以胆管炎起病的胰腺导管内乳头状粘液瘤一例并文献复习

胰腺导管内乳头状粘液瘤(IPMN)为胰腺囊性肿瘤之一,特点为囊肿与胰管相通,伴胰管扩张。病理表现为囊肿分泌大量粘液及囊壁结节堵塞胰管,导致胰管扩张。文献报道临床以腹痛发病最为常见,本病例以少见的胆管炎表现起病,现报告并作文献复习如下。     

Intraductal papillary mucinous tumors and mucinous cystic tumors of the pancreas: imaging

Most cystic lesions of the pancreas are nonneoplastic and inflammatory in nature. However, approximately 5%–15% of cystic pancreatic masses may be neoplastic. Among the cystic neoplasms are the mucin-producing tumors, both the intraductal papillary mucinous neoplasms and the mucinous cystic neoplasms. Their imaging features on contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) can assist in the differentiation of these lesions. The imaging findings of both intraductal papillary mucinous neoplasm and mucinous cystic neoplasm are reviewed with attention to CT and MRI.     

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 neoplasm originating from a jejunal heterotopic pancreas: report of a case

We report a rare case of an intraductal papillary mucinous neoplasm (IPMN) originating from a jejunal heterotopic pancreas, found incidentally during surgery. A 75-year-old woman was referred to our department for surgical treatment of an abdominal aortic aneurysm (AAA) and a concurrent oropharyngeal tumor. During surgery to correct the AAA, we found a jejunal tumor incidentally and performed partial resection of the jejunum. Microscopically, the jejunal tumor showed dilated ducts containing intraluminal papillae lined with mucinous epithelium with low-grade cytological and architectural atypia within the pancreatic tissue. Immunohistochemical staining revealed MUC1 (?), MUC2 (?), MUC5AC (+), and MUC6 (?) proteins. To the best of our knowledge, only six cases of IPMN originating from a heterotopic pancreas have been reported in English, and this is the first report of an IPMN originating from a jejunal heterotopic pancreas.     

Intraductal papillary mucinous tumor of the pancreas

BACKGROUND: Since 1996 the classification of pancreatic tumors was replaced by the new World Health Organization nomenclature. Formerly mucinous cystadenomas are now distinguished between intraductal papillary mucinous tumors of the pancreas (IPMT) and mucinous cystadenomas. METHODS: We reevaluated the pathological specimen and surgical therapy of 23 consecutive patients and followed up these patients up for 4 years in median. Between 1987 and 1997 we treated 8 patients with IPMT and 15 patients with mucinous cystadenomas. RESULTS: Eighty-five per cent of all patients were symptomatic. Ultrasonography and computed tomography were the most sensitive diagnostic techniques. In 25%, the entire pancreas was involved with IPMT; that was not the case in any of the patients with mucinous cystadenoma. All patients were resected with no perioperative mortality. After dismissal from the hospital, all resected patients are still alive after a median follow-up of 4 years. In no patient with IPMT, but in 1 patient with mucinous cystadenoma, the tumor recurred. CONCLUSION: Surgical resection is the treatment of choice in all cystic tumors, and the late outcome of IPMT is as good as for mucinous cystadenoma.     

Pancreatic neuroendocrine tumor with splenic vein tumor thrombus: A case report

INTRODUCTION

Pancreatic neuroendocrine tumors (PNET) are rare, often indolent malignancies. PNET are classified as functional or nonfunctional based on the secretion of hormones without a negative feedback loop; the latter account for up to 60% of PNET. Although PNET are associated with a better prognosis compared to pancreatic adenocarcinomas, they are often diagnosed in advanced stages, making them a significant source of morbidity for patients. Here we present a rare case of venous tumor thrombus arising from a nonfunctional PNET.

PRESENTATION OF CASE

A 44-year-old woman was referred for evaluation and treatment of a possible tail of pancreas PNET discovered during work-up for a 9 year history of intermittent subcostal pain. Previous endoscopic ultrasound with fine needle aspiration revealed a 3.5 cm × 3 cm mass, with cytological diagnosis of neuroendocrine tumor. Patient was scheduled for laparoscopic distal pancreatectomy. During surgery the mass was found to encase the splenic vein leading the surgeon to perform an en bloc distal pancreatectomy and splenectomy. Pathologic analysis revealed a 1.8 cm × 5 cm tumor thrombus lodged in the splenic vein.

DISCUSSION

Nonfunctional PNET usually present in advanced stages and can be associated with venous tumor thrombi. Preoperative imaging may not accurately predict the presence of venous tumor thrombi.

CONCLUSION

En bloc resection of primary tumor, involved organs and thrombus is the recommended treatment option and often results in long term survival. New multi-modality strategies are needed for detection of venous involvement in nonfunctional PNET to better assist with preoperative planning and counseling.     

Intraductal papillary mucinous neoplasm of the pancreas with a bifid pancreatic duct

A bifid pancreatic duct presenting a major bifurcation in the main pancreatic duct is one of the anatomical variations of the pancreatic ducts. We encountered a 71-year-old female with a 5-cm-diameter branch duct intraductal papillary mucinous neoplasm of the pancreas in whom preoperative endoscopic retrograde pancreatography demonstrated an anomalous bifurcation of the main pancreatic duct at the body of the pancreas. We performed a distal pancreatectomy, instead of a middle pancreatectomy, with a cutting line at the downstream pancreas to the duct bifurcation point. Intraoperative ultrasonography was useful to confirm the exact location of the pancreatic duct bifurcation as well as the tumor extension. The procedure resulted in a favorable outcome without any postoperative complications. Although a bifid pancreatic duct is an unusual anomalous condition, this case should alert surgeons to be aware of such anatomical variants when performing pancreatic resection, otherwise, incurable pancreatic complications may occur postoperatively.     

Intraductal papillary mucinous neoplasm of the pancreas: clinical characteristics and treatment outcomes of 118 consecutive patients from a single center

Background/Purpose Appropriate surgical treatment strategies based on clinicopathological findings are unavailable for intraductal papillary mucinous neoplasm (IPMN) of the pancreas. We investigated the clinical features of pancreatic IPMN in a single-center database in order to design an optimal surgical strategy. Methods The medical records of 118 consecutive patients who had undergone surgical resection between August 1994 and December 2004, in whom IPMN was histologically confirmed, were reviewed retrospectively for radiological and pathological findings. Results Most of the invasive carcinomas in these patients were detected as the main-duct type (88.5%). The type of tumor (main-duct type vs branched-duct type), the tumor size, and the dilated duct size were significant predictive factors associated with malignancy. The relative risk of malignancy was greatest at 13-mm or more ductal dilation in the main-duct type (Odds ratio, 4.1), at 35-mm or more tumor size (Odds ratio, 7.6), and for main-duct type (Odds ratio, 3.9). Major pancreatic resections such as total pancreatectomy and pancreatoduodenectomy were performed in 14.5% and 69% of the patients, respectively. There was a 19.5% rate of incomplete resection, with these patients having a positive resection margin. However, significant recurrence did not occur in patients with a benign IPMN lesion which remained at the resection margin. The overall postoperative survival rate at 5 years was 98.2% for benign IPMN and 65.3% for malignant IPMN. Conclusions Function-preserving strategies, based on the clinical status of the patient, are necessary in order to avoid possible severe metabolic complications following extended pancreatectomy in patients with benign IPMN because of the low recurrence rate and good prognosis of this entity, irrespective of margin status.     

Intraductal mucinous tumor of the pancreas: report of a clinical case

A 49-year-old diabetic patient with abdominal pain was found at ultrasonography and computed tomography to have a cystic mass in the head of the pancreas with dilatation of the main pancreatic duct. The head of the pancreas and the duodenum were removed surgically. Examination of the operative specimen showed chronic pancreatitis, dilatation of the main pancreatic duct, and impacted mucus in the secondary ducts with villous proliferation of the ductal epithelium, thus allowing a diagnosis of intraductal adenomatosis. There was no evidence of malignancy. The resection margin was involved, and consequently the remainder of the pancreas was removed six months after the initial surgical procedure. A review of the literature showed that intraductal adenomatosis tends to spread and carries a high risk of malignant transformation. Surgery is required because of the risk of pancreatic duct obstruction and pancreatic cancer. Intraductal papillary tumour of the pancreas shares many characteristic with other adenomatous proliferation of the gastrointestinal tract (colorectal villous adenoma, bile duct adenomatosis) including the presence of villous structures with increased mucus production, a tendency to spread massively, and a high risk of malignant transformation.     

Management of unresectable solid papillary cystic tumor of the pancreas. A case report and literature review

Pancreatic solid papillary cystic tumor is a rare neoplasm with an excellent prognosis if surgical excision is complete. We report on a case and review 47 more cases extracted from the published literature to assess the treatment options when solid papillary cystic tumor is considered unresectable. Chemotherapy and radiotherapy were beneficial in a limited number of patients, but therapeutic decisions must be made bearing in mind that patients may be long-term survivors without any treatment because of the tumor's slow growth.     

Intraductal papillary mucinous tumors of the pancreas

BACKGROUND: An increasing number of intraductal papillary mucinous tumors of the pancreas have been reported in recent years. The indolent character and favorable prognosis of this neoplasm have been described. METHODS: Intraductal papillary mucinous tumors were classified into main duct type (n = 8) and branch type (n = 28) according to the dominant location of the tumor. This single-institute study examined the clinicopathological features and outcome after surgical resection in patients with intraductal papillary mucinous tumors. RESULTS: The gender, age, tumor size, and prognosis were quite similar for the main duct type and branch type groups. Branch type tumors were more frequently located in the head of the pancreas than were main duct type tumors. Histological examination revealed that 88% of main duct type tumors were adenocarcinomas; however, only 46% of branch type tumors were adenocarcinomas. Five-year survival rates for the patients with all main duct type tumors (n = 8), main duct type adenocarcinoma (n = 7), all branch type tumors (n = 28), and branch duct adenocarcinoma (n = 13) were 100%, 100%, 90.6%, and 90.9%, respectively. CONCLUSIONS: Intraductal papillary mucinous tumors had a favorable prognosis after surgical treatment. A curative pancreatectomy should be indicated for this localized malignant tumor.