A rare ring-shaped anomaly of the main pancreatic duct accompanying a branch duct IPMN

The patient was a 74-year-old female. Screening computed tomography for examination of the abdomen showed a cystic mass in the pancreatic body. Close investigation using endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography revealed a very rare finding: the main pancreatic duct bifurcated at the pancreatic body, and these two ducts converged at the caudal side. A multilocular cystic mass in the pancreatic body and mucus discharge from the orifice of major papilla were observed. There was no protruded lesion in the main pancreatic duct. No findings suggested apparent malignancy. The patient was diagnosed as having hyperplastic intraductal papillary mucinous neoplasm of branch type showing a ring-shaped pancreatic duct, and was placed under follow-up.     

European experts consensus statement on cystic tumours of the pancreas

Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6 mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.     

Role of SpyGlass-DStm in the preoperative assessment of pancreatic intraductal papillary mucinous neoplasm involving the main pancreatic duct

Background/Objectives

It is often difficult to determine an adequate resection line during pancreatectomy for intraductal papillary mucinous neoplasm involving the main pancreatic duct during partial pancreatectomy. The aim of this study was to evaluate the usefulness of improved peroral pancreatoscopy using SpyGlass-DS^tm in the preoperative assessment of intraductal papillary mucinous neoplasm involving the main pancreatic duct.

Precursors to pancreatic cancer

Infiltrating ductal adenocarcinoma of the pancreas is believed to arise from morphologically distinct noninvasive precursor lesions. These precursors include the intraductal papillary mucinous neoplasm, the mucinous cystic neoplasm, and pancreatic intraepithelial neoplasia. Intraductal papillary mucinous neoplasms are grossly visible mucin-producing epithelial neoplasms that arise in the main pancreatic duct or one of its branches. The cysts of mucinous cystic neoplasms do not communicate with the major pancreatic ducts, and these neoplasms are characterized by a distinct ovarian-type stroma. Pancreatic intraepithelial neoplasia is a microscopic lesion. This article focuses on the clinical significance of these three important precursor lesions, with emphasis on their clinical manifestations, detection, and treatment.     

Serous cystadenoma of the pancreas communicating with a pancreatic duct

Summary Conclusion To differentiate serous cystadenoma from other cystic lesions communicating with the pancreatic duct, careful radiological examination is necessary. Background Communication between the cystic cavity and the pancreatic duct in an ordinary serous cystadenoma is uncommon, although it is not uncommon in other cystic lesions, such as pseudocyst, mucinous cystadenoma/adenocarcinoma, and intraductal papillary tumor. Methods A serous cystadenoma of the pancreas communicating with main pancreatic duct in a 76-yr-old male is reported. Results The communication was preoperatively demonstrated by endoscopic retrograde cholangiopancreatography and confirmed by histopathological examination of the resected specimen.     

Mass forming chronic pancreatitis mimicking pancreatic cystic neoplasm: A case report

Mass forming chronic pancreatitis is very rare. Diagnosis could be done by the pathologic findings of focal inflammatory fibrosis without evidence of tumor in pancreas. A 34-year-old man presented with right upper abdominal pain for a few weeks and slightly elevated bilirubin level on clinical findings. Radiological findings of multidetector-row computed tomography, magnetic resonance(MR) imaging with MR cholangiopancreatography and endoscopic ultrasonography revealed focal branch pancreatic duct dilatation with surrounding delayed enhancing solid component at uncinate process and head of pancreas, suggesting branch duct type intraductal papillary mucinous neoplasm. Surgery was done and pathology revealed the focal chronic inflammation, fibrosis, and branch duct dilatation. Herein, I would like to report the first case report of mass forming chronic pancreatitis mimicking pancreatic cystic neoplasm.     

Trifidum anomaly of the main pancreatic duct

BackgroundEmbryology of the human pancreas is very complex and any alteration in its development may lead to congenital biliopancreatic malformations and anomalies not thoroughly studied in literature. We here report a case of trifurcation of the Wirsung duct, avery unusual variant of the main pancreatic duct.MethodAn 80- year-old woman underwent a magnetic resonance imaging (MRI) of the abdomen and a magnetic resonance cholangiopancreatography (MRCP) to characterize a hypoechoic lesion of the pancreas detected with ultrasonography.ResultsMRI and MRCP showed a 24-mm multicystic lesion communicating with a prominent main pancreatic duct, consistent with an intraductal papillary mucinous neoplasm, as well as an ansa pancreatica. Moreover a bifidity of the distal pancreatic duct and a further accessory duct of the body of the pancreas draining into the main pancreatic duct were identified. The pancreatic tail presented normal size and morphology on axial imaging. This anomaly, not reported yet in the literature, can be categorized as a number of a duplication anomaly, in which the main pancreatic duct is trifurcated along its length.ConclusionCongenital anomalies of the pancreas and pancreatic duct are rare but not uncommonly detected on diagnostic imaging. MRI and MRCP are the non-invasive imaging modalities of choice for diagnosing congenital anomalies of the pancreas and the pancreatic duct.     

Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas

Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed. Endoscopy showed opening of and hemorrhaging from the papilla of Vater. The patient was diagnosed with hemosuccus pancreaticus caused by hemorrhaging into the cyst from the branch duct intraductal papillary mucinous neoplasm. Based on this diagnosis, elective distal pancreatectomy was performed. The histopathological diagnosis was a mucinous cystic neoplasm with intermediate-grade dysplasia based upon the pathological findings that fibrous ovarian-type stroma existed abundantly and the stroma cells were positive for progesterone receptor and inhibin. Hemosuccus pancreaticus caused by a mucinous cystic neoplasm is extremely rare and there has been only one case reported to date. In conclusion, it should be recognized that pancreatic cystic neoplasms including mucinous cystic neoplasms may cause hemosuccus pancreaticus.     

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.     

A case of mixed adenoneuroendocrine carcinoma of the pancreas mimicking intraductal papillary mucinous carcinoma

A previously healthy 52-year-old man was referred to our hospital for further evaluation of main pancreatic duct dilatation. The preoperative work-up was consistent with intraductal papillary mucinous carcinoma (IPMC) derived from a mixed type intraductal papillary mucinous neoplasm (IPMN), because multilocular cysts with enhancing thickened pancreatic head walls and dilated pancreatic ducts lined with dysplastic mucinous epithelium, with papillary proliferation from the pancreatic body to the tail, were observed; in addition, the pancreatic juice cytology was class V, which is suggestive of adenocarcinoma. Total pancreatectomy was performed because a definite mass was not found before surgical resection and the tumors could have spread to the tail. The pathological diagnosis was mixed adenoneuroendocrine carcinoma of the pancreatic head. IPMN with high- or low-grade dysplasia was not observed anywhere in the pancreatic duct. The pancreatic ductal adenocarcinoma consisted of large caliber malignant glands with intraluminal flat or papillary structures; therefore, we were unable to recognize a definite pancreatic mass before surgical resection, and suspected an IPMC derived from a mixed type IPMN.     

Ventral pancreas-preserving pancreatic head and body resection

A new procedure that included ventral pancreas-preserving pancreatic head and body resection for a huge mucinous cystic neoplasm was performed to preserve pancreatic function. A 45-year-old man was diagnosed as having a huge mucin-producing pancreatic neoplasm. The ventral pancreas-preserving pancreatic head and body resection was performed. The stumps of the caudal pancreas and the uncinate process were negative for cancer by frozen-section histology. The remaining pancreas was anastomosed by a method of double pancreatojejunostomy with a Roux-en-Y loop. The resected specimen was 15 x 10 cm in size and contained mucin. The papillary tumor was 5.0 x 4.5 x 3.0 cm in size, which was an intraductal papillary adenocarcinoma without pancreatic invasion microscopically. The postoperative course was uneventful and the patient was discharged 14 days after surgery. Glucose tolerance test was normal 24 months after surgery and both sides of the pancreatic duct were patent confirmed by magnetic resonance. The patient is well now two years after surgery. A new limited pancreatic resection, ventral pancreas-preserving pancreatic head and body resection, was safely applied to a huge mucinous cystic neoplasm of the pancreas.     

Pancreatoscopy assisted by endoscopic sphincter dilation

To investigate tumor extension to the main pancreatic duct in patients with intraductal papillary mucinous tumors, we conducted pancreatoscopy assisted by endoscopic sphincter dilation (ESD). Two patients with intraductal papillary mucinous tumors were included in this study. The endoscopic sphincter dilation was performed using an Olbert balloon-tipped catheter. A mother-baby scope system was used for endoscopic examination. After dilation of the orifice, the babyscope was inserted smoothly. Tumorous lesions (especially the typical salmon-roe appearance of the tumors) were clearly observed in the main pancreatic duct in one patient, while no tumorous lesions were identified in the other patient. The ESD procedure may be applicable for assisting insertion of the endoscope through the papilla of Vater into the pancreatic duct. ESD is useful both for treating and for diagnosing pancreatic lesions. Received: June 19, 2001 / Accepted: February 22, 2002 Reprint requests to: N. Ueno, Present address: Third Department of Internal Medicine, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan     

A case of minimal invasive carcinoma from intraductal papillary mucinous neoplasm with invasive ductal carcinoma of the pancreas]

A 69-year-old man was referred to our hospital for epigastralgia. He was found to have elevation of serum amylase and CA19-9. Ultrasonography, abdominal CT, MRCP, ERCP and EUS showed the cystic lesion and a possibility of an other tumor. There was a stenosis of the main pancreatic duct (MPD) at the pancreas head and dilatation of the MPD from the body to the tail. Intraductal papillary mucinous neoplasm (IPMN) of the branch pancreatic duct was diagnosed, and there was a likelihood of ductal carcinoma of the pancreas. We therefore performed pancreatoduodenectomy. Pathological finding showed invasive carcinoma from an intraductal papillary mucinous neoplasm with invasive ductal carcinoma of the pancreas.     

Granular cell tumor of the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration

A 68-year-old woman was referred to our hospital for the treatment of bile duct stone, pancreatic tumor, and pancreatic cysts. First, bile duct stone was removed using endoscopic retrograde cholangiopancreatography. By abdominal contrast-enhanced computed tomography, a 12-mm diameter tumor was found in the pancreatic body. The tumor was isodense compared with the surrounding pancreatic parenchyma in the non-contrast phase and poorly enhanced in the arterial phase; it exhibited gradual enhancement from the portal vein phase to the late phase. Numerous pancreatic cysts were also observed by contrast-enhanced computed tomography. By magnetic resonance imaging, the tumor was hypointense in T1-weighted images, isointense in T2-weighted images, and hyperintense in diffusion-weighted images. By magnetic resonance cholangiopancreatography, the main pancreatic duct was not dilated, and pancreatic cysts communicated with the main pancreatic duct. The pancreatic cysts were diagnosed as branch-type intraductal papillary mucinous neoplasm. Histopathologic assessment of the specimens obtained by endoscopic ultrasound-guided fine-needle aspiration revealed the tumor as benign pancreatic granular cell tumor. The patient was followed up without surgical resection. On contrast-enhanced computed tomography at 6 months after admission, the tumor did not show any changes in diameter or characteristics.     

The pathology of ductal-type pancreatic carcinomas and pancreatic intraepithelial neoplasia: Insights for clinicians

The phenotypic classification of pancreatic neoplasms is based on their cellular lineage. Thus, tumors with a ductal, acinar, and endocrine phenotype can be distinguished. Most pancreatic neoplasms show a ductal phenotype and can be classified as ductal adenocarcinomas. Less common tumors with a ductal phenotype are the variants of ductal adenocarcinoma, intraductal papillary mucinous neoplasm (including colloid carcinoma), mucinous cystic neoplasm, medullary carcinoma, and other rare tumors. Ductal adenocarcinomas most likely develop from ductal proliferative lesions arising in the pancreatic duct system. A recently adopted classification system for these lesions distinguishes between three grades of pancreatic intraepithelial neoplasia (PanIN). Molecular studies have revealed that PanIN-2 and PanIN-3 lesions represent a distinct step toward invasive carcinoma.     

Synchronous pancreatic solid pseudopapillary neoplasm and intraductal papillary mucinous neoplasm

Solid pseudopapillary neoplasm (SPN) is a rare and low-grade malignant pancreatic neoplasm composed of poorly cohesive monomorphic neoplastic cells forming solid and pseudopapillary structures with frequent hemorrhagic-cystic degeneration. Intraductal papillary mucinous neoplasm (IPMN) is a pancreatic exocrine tumor composed of intraductal papillary growth of mucin containing neoplastic cells in the main pancreatic duct or its major branches. In the case presented here, a 53-year-old, Japanese man was found to have multiple cystic lesions and dilatation of the main pancreatic duct in the neck of the pancreas. Histological examination revealed a main-duct and branch-duct type IPMN, of the gastric-type, involving the neck of the pancreas, associated with a 0.5 cm SPN in the caudal side of the IPMN. We diagnosed this case as synchronous SPN and IPMN. As far as we know, only one other case of synchronous SPN and IPMN has been reported. Both the present case and the previously reported case showed abnormal nuclear expression of β-catenin in SPN, whereas IPMN showed no abnormal nuclear expression. These results suggest that β-catenin abnormality is not a common pathogenetic factor of synchronous SPN and IPMN.     

Updates in diagnosis and management of pancreatic cysts

Incidental pancreatic cysts are commonly encountered with some cysts having malignant potential. The most common pancreatic cystic neoplasms include serous cystadenoma, mucinous cystic neoplasm and intraductal papillary mucinous neoplasm. Risk stratifying pancreatic cysts is important in deciding whether patients may benefit from endoscopic ultrasound (EUS) or surgical resection. Surgery should be reserved for patients with malignant cysts or cysts at high risk for developing malignancy as suggested by various risk features including solid mass, nodule and dilated main pancreatic duct. EUS may supplement magnetic resonance imaging findings for cysts that remain indeterminate or have concerning features on imaging. Various cyst fluid markers including carcinoembryonic antigen, glucose, amylase, cytology, and DNA markers help distinguish mucinous from nonmucinous cysts. This review will guide the practicing gastroenterologist in how to evaluate incidental pancreatic cysts and when to consider referral for EUS or surgery. For presumed low risk cysts, surveillance strategies will be discussed. Managing pancreatic cysts requires an individualized approach that is directed by the various guidelines.     

Surgical decompression in the patients of pancreatic intraductal papillary mucinous neoplasm

Intraductal papillary mucinous neoplasm (IPMN) is characterized by cystic dilatation of the main and/or branch pancreatic duct by intraductal growth of mucin-producing columnar epithelia. The malignancy is determined by the degree of epithelial dysplasia. Because most IPMNs are slow growing and the prognosis may be favorable even when the IPMN is malignant, aggressive surgical treatment is suggested after considering operative and postoperative risks. Palliative surgery should be considered in some circumstances, such as other synchronous malignancy or systemic comorbidity. Here, we report two patients with IPMN treated successfully by surgical decompression of pancreatic duct.     

Positron emission tomography with 2-deoxy-2-[18f] fluoro-D-glucose in the detection of malignancy in intraductal papillary mucinous neoplasms of the pancreas

A 79-year-old Indian male was admitted with upper abdominal discomfort of 1-year duration which was associated with loss of weight and appetite. Serum tumor markers, including carcinoembryonic antigen and carbohydrate antigen 19-9, were within normal limits. A computed tomographic scan demonstrated a cystically dilated and tortuous pancreatic duct measuring 1.9 cm, suggestive of an intraductal papillary mucinous neoplasm. Fusion positron emission tomography/computed tomography with 2-deoxy-2-[18F] fluoro-D-glucose positron emission tomography (FDG-PET/CT) which was subsequently performed confirmed a metabolically active focus within the pancreatic head mass with a standard uptake value (SUVmax) of 3.5 compatible with carcinoma. A total pancreatectomy was performed and the final histology demonstrated a main-duct type intraductal papillary mucinous neoplasm with a focus of high-grade dysplasia compatible with carcinoma-in-situ. These images illustrate the emerging utility of FDG-PET/CT in the preoperative detection of malignancy in intraductal papillary mucinous neoplasm.     

ENDOSCOPIC DIAGNOSIS OF INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM USING PERORAL PANCREATOSCOPY WITH NARROW BAND IMAGING

A 77‐year‐old man was diagnosed with a pancreas cyst at another hospital. Abdominal ultrasonography revealed a cyst in the head of the pancreas and a small protrusion. These findings suggested intraductal papillary mucinous neoplasm. Cytologic finding of the pancreatic juice revealed a Class III lesion, and intraductal ultrasonography and peroral pancreatoscopy (PPS) were performed. An abnormal course of main pancreatic duct (MPD) prevented the insertion of an ultrasonography probe into the MPD, and PPS was performed. The mucosal surface of the MPD near the papillary area was normal, and narrow band imaging (NBI) clearly showed the vascular structure. When the PPS was inserted more deeply, many small protrusions were observed and NBI delineated the protrusions more clearly. Papillary protrusions were observed in the cyst, but NBI did not reveal any tumor vessels. These findings led to a diagnosis of benign intraductal papillary mucinous neoplasm lesion. Since then, follow‐up examinations have been made. Changes in the cyst and protrusion have not been observed.