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     

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.     

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.     

IDUS IN THE DIAGNOSIS OF IPMT

Intraductal papillary mucinous tumor of the pancreas (IPMT) is considered as having low‐grade malignant potential, and malignancy is not infrequent. Therefore, accurate diagnosis is indispensable for appropriate patient care. IPMT is classified mainly into two categories based on the distribution of tumor, the main duct type and the branch duct type. In the diagnosis of IPMT, there are four important points: differentiation of IPMT from other pancreatic pathologies; differentiation of malignancy from benign lesions; evaluation of tumor extent along the MPD; and investigation of duct cell carcinoma coexistent with or derived from IPMT. IPMT should be distinguished from chronic pancreatitis, ductal adenocarcinoma, or cystic neoplasms. IPMT often requires pancreaticoduodenectomy for cure, but such invasive surgery should be avoided in patients with benign hyperplasia. Mural nodules in the MPD can be clearly visualized by intraductal ultrasonography (IDUS). Measurement of the height of the tumor mass by IDUS is useful in distinguishing benign from malignant IPMT. Intraductal spread of IPMT along the MPD is demonstrated by IDUS as irregular thickening of the MPD wall. To achieve a tumor‐free margin in surgery, evaluation by IDUS of the extent of the tumor along the MPD is important. The expected accuracy of IDUS in the diagnosis of invasive IPMT is reportedly over 90%.     

Synchronous pancreatic serous cystic tumor, intraductal papillary mucinous tumor and gastric carcinoma: report of a case

Synchronous cystic tumors of the pancreas are rarely reported in the literature. We report an unusual case of synchronous pancreatic serous cystic tumor (SCT) and intraductal pancreatic mucinous tumor (IPMT) with concomitant gastric carcinoma. This study highlights the importance of careful intra-operative and pathologic examination for concomitant pancreatic neoplasms.     

Intraductal papillary-mucinous tumors of the pancreas: Clinicopathologic features, outcome, and nomenclature. Members of the Pancreas Clinic, and Pancreatic Surgeons of Mayo Clinic

BACKGROUND & AIMS: Intraductal papillary-mucinous tumor (IPMT) of the pancreatic ducts is increasingly recognized. This study investigated if clinical, imaging, or, histological features predicated outcome, formulated a treatment algorithm, and clarified relationships among IPMT, mucinous cystic neoplasms of the pancreas (MCN), and chronic pancreatitis. METHODS: The medical records, radiographs, and pathological specimens of 15 patients with IPMT (dilated main pancreatic duct or branch ducts with mucin overproduction) who were evaluated between October 1983 and January 1994 were reviewed. RESULTS: One patient had hepatic metastases. Fourteen underwent an operation (6 distal pancreatectomy, 4 total pancreatectomy, and 4 pancreaticoduodenectomy); all had dysplastic intraductal epithelium and chronic pancreatitis, whereas 3 had invasive adenocarcinoma. After a median of 25 months, 10 patients were alive; 3 of 4 with malignant and 2 of 11 with benign IPMT died (P < 0.05). Patients with or without carcinoma had similar clinical and radiographic features. A clinical diagnosis of chronic pancreatitis had been made in 9 patients with benign IMPT and in none with malignant IPMT (P < 0.05). CONCLUSIONS: IPMT is a dysplastic and likely precancerous lesion that is frequently diagnosed as chronic pancreatitis and is separate from MCN. Because it is not possible to distinguish noninvasive from invasive IPMT preoperatively, complete surgical excision of the dysplastic process is our treatment of choice whenever appropriate. (Gastroenterology 1996 Jun;110(6):1909-18)     

Differential diagnosis of intraductal papillary‐mucinous tumor of the pancreas by endoscopic ultrasonography and intraductal ultrasonography

Background: Intraductal papillary‐mucinous tumor (IPMT) of the pancreas has a broad spectrum of histology ranging from hyperplasia to adenocarcinoma. Therefore, it is important to differentiate between the malignant and benign lesions to determine the therapeutic strategy for IPMT. Patients and Methods: Thirty‐nine patients with IPMT (27 men and 12 women, mean age: 63.3 years) underwent surgery between January 1985 and March 2002. The size of the cystic lesion, the maximum diameter of the main pancreatic duct (MPD), and the height of the papillary tumor inside the cyst were investigated by endoscopic ultrasonography (EUS) and/or intraductal ultrasonography (IDUS) before operation. These preoperative clinical findings were compared with the pathological findings of the resected specimen. Results: The size of the cystic lesion, the diameter of MPD, and the height of the papillary tumor in cases with malignant IPMT (invasive and non‐invasive carcinoma) were larger than those in cases with benign IPMT (adenoma and hyperplasia). Analysis of the images of the lesions revealed that the following three factors are important for diagnosing IPMT: (i) the size of the cystic lesion is ≥ 30 mm; (ii) the diameter of MPD is ≥ 8 mm; (iii) the height of the papillary tumor inside the cyst is ≥ 3 mm. It was not significant to differentiate between benign and malignant IPMT based on factor (i), but statistically significant (P < 0.001) based on factors (ii) and (iii). Conclusions: EUS and IDUS are useful in the differential diagnosis of IPMT, especially in the differentiation between malignant and benign IPMT.     

Cystic neoplasm of the pancreas: a Japanese multiinstitutional study of intraductal papillary mucinous tumor and mucinous cystic tumor

The Japan Pancreas Society performed a multiinstitutional, retrospective study of 1379 cases of intraductal papillary mucinous tumor (IPMT) and 179 cases of mucinous cystic tumor (MCT) of the pancreas. Clinicopathologic features and postoperative long-term outcomes were investigated. IPMT were most frequently found in men and in the head of the pancreas. In contrast, all patients with MCT were women. Ovarian-type stroma were found in only 42.2% of the MCT cases. Prognostic indicators of malignant IPMT included advanced age, positive symptoms, abundant mucous secretion, presence of large nodules and/or large cysts, remarkable dilatation of the main pancreatic duct, and main duct- or combined-type IPMT. Advanced age, positive symptoms, and presence of large nodules and/or large cysts were predictive of malignant MCT. The 5-year survival rate of IPMT patients was 98%-100% in adenoma to noninvasive carcinoma cases, 89% in minimally invasive carcinoma cases, and 57.7% in invasive carcinoma cases. The 5-year survival rate of MCT patients was 100% in adenoma to minimally invasive carcinoma cases and 37.5% in invasive carcinoma cases. In conclusion, IPMT and MCT show distinct clinicopathologic and prognostic differences. The results from this study may contribute to the diagnosis and treatment of IPMT and MCT.     

Intraductal papillary mucinous tumors of the pancreas

Cystic neoplasms of the exocrine pancreas are a small fraction of pancreatic tumors. Within that group of cystic neoplasms, intraductal papillary mucinous tumors (IPMTs) can be distinguished from mucinous cystic neoplasms, serous cystic neoplasms, and pseudopapillary cystic tumors. Awareness of IPMTs has increased since the World Health Organization classified these tumors as its own group in 1996. Because of their favorable prognosis, an extensive diagnostic workup for IPMTs should be performed in patients presenting with cystic lesions of the pancreas. This workup often leads to the diagnosis and the predominant tumor location and size, although the extent of the ductal changes can only be established by histopathology. Surgical resection is the therapy of choice for IPMTs. The type of resection depends upon the extent of the quantitative and qualitative ductal involvement. Total pancreatectomy is currently the treatment for an IPMT that comprises the entire main duct.     

PERORAL PANCREATOSCOPY AND INTRADUCTAL ULTRASONOGRAPHY IN DETERMINING THE RESECTION LINES OF INTRADUCTAL PAPILLARY MUCINOUS TUMORS OF THE PANCREAS

In patients with malignant intraductal papillary–mucinous tumors (IPMT), the tumor spread along the main pancreatic duct is generally inspected as mural nodules within the ducts by peroral pancreatoscopy (POPS). Even the lower mural nodules including fish‐eggs‐like lesions and granular mucosa continued to the main taller mural nodules should be resected. Mural nodules along the main duct and cystic dilation of the branch ducts observed by intraductal ultrasonography (IDUS) are regarded as the tumor spread. Although POPS is superior to IDUS in identifying mural nodules along the main duct, POPS has limitations to detect mural nodules within the branch ducts. Therefore, the accuracy of the diagnosis by POPS is higher in main duct IPMT, whereas that by IDUS is higher in branch duct IPMT. These modalities should be performed as complementary tests in determining the resection lines preoperatively.     

Cytopathological Analysis of Cyst Fluid Enhances Diagnostic Accuracy of Mucinous Pancreatic Cystic Neoplasms

Widespread use of cross-sectional imaging and increasing age of the general population has increased the number of detected pancreatic cystic lesions. However, several pathological entities with a variety in malignant potential have to be discriminated to allow clinical decision making. Discrimination between mucinous pancreatic cystic neoplasms (PCNs) and nonmucinous pancreatic lesions is the primary step in the clinical work-up, as malignant transformation is mostly associated with mucinous PCN. We performed a retrospective analysis of all resected PCN in our tertiary center from 2000 to 2014, to evaluate preoperative diagnostic performance and the results of implementation of the consensus guidelines over time. This was followed by a prospective cohort study of patients with an undefined pancreatic cyst, where the added value of cytopathological mucin evaluation to carcinoembryonic antigen (CEA) in cyst fluid for the discrimination of mucinous PCN and nonmucinous cysts was investigated. Retrospective analysis showed 115 patients operated for a PCN, with a correct preoperative classification in 96.2% of the patients. High-grade dysplasia or invasive carcinoma was observed in only 32.3% of mucinous PCN. In our prospective cohort (n = 71), 57.7% of patients were classified as having a mucinous PCN. CEA ≥192 ng/mL had an accuracy of 63.4%, and cytopathological mucin evaluation an accuracy of 73.0%. Combining these 2 tests further improved diagnostic accuracy of a mucinous PCN to 76.8%. CEA level and mucin evaluation were not predictive of the degree of dysplasia. These findings show that adding cytopathology to cyst fluid biochemistry improves discrimination between mucinous PCN and nonmucinous cysts.     

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.     

胰腺导管内乳头状黏液瘤

胰腺导管内乳头状黏液瘤（IPMN）是由胰腺导管内产生黏液的上皮细胞呈乳头状增殖形成的肿瘤。与经典的胰腺癌相比,IPMN具有低度恶性、生长缓慢、少有侵犯周围组织、淋巴结转移率和再发率低的特点。IPMN根据肿瘤累及的部位可分为主胰管型、分支胰管型和混合型,病理组织特征涵盖从单纯腺瘤到浸润癌等多个亚型,临床表现多样,多种影像学检查手段可显示弥漫性或节段性扩张的主胰管和囊状扩张的分支胰管,ERCP经扩大的乳头获取黏液和胰液,取胰腺导管内皮组织和壁结节供活检均有助于诊断。IPMN确诊后应积极手术,手术切除率高,术后5年生存率高于一般的胰腺癌。本文就其临床表现、分类、病理特征、影像学诊断和治疗等方面做一综述。     

Two cases of pancreatic ductal adenocarcinoma, manifested as solid pseudopapillary tumor and intraductal papillary mucinous neoplasm]

Compared with other types of cancers, pancreatic cancer is one of the most dreadful malignancies and is fifth leading cause of cancer-related death in Korea. It is difficult to expect early diagnosis or improvement in prognosis due to lack of specific early symptoms and effective diagnostic methods. Whereas cystic neoplasm of the pancreas is a rare type of pancreatic tumor, surgical resection provides good prognosis because of its low possibility of local invasion or distant metastasis. In case of pancreatic cystic tumor, radiologic differentiation between benign and malignant lesions is crucial for the selection of appropriate treatment and the prediction of prognosis. And ductal adenocarcinoma of pancreas presenting in cystic form is an uncommon type of cystic tumor, making it extremely rare among all pancreatic malignancies. We report two cases of atypical pancreatic ductal adenocarcinoma presenting as solid pseudopapillary tumor and intraductal papillary mucinous neoplasm, respectively.     

Characterisation of oestrogen receptor,progesterone receptor,trefoil factor 1, and epidermal growth factor and its receptor in pancreatic cystic neoplasms and pancreatic ductal adenocarcinoma

BACKGROUND AND AIMS: The pancreatic cystic neoplasms, including solid pseudopapillary tumour (SPT), mucinous cystic neoplasm (MCN), and intraductal papillary mucin producing tumour (IPMT), have their characteristic clinicopathological features. A systematic investigation of oestrogen receptor (OR), progesterone receptor (PR), trefoil factor 1(TFF1), and epidermal growth factor and its receptor (EGF and EGFR) expressed in pancreatic cystic neoplasms and pancreatic ductal adenocarcinoma was determined to elucidate their corresponding sex and age predilection, cell origin, and pathway of malignant transformation. METHODS: Surgical specimens of SPT (n=10), MCN (n=12), IPMT (n=10), and ductal adenocarcinoma (n=20) were studied. The expression of OR, PR, TFF1, EGF, and EGFR were each determined in each disease entity using monoclonal antibodies by immunohistochemical method. The results were correlated with the clinicopathological data. RESULTS: PR was expressed in all 10 SPT, whereas OR was expressed in none of 10 SPT. TFF1 was not or weakly expressed in SPT. Although EGF was strongly expressed in seven of 10 SPT, synchronous expression of EGF and its receptor was expressed in none of 10 SPT. Of the 12 MCN, six had PR expression in the stroma cells but not in the neoplastic epithelium, seven had a moderate or strong expression of TFF1, and 10 had no or weak EGFR expression, irrespective of their benigneity or malignancy. Synchronous expression of EGF and EGFR was observed in only one of 12 MCN. Among 10 IPMT, TFF1 and EGFR were moderately or strongly expressed in all six malignancies, whereas TFF1 and EGFR were not or weakly expressed in three of four benigneity. Of 20 ductal adenocarcinomas, TFF1 and EGFR were moderately or strongly expressed in 16 and 12, respectively. Synchronous expression of EGF and EGFR was observed in six of 10 IPMT and nine of 20 ductal adenocarcinoma, respectively. CONCLUSION: PR was uniquely expressed in SPT, and OR and PR were expressed in stroma of MCN, reflecting their sex and age predilection. TFF1 expression was related to EGFR such as in IPMT and ductal adenocarcinoma, not related to EGFR such as in MCN, and not related to hormonal receptors such as in SPT. EGF and its receptor might play a part in the malignant transformation of IPMT and ductal adenocarcinoma, but not of SPT and MCN.     

EUS FNA in intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: There is little information concerning the potential role of fine-needle aspiration guided by endoscopic ultrasonography in the pathologic diagnosis of intraductal papillary mucinous tumors of the pancreas. METHODOLOGY: Patients with an intraductal papillary mucinous tumor of the pancreas suggested by endoscopic ultrasonography underwent fine-needle aspiration guided by endoscopic ultrasonography in order to investigate the presence of mucin and/or cytologic changes consistent with this diagnosis. A group of 111 patients with other pancreatic lesions explored during the same period of time was used as a control group. RESULTS: Fine-needle aspiration guided by endoscopic ultrasonography was safely performed in 19 patients and supported the diagnosis in 17 of them. Nine out of the 17 patients with suspicion of intraductal papillary mucinous tumors of the pancreas went to surgery and this diagnosis was confirmed in the resected specimen in all of them. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of EUS FNA in the diagnosis of IPMT were 82%, 100%, 100%, 92% and 94% respectively. CONCLUSIONS: Fine-needle aspiration guided by endoscopic ultrasonography is a good technique to support the diagnosis of intraductal papillary mucinous tumors of the pancreas and should be considered in this group of patients if pathologic confirmation is judged to be necessary.     

Clinicopathologic study of intraductal papillary-mucinous tumors and mucinous cystic tumors of the pancreas

BACKGROUND/AIMS: We analyzed clinicopathologic and imaging findings of intraductal papillary-mucinous tumors (IPMTs) and mucinous cystic tumors (MCTs) of the pancreas to evaluate the difference between IPMTs and MCTs, and to identify the signs indicative of malignancy in IPMTs. METHODOLOGY: Clinicopathological features of 20 patients with IPMT and six patients with MCT of the pancreas were studied. RESULTS: The patients with IPMT comprised 16 males and four females with a mean age of 62.9 years. Eighty percent of IPMTs were located in the pancreatic head, and the mean tumor size was 38.6mm. Recurrence was observed in one patient, who died of IPM adenocarcinoma. In contrast, all patients with MCT were females, with a mean age of 53.0 years. None of the MCTs arose in the pancreatic head, and the mean tumor size was 42.7mm. One patient died of MC adenocarcinoma, but all of the others survived without recurrence. The difference in gender, location of the tumor, and connection to the pancreatic duct reached statistical significance between IPMTs and MCTs. A significant connection to the pancreatic duct and high level of serum carbohydrate antigen 19-9 (CA19-9) was observed in the adenocarcinoma and moderate dysplasia groups of IPMT. CONCLUSIONS: The main duct type and an elevation of serum CA19-9 level suggested malignancy in IPMTs.     

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。     

EUS in the evaluation of pancreatic cystic lesions

BACKGROUND: The differential diagnosis in pancreatic cystic lesions is often difficult despite the availability of various modern imaging modalities. This study assessed the role of EUS in the following: (1) discrimination of pseudocysts from pancreatic cystic tumors, (2) differential diagnosis between serous cystadenoma and mucinous cystic tumor, and (3) prediction of accompanying malignancy in intraductal papillary mucinous tumor. METHODS: EUS findings in 75 patients with pancreatic cystic lesions (58 cystic tumors, 17 pseudocysts) were evaluated. In the comparison of pseudocysts and cystic tumors, the latter included intraductal papillary mucinous tumor, mucinous cystic tumors, and serous cystadenomas, but not solid-pseudopapillary tumors. RESULTS: In univariate analysis, pseudocysts exhibited echogenic debris and parenchymal changes more often than cystic tumors did (respectively, 29% vs. 6%, p < 0.05; and 65% vs. 4%, p < 0.001). In contrast, septa and mural nodules were found more frequently in cystic tumors than pseudocysts (respectively, 69% vs. 12%, p < 0.001; 56% vs. 12%, p < 0.01). Multivariate analysis revealed that parenchymal changes (odds ratio [OR] = 83.59; p < 0.01); septa (OR = 30.75; p < 0.05); and mural nodules (OR = 21.38; p < 0.05) were independent predictors of differentiation between pseudocysts and cystic tumors. Serous cystadenoma exhibited diverse EUS features, as well as a honeycomb appearance. Mural nodules were found more often in mucinous cystic tumors than in serous cystadenomas (p < 0.05). There were no factors that predicted malignancy in intraductal papillary mucinous tumor. CONCLUSIONS: EUS is a useful complementary imaging method for differentiation of pancreatic cystic lesions.