Intraductal mucinous tumors occurring simultaneously in the liver and pancreas

A case of simultaneous intraductal mucinous tumors of the liver and pancreas in a 67-year-old man is described. Abdominal ultrasonography and computed tomography (CT) revealed the presence of cystic lesions with intraluminal septae both in the caudate lobe of the liver and in the uncinate process of the pancreas; these cystic lesions communicated with the hepatic duct and pancreatic duct, respectively. Mucin retention was observed in the cysts, and cholestasis was induced by mucin secretion into the common bile duct. The lesions were resected by left hepatic lobectomy with caudate lobectomy, and segmental pancreatectomy. Both lesions were multilocular cystic tumors with no papillary projections or focal mass effect in their walls. Histologically, both cystic lesions were a mixture of hyperplasia and adenoma lined by low papillary columnar epithelium. There were no cellular or histological features to suggest malignant change. The fibrous intratumor interstitium lacked any mesenchymal or ovarian-like stroma. The hepatic lesion was considered to be of a similar nature to intraductal papillary mucinous tumor (IPMT) of the pancreas. However, the two lesions occurred simultaneously in the liver and pancreas. This case is of interest in regard to the diagnosis and management of mucinous hepatopancreatobiliary lesions. Received: March 16, 2001 / Accepted: September 14, 2001     

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.     

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.     

Biliary cystadenocarcinoma followed up as benign cystadenoma for 10 years

We describe a case of biliary cystadenocarcinoma that showed a longterm clinical course. A 69-year-old Japanese man was admitted to our hospital because of abdominal discomfort. The patient had been diagnosed with benign cystadenoma of the liver at another hospital in 1987 and had been followed up for 10 years. Abdominal ultrasonography (US) and computed tomography (CT) scan demonstrated a unilocular cystic lesion, which included multiple conspicuous papillary protrusions in the left hepatic lobe. Left lateral segmentectomy was performed, and intraoperative cholangiography revealed a communication between the cystic tumor and intrahepatic bile duct. The tumor contained clear mucinous fluid and enfolded multiple yellowish papillary projections on the cystic wall. Histological examination showed the tumor to be biliary cystadenocarcinoma. The patient is doing well 4 years after the operation.     

Risk of malignancy in serous cystic neoplasms of the pancreas

BACKGROUND: In contrast to mucinous cystic neoplasms of the pancreas, which are known to have considerable malignant potential, the serous variant is generally thought to be benign. There are, however, several reports of malignancy in serous cystic neoplasms of the pancreas. AIMS: To assess the risk of malignancy of serous cystic tumors of the pancreas and to investigate specific clinical and histological features. METHODS: Clinical and pathological characteristics of benign and malignant serous cystic neoplasms of the pancreas were investigated by a review of the literature and documented by a case of a serous cystadenocarcinoma and immunohistochemical analysis of a series of serous cystadenomas. Reviewing the literature prevalence, age and sex distribution of serous cystic neoplasms were analyzed. RESULTS: The prevalence of cancer among serous cystic neoplasms reported since 1989 was 3%. Serous cystadenoma of the pancreas present at an earlier age (61 years) than serous cystadenocarcinoma (66 years; p = 0.056) and are symptomatic in the majority of patients.Pathological examination of the primary tumor was not able to distinguish cystadenoma from cystadenocarcinoma in 38% of cases. In 25% the diagnosis of cancer was established only after growth of metachronous metastases. In the present case, nuclear atypia, papillary structures, proliferation marker Ki-67 and p53 protein were increased in the primary tumor and/or metachronous metastasis. CONCLUSION: Serous cystic neoplasms of the pancreas do have malignant potential with a risk of malignancy of 3% and should be surgically treated if the operative risk is acceptable. Routine analysis of genetic and proliferation markers may improve diagnosis of malignancy in these tumors.     

Image-diagnostic features of mature cystic teratomas of the pancreas: report on two cases difficult to diagnose preoperatively

This report documents the findings of two rare cases of mature cystic teratoma of the pancreas. Although they could not be diagnosed preoperatively, our retrospective report suggests that the combined diagnosis of ultrasonography (US), enhanced computed tomography (CT), and magnetic resonance cholangiopancreatography (MRCP) might allow differentiation from other cystic lesions such as mucinous cystic tumors (MCTs) and intraductal papillary–mucinous tumors (IPMTs). Since the cystic teratomas were both filled with keratinous and sebaceous material, they were echogenic, appearing as solid masses on US. Enhanced CT showed their cystic nature, with values slightly higher than water, and MRCP revealed defects of internal signals.     

Serous cystic neoplasm of the pancreas-indications for surgery

BACKGROUND/AIMS: Serous cystic neoplasm is a rare pancreatic tumor. Almost all of these tumors are benign and only 9 cases of serous cystadenocarcinoma have been reported. Although serous cystic neoplasm is typically a microcystic lesion, there is a wide range of cyst sizes from micro to macro and even unilocular cysts have been reported. Thus, the diagnosis is difficult and indications for surgery are controversial. We aimed to elucidate the clinicopathological and imaging features of serous cystic neoplasm of the pancreas. METHODOLOGY: We investigated 15 cases of resected and 6 cases of nonresected cases of serous cystic neoplasm, evaluating the symptoms, imaging findings, preoperative diagnosis, macroscopic morphology, microscopic findings, and results of follow-up. RESULTS: Imaging diagnosis of serous cystic neoplasm was not easy, because not so many tumors had the typical microcystic pattern. Most of the resected serous cystic neoplasms were non-microcystic or were small tumors, which could not be precisely evaluated. CONCLUSIONS: Small serous cystic neoplasms, which can be diagnosed by imaging, do not need to be resected because serous cystadenocarcinoma is rare. Tumors of the pancreas that cannot be confirmed to be serous cystic neoplasm should be resected because of the possibility of pancreatic cancer, mucinous cystadenocarcinoma, or mucinous cystadenoma with malignant potential.     

p53 protein expression in intraductal papillary mucinous tumors (IPMT) of the pancreas as an indicator of tumor malignancy

BACKGROUND/AIMS: Intraductal papillary mucinous tumors, as a cystic disease in the pancreas, clinically has a more indolent and favorable course than invasive ductal pancreas carcinoma. However, some cases of intraductal papillary mucinous tumors show invasive and rapid progression like ductal pancreas carcinoma and the prognosis of such patients is sometimes poor. In the current study, we carried out immunohistochemical staining of intraductal papillary mucinous tumor tissues for p53 and investigated whether positive staining indicates tumor malignancies and has a prognostic value for intraductal papillary mucinous tumors. METHODOLOGY: Nineteen (19) patients who underwent pancreatic resection under the diagnosis of intraductal papillary mucinous tumors at the Chiba University Hospital between April 1992 and December 1996 were studied. We performed immunohistochemical staining of p53 as well as of PCNA, Ki-67 and Bcl-2 using their respective antibodies. Pathological findings revealed that 9 cases were intraductal papillary adenoma, 9 were intraductal papillary adenocarcinoma, and one was invasive ductal papillary adenocarcinoma. RESULTS: p53 expression could only be detected in the 1 case with invasive ductal papillary adenocarcinoma. Significant association could not be found between histological features and immunohistochemical staining of PCNA, Ki-67 and Bcl-2. CONCLUSIONS: p53 protein expression could be detected after progression to invasive type of intraductal papillary mucinous tumors. The present results demonstrate that p53 expression might be an indicator of invasive progression in intraductal papillary mucinous tumors, and might represent a surgical indicator of intraductal papillary mucinous tumors.     

Operative indications for cystic lesions of the pancreas with malignant potential--our experience

BACKGROUND/AIMS: There are still many important but unclear points regarding the differential diagnosis and operative indications of cystic lesions of the pancreas with malignant potential. Studies of the clinicopathological and molecular biological characteristics of such diseases are necessary. In this paper, we discuss operative indications for this condition based on a review of the literature and our own experience. METHODOLOGY: Seven cases of serous cystadenoma and 9 cases of mucinous cystadenoma or cystadenocarcinoma of the pancreas that were operated on or autopsied in our department from 1980 to 1996 were analyzed clinicopathologically. Small cystic lesions incidentally found in 300 autopsied cases were also studied. Finally, mucin-producing tumors described in several reports were reviewed, and the branch type of this tumor was especially investigated. RESULTS: A marked disappearance of pancreatic acini in the upstream pancreas was found when serous cystadenoma became large. Papillary projection was histologically found in all of the cases. Tumorous invasion to the interstitium was suspected in tumors more than 5 cm in diameter, and malignancy was reported when tumors were larger than 6 cm. As for mucinous cystadenocarcinoma, the patients had a poor prognosis. In 2 of 42 cases with a pseudocyst, small duct cell carcinoma was incidentally found adjacent to the pseudocyst on the duodenal side. With regard to branch-type intraductal papillary neoplasm, 80% of the tumors larger than 4 cm were malignant. Most of the small cystic lesions found in elderly autopsy cases were accompanied by hyperplastic epithelia without evidence of malignancy. CONCLUSIONS: Based on our experience, an operation should be considered and resection is recommended under the following circumstances: 1) cystic lesions in the body and tail of the pancreas in middle-aged women; 2) typical serous cystadenoma larger than 4 cm; 3) mucinous cystadenoma of any size; 4) branch-type intraductal papillary neoplasm larger than about 3 cm; and, 5) pseudocysts of unknown cause. Small cystic lesions in elderly patients should not necessarily be operated on, but should be followed-up carefully.     

胰腺黏液性囊腺瘤的诊断和外科治疗

目的 探讨胰腺黏液性囊腺瘤(MCN)的诊断和外科治疗效果.方法 回顾性分析2003年1月至2008年6月问在青岛大学医学院附属医院手术治疗的20例MCN患者的临床表现、病理特征、治疗方法和生存率等临床和随访资料.结果 患者以腹痛、上腹饱胀不适、恶心、呕吐为主要临床表现,11例有上腹部压痛,6例可触及上腹肿块.术前均行B超及CT检查,诊断13例为良性MCN,4例为胰腺浆液性囊腺瘤,3例为黏液性囊腺癌.肿瘤主要位于胰腺体、尾部,直径4～14 cm.20例均行手术治疗,以胰十二指肠切除术、胰体尾切除术为主.术后病理证实,良性MCN 10例,交界性MCN 3例,黏液性囊腺癌7例.术后平均随访26个月,良性MCN和交界性MCN患者均健在,无复发;黏液性囊腺癌患者3年生存率50%.结论 MCN主要好发于女性,临床表现无特征性,术前B超和CT检查可进行诊断,手术切除是惟一有效的治疗方法,预后较好.     

The prognosis of intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: Intraductal papillary mucinous tumors of the pancreas have been recognized as a distinct clinical entity. However, their biological behavior has not been clearly defined. The aim of this study was to examine the prognosis of this tumor, to clarify the biological behavior and determine the most appropriate treatment. METHODOLOGY: Correlations between prognosis of operated cases and histopathologic features were investigated. RESULTS: In 105 patients with characteristic clinical features of intraductal papillary mucinous tumors, the lesions were classified as hyperplasias in 21%, intraductal tumors in 48% and invasive carcinomas in 31%. Minimal invasion was apparent in 25%, lymph node metastasis in 21%, and fistula formation in 31% of the invasive lesions. Non-invasive and minimally invasive intraductal papillary mucinous tumors were essentially free from risk of tumor recurrence. Other invasive intraductal papillary mucinous tumors showed a significantly poor prognosis. CONCLUSIONS: Because of the variation in pathological characteristics, patient outcome and the possibility of differential diagnosis, the treatment might be recommended as follows: the case of hyperplasia can be followed-up with close surveillance. Non-invasive and minimally invasive intraductal papillary mucinous tumors should be operated with function-preserving minimal pancreatectomy. For patients with invasive intraductal papillary mucinous tumors evident with preoperative imaging modalities, radical operations with lymph node dissection might be needed.     

Magnetic resonance imaging in the detection of pancreatic neoplasms

Recently, with the rapid scanning time and improved image quality, outstanding advances in magnetic resonance (MR) methods have resulted in an increase in the use of MRI for patients with a variety of pancreatic neoplasms. MR multi-imaging protocol, which includes MR cross-sectional imaging, MR cholangiopancreatography and dynamic contrast-enhanced MR angiography, integrates the advantages of various special imaging techniques. The non-invasive all-in-one MR multi-imaging techniques may provide the comprehensive information needed for the preoperative diagnosis and evaluation of pancreatic neoplasms. Pancreatic neoplasms include primary tumors and pancreatic metastases. Primary tumors of the pancreas may be mainly classified as ductal adenocarcinomas, cystic tumors and islet cell tumors (ICT). Pancreatic adenocarcinomas can be diagnosed in a MRI study depending on direct evidence or both direct and indirect evidence. The combined MRI features of a focal pancreatic mass, pancreatic duct dilatation and parenchymal atrophy are highly suggestive of a ductal adenocarcinoma. Most cystic neoplasms of the pancreas are either microcystic adenomas or mucinous cystic neoplasms. Intraductal papillary mucinous tumors are the uncommon low-grade malignancy of the pancreatic duct. ICT are rare neoplasms arising from neuroendocrine cells in the pancreas or the periampullary region. ICT are classified as functioning and non-functioning. The most frequent tumors to metastasize to the pancreas are cancers of the breast, lung, kidney and melanoma. The majority of metastases present as large solitary masses with well-defined margins.     

Diagnostic dilemmas in patients with cystic neoplasms of the pancreas

Cystic neoplasms of the pancreas (CNP) are rare lesions that can be difficult to diagnose preoperatively. Twenty patients with cystic neoplasms of the pancreas including five microcystic adenomas, six benign mucinous cystic neoplasms, three malignant mucinous cystic neoplasms, two solid and papillary epithelial neoplasms, and four cystic neuroendocrine tumors were treated at a single institution between 1962 and 1987. The average duration of symptoms prior to diagnosis was 10 months. Five patients were asymptomatic. Forty percent of patients presented with an abdominal mass. Plain abdominal x-rays and UGI barium contrast studies were never diagnostic. Ultrasonography, computerized tomography (CT) and visceral angiography aided in the correct diagnosis in 28%, 36%, and 75% of patients studied, respectively. Overall a correct diagnosis was made preoperatively in only 35% of patients. Twelve of 13 patients were correctly diagnosed at laparotomy with intraoperative biopsy. Without biopsy the mass was misdiagnosed at laparotomy in five of six cases. CNP must be suspected in any patients who present with an upper abdominal mass with or without abdominal pain and no history of pancreatitis. CT may be diagnostic in up to one third of cases and should be obtained routinely to demonstrate the proximity of the lesion to other structures. Visceral angiography should also be obtained prior to operation. A generous incisional biopsy should be obtained of all pancreatic cysts that are not to be resected.     

Diagnostic dilemmas in patients with cystic neoplasms of the pancreas

Summary Cystic neoplasms of the pancreas (CNP) are rare lesions that can be difficult to diagnose preoperatively. Twenty patients with cystic neoplasms of the pancreas including five microcystic adenomas, six benign mucinous cystic neoplasms, three malignant mucinous cystic neoplasms, two solid and papillary epithelial neoplasms, and four cystic neuroendocrine tumors were treated at a single institution between 1962 and 1987. The average duration of symptoms prior to diagnosis was 10 months. Five patients were asymptomatic. Forty percent of patients presented with an abdominal mass. Plain abdominal x-rays and UGI barium contrast studies were never diagnostic. Ultrasonography, computerized tomography (CT) and visceral angiography aided in the correct diagnosis in 28%, 36%, and 75% of patients studied, respectively. Overall a correct diagnosis was made preoperatively in only 35% of patients. Twelve of 13 patients were correctly diagnosed at laparotomy with intraoperative biopsy. Without biopsy the mass was misdiagnosed at laparotomy in five of six cases. CNP must be suspected inany patients who present with an upper abdominal mass with or without abdominal pain and no history of pancreatitis. CT may be diagnostic in up to one third of cases and should be obtained routinely to demonstrate the proximity of the lesion to other structures. Visceral angiography should also be obtained prior to operation. A generous incisional biopsy should be obtained of all pancreatic cysts that are not to be resected.     

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.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

胰腺囊性肿瘤19例的诊断与外科治疗

目的 探讨胰腺囊性肿瘤的诊断及外科治疗方法.方法 对我院普外科2000年1月至2009年8月诊治的19例胰腺囊性肿瘤的临床资料进行回顾性分析.结果 胰腺囊性肿瘤无特征性临床表现,B超和CT是其主要诊断手段,但均不能准确区分其病理类型,与术后病理对照的定性诊断符合率分别为57.9%(11/19)和68.4%(13/19).肿瘤位于胰头颈部5例,胰体尾部14例,最大直径3～15cm.19例均行手术治疗,切除肿瘤16例,总切除率为84.2%.术中误诊误治4例(21.0%).病理证实浆液性囊腺瘤6例,黏液性囊腺瘤6例,黏液性囊腺癌5例,导管内乳头状黏液腺瘤2例.获得随访15例(78.9%),3例囊腺癌患者中1例切除者已存活4年,无复发;2例未切除者分别于术后4个月和7个月病死.12例囊腺瘤患者目前均存活,肿瘤无复发.失访4例,囊腺癌和囊腺瘤各2例.结论 加强对胰腺囊性肿瘤的认识是减少误诊误治的关键;胰腺囊性肿瘤手术切除后疗效满意,故一经诊断即应积极行外科手术切除.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas.

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

Cystic ductal adenocarcinoma of pancreas: an unusual variant.

Cystic lesions of the pancreas are usually pseudocysts (90%); only 10% of them are cystic tumors. These cystic tumors constitute less than 10% of all pancreatic neoplasms, making them an extremely uncommon type of pancreatic malignancy. What is more important is that these tumors are very easily misdiagnosed as pseudocysts because their characteristics are very similar to those of the benign pseudocysts. This gains importance as the cystic tumors have a high cure rate and good prognosis if diagnosed and treated on time. Of all the cystic tumors, the most common are the benign serous cystadenomas, mucinous cystic tumors, intraductal papillary mucinous neoplasms etc. Ductal adenocarcinoma of pancreas presenting in cystic form is an uncommon type of cystic tumor, making it extremely rare among all pancreatic malignancies (solid or cystic). We present the following case report. The review of literature concerning the diagnosis and management has also been discussed.     

Clinicopathologic review of 41 cases of pancreatic mucinous cystic neoplasms]

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.