigastralgia. Computed tomography demonstrated a multilocular cystic mass in the head of the pancreas and a solid tumor in the tail. Endoscopic retrograde pancreatography showed mucin secretion from an enlarged papilla of Vater, marked dilatation of the main pancreatic duct in the head and body, cystic dilatation of the uncinate branch, and irregular narrowing of the main pancreatic duct in the tail. Total pancreatectomy was performed. Between the cystic tumor and the solid tumor there was a distance of 4.8 cm of normal pancreatic parenchyma and duct, recognized both grossly and microscopically. The patient died 35 months after the operation. At autopsy, peritonitis carcinomatosa was found in the abdominal cavity. Microscopically, disseminated nodules were also well differentiated tubular adenocarcinoma. The apparent anatomic separation of these two tumors within the pancreas is extremely unusual.     

A case of ampullary cancer with pancreas divisum treated by endoscopic papillectomy

A 60's man underwent a medical check-up and esophagogastroduodenoscopy revealed an exposed-type tumor at the ampulla of Vater. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a slightly dilated ventral pancreatic duct not connected to the dorsal duct. Endoscopic papillectomy was performed without pancreatic stent placement and his postprocedural course was uneventful. The specimen was histologically diagnosed as well-differentiated adenocarcinoma limited to the mucosa of the ampulla of Vater. Endoscopic papillectomy without pancreatic stent placement can be performed without a risk of post-ERCP pancreatitis for ampullary tumor limited to the mucosa of the ampulla of Vater associated with pancreas divisum.     

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 (750U/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.     

IHPBA in Tokyo, 2002: surgical treatment of IPMT vs MCT: a Japanese experience

The differences and similarities between intraductal papillary mucinous tumor (IPMT) and mucinous cystadenoma or carcinoma (mucinous cystic tumor; MCT) of the pancreas have been noted. The similarities include: (1) both tumors originate from pancreatic duct cells, (2) massive mucin production is found in both tumors, and (3) papillary projection is a common histological characteristic. However, there are also many differences. IPMT is most frequently found in men in their sixties, and originates in the head of the pancreas, with 62% (123/199) of tumors reported to be found in the head of the pancreas. This tumor sometimes spreads throughout the entire pancreas. The tumor itself basically is of the dilated pancreatic duct type, and the prognosis is generally good. In contrast, MCT frequently develops in women in their forties. This tumor is usually large, round, and almost totally encapsulated by fibrous tissue, with no communication with the pancreatic duct. The tumor histologically has an ovarian-like stroma. It most often develops in the body or tail of the pancreas. Invasion is often present and the operative prognosis is not good. IPMT resembles the shape of a bunch of grapes and MCT resembles that of an orange. From the differences between these two types of tumors, they are classified into different categories. With regard to therapeutic strategies for MCT, the tumor should be resected with lymph node dissection immediately when it is detected. In contrast, some patients with branch-type IPMT can be followed without surgical procedures. Because IPMT shows good prognosis and little tendency for infiltration, some kinds of organ-preserving procedures would be possible for some patients with this tumor. Such organ-preserving procedures are: duodenum-preserving pancreas head resection, spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein, and so on.     

Preservation of arterial arcades during duodenum-preserving total pancreatic head resection for intraductal papillary tumor

Duodenum-preserving pancreatic head resection with preservation of the bile duct or without, has been performed in cases of benign or low-grade malignancies, such as intraductal papillary tumors, of the head of the pancreas. However, the selection of the patients, the area of resection in the head of the pancreas, and the operative procedures for the preservation of the pancreaticoduodenal vessels has not been realized among surgeons to apply duodenum-preserving pancreatic head resection as a radical treatment of intraductal papillary tumors. In our experience, duodenum-preserving pancreatic head resection can be applied in the majority of the patients with the branch type of intraductal papillary tumors, and it is necessary to resect completely the head of the pancreas to avoid tumor remnant and pancreatic fistula from the remaining pancreatic rim. Therefore, we modified it to include a total resection of the pancreatic head and the preservation of both anterior and posterior arterial arcades, due to the multiformity of the location of the tumor, the variation of the branch duct in the head of the pancreas, the closure of the minor papilla in some patients, and the unbalanced development of the arterial arcades of the pancreaticoduodenal region. We performed a duodenum-preserving total pancreatic head resection with preservation of the bile duct and the both anterior- and posterior-arterial arcades for 6 patients with the normal gland involving intraductal papillary tumors. The blood flow in this organ was based on the blood supply from both preserved arterial arcades, and the duodenum had retained good color, and the postoperative results were satisfactory.     

Minute invasive ductal carcinoma of the residual pancreas after distal pancreatectomy for intraductal papillary-mucinous tumor

Summary Our report describes a 66-yr-old man who underwent surgical resection of the pancreas twice within a period of 3 yr for primary and recurrent intraductal papillary mucinous tumors (IPMTs). During the second operation, a minute invasive ductal carcinoma (IDC) was accidentally discovered in the resected specimen of the residual pancreas. The similarity and continuity between this IDC and recurrent IPMT were not recognized histologically. A solid tumor was found in the hepatoduodenal ligament 3 mo after the second operation. We performed a third operation, performing laparotomy and intra-operative radiotherapy, but could not extirpate the tumor. A biopsy specimen obtained from the tumor during this third operation revealed adenocarcinoma, and the patient later died because of tumor progression. We immunohistochemically analyzed the expression of HER-2/neu, Smad4, p16, p21, p53, mucin immunophenotypes and the Ki-67 labeling index in this series of pancreatic-duct neoplasias. Overexpression of HER-2/neu and loss of Smad4 were detected in the minute IDC, which was very different from the immunohistochemical features of both the primary and recurrent IPMTs. The IDC also showed a MUC1-positive/MUC2-negative phenotype. Therefore, we suggest that de novo IDC may occur in IPMT patients, especially those with multiple tumor recurrence. The present case may be helpful in understanding the pathogenesis of pancreatic duct lesions.