Multifocal intraductal papillary mucinous neoplasm of the pancreas-A case report

Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas （IPMNs）. IPMN was first described in 1982. It has been most commonly described in 60 to 70 years old males, and represents a relatively ＂new＂ but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head （75%） while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-year- old male diagnosed with IPMN （carcinoma in situ） in the pancreatic head and a branch duct type IPMN （duct atypia） in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.     

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.     

Endoluminal ultrasonography for pancreatic diseases.

Endoluminal ultrasonography was performed on 146 patients with pancreatobiliary diseases by using high-frequency, thin ultrasonic probes, and the usefulness of the new technique in diagnosis of pancreatic diseases was reported. The ultrasound probe could be inserted into the main pancreatic duct in 43 of 46 patients (93.5%), and images of the lesions could be obtained in 42 patients (91.3%). Endoluminal ultrasonography revealed a hypoechoic mass with clear margins and central echogenicity in patients with pancreatic carcinoma. Endoluminal ultrasonography showed normal pancreatic parenchyma as a fine reticular pattern and did not reveal the tumors surrounding the stenosis in patients with focal pancreatitis. Endoluminal ultrasonography in patients with intraductal papillary adenocarcinoma of the pancreas revealed cystic lesions with mural nodules more than 4 mm, mucus echoes, and solid tumors with mixed echo patterns. There were no severe complications, and acute pancreatitis occurred in none of 46 patients, but high-level serum amylase after examination occurred in 5 patients (10.9%). Endoluminal ultrasonography is useful for differential diagnosis in patients with small pancreatic tumors or cystic lesions, especially intraductal papillary tumors of the pancreas. Endoluminal ultrasonography is recommended as a precise examination for the diagnosis of cystic lesions of the pancreas or stenosis of the main pancreatic duct after ERCP and EUS.     

A case of papillary tumor of the pancreas

The authors report a case of papillary cystic tumor which is a rare form of pancreatic tumor of exocrine origin. A young woman presenting with a painful abdominal mass was operated on for a tumor of the head of the pancreas. Gross and microscopic examination revealed a papillary cystic tumor of the pancreas. Tests for histologic markers found progesterone receptors. A review of the literature found approximately 100 cases of papillary cystic tumors of the pancreas. This type of tumor is seen mainly in young women presenting with an abdominal mass. After complete resection of the tumor, these patients usually make an uneventful recovery. Since regrowth of the tumor may occur these patients should be submitted to prolonged follow-up.     

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.     

Focal ductal branch dilatation on magnetic resonance cholangiopancreatography: a hint for early diagnosis of pancreatic carcinoma

A 63-year-old man with a combination of early pancreatic carcinoma and an intraductal papillary adenoma was reported. A pancreatic cyst was detected by chance at the head of the pancreas by computed tomography for a follow-up study of early rectal carcinoma previously operated. Detailed studies by endoscopic retrograde pancreatography (ERP) showed irregular narrowing of the main pancreatic duct at the pancreatic body and magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of ductal branches draining there. Brushing cytology of the pancreatic duct demonstrated cancer cells and total pancreatectomy was performed. Stepwise histo-pathological examinations of the specimen showed two foci of invasive carcinoma in the neck and body and multiple foci of severe dysplasia, some of which contained carcinoma in situ, in the body of the pancreas. The cystic tumor in the head of the pancreas was an intraductal papillary adenoma. In this case, the scrutiny of a pancreatic cyst including MRCP and ERP led to an early diagnosis of pancreatic cancer. Dilatation of ductal branches depicted by MRCP might be a new hint for early diagnosis of pancreatic carcinoma.     

Focal Ductal Branch Dilatation on Magnetic Resonance Cholangiopancreatography: A Hint for Early Diagnosis of Pancreatic Carcinoma

A 63-year-old man with a combination of early pancreatic carcinoma and an intraductal papillary adenoma was reported. A pancreatic cyst was detected by chance at the head of the pancreas by computed tomography for a follow-up study of early rectal carcinoma previously operated. Detailed studies by endoscopic retrograde pancreatography (ERP) showed irregular narrowing of the main pancreatic duct at the pancreatic body and magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of ductal branches draining there. Brushing cytology of the pancreatic duct demonstrated cancer cells and total pancreatectomy was performed. Stepwise histo-pathological examinations of the specimen showed two foci of invasive carcinoma in the neck and body and multiple foci of severe dysplasia, some of which contained carcinoma in situ, in the body of the pancreas. The cystic tumor in the head of the pancreas was an intraductal papillary adenoma. In this case, the scrutiny of a pancreatic cyst including MRCP and ERP led to an early diagnosis of pancreatic cancer. Dilatation of ductal branches depicted by MRCP might be a new hint for early diagnosis of pancreatic carcinoma.     

Localized type 1 autoimmune pancreatitis superimposed upon preexisting intraductal papillary mucinous neoplasms

A 70-year-old woman was found to have 2 cystic lesions in the head of the pancreas on abdominal ultrasonography during a routine medical examination.Endoscopic ultrasonography(EUS)and magnetic resonance cholangiopancreatography showed multilocular cysts in the head of the pancreas without dilation of the main pancreatic duct.The patient was followed-up semiannually with imaging studies for suspected branch duct-type intraductal papillary mucinous neoplasm(IPMN).At 3 years after initial presentation,hypoechoic lesions were observed around each pancreatic cyst by EUS.Diffusion-weighted imaging showed high-intensity regions corresponding to these lesions.Therefore,a diagnosis of invasive carcinoma derived from IPMN could not be excluded,and subtotal stomach-preserving pancreaticoduodenectomy was performed.The macroscopic examination of the surgical specimen showed whitish solid masses in the head of the pancreas,with multilocular cysts within each mass.Microscopically,each solid mass consisted of inflammatory cells such as lymphocytes and plasma cells.Furthermore,immunochemical staining revealed immunoglobulin G4-positive cells,and many obliterating phlebitides were observed.The cysts consisted of mucus-producing epithelial cells and showed a papillary growth pattern.Based on these findings,we diagnosed multiple localized type 1 autoimmune pancreatitis occurring only in the vicinity of the branch ducttype IPMN.     

Management of intraductal papillary mucinous neoplasm of the pancreas

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a distinct entity characterized by papillary proliferations of mucin-producing epithelial cells with excessive mucus production and cystic dilatation of the pancreatic ducts. IPMNs have malignant potential and exhibit a broad histologic spectrum, ranging from adenoma to invasive carcinoma. IPMNs are classified into main duct and branch duct types, based on the site of tumor involvement. IPMN patients have a favorable prognosis if appropriately treated. The postoperative 5-year survival rate is nearly 100% for benign tumors and noninvasive carcinoma, and approximately 60% for invasive carcinoma. A main duct type IPMN should be resected. Surgical treatment is indicated for a branch duct IPMN with suspected malignancy (tumor diameter ≥ 30 mm, mural nodules, dilated main pancreatic duct, or positive cytology) or positive symptoms. Malignant IPMNs necessitate lymph node dissection (D1). IPMNs are associated with a high incidence of extrapancreatic malignancies and pancreatic ductal carcinoma.     

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.     

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.     

Pancreatic cancer in patients with pancreatic cystic lesions: a prospective study in 197 patients.

BACKGROUND & AIMS: K-ras mutation is frequently detected in pancreatic juice of patients with pancreatic small cystic lesions, as well as those with pancreatic cancer. Those cystic lesions are often found by chance with modern radiologic imaging modalities. In this study, we prospectively examined the prognosis of patients with pancreatic cystic lesions, focusing on pancreatic cancer development. METHODS: A total of 197 patients with pancreatic cystic lesions, 80 with intraductal papillary mucinous neoplasm (IPMN) and 117 with non-IPMN cysts, were followed up for 3.8 years on average. Blood tests and imaging diagnosis were performed twice a year. The observed incidence of pancreatic cancer was compared with the expected incidence calculated on the basis of age- and gender-matched mortality of pancreatic cancer in the general Japanese population. RESULTS: Pancreatic cancer developed in 7 patients during the observation period (0.95% per year), infiltrating ductal carcinoma in 5 and intraductal papillary mucinous carcinoma in 2. Three of the ductal cancer cases had pancreatic non-IPMN cyst as preexisting lesion. At least 2 of the carcinomas arose in regions remote from preexisting lesions. The observed incidence of pancreatic cancer was 22.5 times higher (95% confidence interval, 11.0-45.3) than expected mortality from this cancer among general population. CONCLUSIONS: Patients with pancreatic cystic lesions are at a considerably high risk for pancreatic cancer, with a standardized incidence rate of 22.5. Cancer might develop in regions remote from the preexisting cystic lesion, suggesting diffuse pathologic changes predisposing to malignant transformation in the entire pancreas harboring cystic lesions.     

Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas

OBJECTIVE: Mucinous cystic neoplasms of the pancreas have a more favorable prognosis than ductal adenocarcinoma. Management of a subgroup, intraductal papillary-mucinous neoplasms, is controversial. Endoscopic ultrasound (EUS) with fine-needle aspiration biopsy may emerge as the imaging modality of choice. There are few studies describing the EUS features of these tumors. METHODS: A total of 35 consecutive cases of cystic tumors of the pancreas with an established pathological diagnosis were analyzed for characteristic EUS features. RESULTS: Mucinous cystadenocarcinomas (n = 14) were more likely to be characterized by hypoechoic cystic/solid mass or complex cyst and were frequently associated with a dilated main pancreatic duct. Benign mucinous duct ectasia (n = 6) were characterized by a dilated main pancreatic duct in conjunction with hyperechoic thickening of the duct wall. The two cases of intraductal mucinous hyperplasia additionally showed a hypoechoic mass. Intraductal papillary carcinoma (n = 11) had features in common with mucinous cystadenocarcinoma but also had echogenic foci in the mass and intraductal hyperechoic lesions. The two cases of microcystic cystadenoma showed either a mixed hypoechoic solid/cystic mass or a complex cyst without the additional features seen in mucinous cystadenocarcinoma. CONCLUSIONS: EUS features seem to exist that may help to differentiate cystic neoplasms from adenocarcinoma of the pancreas and, thus, to establish the preoperative diagnosis of cystic tumors of the pancreas.     

Adenosquamous carcinoma of the pancreas: report of two cases

Adenosquamous carcinoma of the pancreas is a rare variant of pancreatic exocrine carcinoma. We herein report two patients with this entity. One patient was a 60-yr-old Japanese man complaining of a palpable mass, 5.5 cm in the greatest diameter, in the epigastrium. Serum CA 19–9 was increased (2010 U/ml). Ultrasonography and computed tomography showed a mass in the pancreatic tail with central necrosis and invading the posterior wall of the stomach. Angiography showed an encasement of the splenic artery and complete obstruction of the splenic vein. Distal pancreatectomy, splenectomy, and partial resection of the stomach were done. The patient died of uncontrolled bleeding from the duodenal ulcer four months after operation. The other patient was a 73-yr-old man who presented with jaundice. The CA 19–9 was also elevated (354.8 U/ml). Ultrasonography showed a pancreatic head mass of heterogeneous echogeneity and computed tomography demonstrated a cystic mass with an enhanced rim, indicating necrosis in the tumor center. Angiography showed a hypervascular mass in the head of the pancreas. Pylorus-preserving pancreatoduodenectomy was done, but the patient died of multiple liver metastases 10 months after the operation. From our experience with the two patients, the presence of central necrosis in an infiltrative huge pancreatic tumor seems to be suggestive of the diagnosis of adenosquamous carcinoma of the pancreas.     

Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid pseudopapillary tumor of the pancreas： A case report and literature review

We describe the clinical, imaging and cytopathological features of solid pseudopapillary tumor of the pancreas (SPTP) diagnosed by endoscopic ultrasound- guided (EUS-guided) fine-needle aspiration (FNA). A 17-year-old woman was admitted to our hospital with complaints of an unexplained episodic abdominal pain for 2 mo and a short history of hypertension in the endocrinology clinic. Clinical laboratory examinations revealed polycystic ovary syndrome, splenomegaly and low serum amylase and carcinoembryonic antigen (CEA) levels. Computed tomography (CT) analysis revealed a mass of the pancreatic tail with solid and cystic consistency. EUS confirmed the mass, both in body and tail of the pancreas, with distinct borders, which caused dilation of the peripheral part of the pancreatic duct (major diameter 3.7 mm). The patient underwent EUS-FNA. EUS-FNA cytology specimens consisted of single cells and aggregates of uniform malignant cells, forming microadenoid structures, branching, papillary clusters with delicate fibrovascular cores and nuclear overlapping. Naked capillaries were also seen. The nuclei of malignant cells were round or oval, eccentric with fine granular chromatin, small nucleoli and nuclear grooves in some of them. The malignant cells were periodic acid Schiff (PAS)-Alcian blue ( ) and immunocytochemically they were vimentin ( ), CA 19.9 ( ), synaptophysin ( ), chromogranin (-), neuro-specific enolase (-), a1- antitrypsin and a1-antichymotrypsin focal positive. Cytologic findings were strongly suggestive of SPTP. Biopsy confirmed the above cytologic diagnosis. EUS- guided FNA diagnosis of SPTP is accurate. EUS findings,cytomorphologic features and immunostains of cell block help distinguish SPTP from pancreatic endocrine tumors, acinar cell carcinoma and papillary mucinous carcinoma.     

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.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Two cases of pancreatic cystadenocarcinoma with elevated CA 19-9 levels in the cystic fluid in comparison with two cases of pancreatic cystadenoma

A 39-year-old woman was hospitalized with left hypochondralgia. Ultrasonography and abdominal computed tomography showed a cystic mass in the tail of the pancreas. Echo-guided aspiration of the cyst was performed, and a markedly elevated CA 19-9 level in the cystic fluid was found. A surgical operation was performed, and the tumor was radically resected. The pathologic diagnosis was papillary adenocarcinoma of the pancreas. Another case of pancreatic cystadenocarcinoma also had an extremely high cystic CA 19-9 level, whereas two cases with benign pancreatic cysts had very low cystic CA 19-9 levels. Moreover, positive localization of CA 19-9 in the cancerous tissue was clearly demonstrated by an immunohistochemical technique, indicating active secretion of CA 19-9 into the cyst, while CA 19-9 in the tissue of pancreatic cystadenoma was almost non-existent. Measurement of the CA 19-9 level in the cystic fluid might therefore be a valuable additional aid in the diagnosis of cystadenocarcinoma of the pancreas.     

Diagnostic usefulness of laparoscopic fine‐needle aspiration for intraductal papillary tumor of the pancreas

A 67‐year‐old man who was followed up for 20 years for a diagnosis of chronic pancreatitis developed a unilocular cystic lesion in the pancreatic body and a gallstone. The cystic lesion (3.0 cm in diameter) was considered to be a pseudocyst with suspicion of a mucinous cystic tumor. Laparoscopic ultrasonography and fine‐needle aspiration (FNA) were performed following laparoscopic cholecystectomy. Under laparoscopic observation, the pinhole puncture was immediately closed. Analysis of the fluid revealed clusters of epithelial cells with mild atypia, remarkably elevated tumor markers (carcinoembryonic antigen and CA19‐9) and a K‐ras oncogene mutation. Distal pancreatectomy was performed 3 months after laparoscopic FNA and the pancreatic mass was diagnosed as an intraductal papillary tumor. The patient’s postoperative course was uneventful and he continues to do well without signs of recurrence. Laparoscopic FNA appears useful and safe for the diagnosis of cystic masses in the pancreas.     

Papillary cystic neoplasm of the pancreas: presentation and natural history in two cases.

Two young women, one white and one Chinese, with the rare but increasingly recognised papillary and cystic neoplasm of the pancreas are reported. The initial symptom in both was non-specific abdominal pain which, after investigation, was found to be caused by a pancreatic tumour. One patient did not come to surgery until five years after the initial diagnosis when she developed jaundice. In the five year interval between diagnosis and the development of jaundice computed tomography showed no change in the size (20 cm) of the pancreatic mass. Histology after resection, however, showed signs of lymphatic invasion. Cystic neoplasm of the pancreas can thus be regarded as an indolent, very slow growing tumour with potential for local invasion and hence metastatic spread.