An aggressive surgical approach is warranted in the management of cystic pancreatic neoplasms

BACKGROUND: Cystic pancreatic neoplasms encompass a range of benign to malignant disease. Recommendations for surgical management vary. METHODS: Records of patients with cystic pancreatic neoplasms from January 1996 through December 2005 were retrospectively reviewed. RESULTS: Sixty resections were performed for 16 serous cystic neoplasms, 7 mucinous cystic neoplasms (MCNs), and 37 intraductal papillary mucinous neoplasms (IPMNs). Twenty-five percent (15/60) of neoplasms contained invasive cancer. Patients with MCN or IPMN invasive neoplasms experienced significantly diminished overall 5-year survival compared to patients with IPMN carcinoma in situ neoplasms and to patients with MCN or IPMN adenoma/borderline neoplasms (22% vs. 73% vs. 94%, P = .004). CONCLUSIONS: Given the poor long-term survival of patients with cystic pancreatic neoplasms containing invasive cancer and the current difficulty to preoperatively distinguish among the various types of lesions in a reliable manner, our data support an aggressive surgical approach to the management of cystic pancreatic neoplasms.     

Current understanding of precursors to pancreatic cancer

Precursors to pancreatic cancer have been investigated for a century. Previous studies have revealed three distinct precursors, i.e. mucinous cystic neoplasm (MCN), intraductal papillary mucinous neoplasm (IPMN), and pancreatic intraepithelial neoplasia (PanIN), harboring identical or similar genetic alterations as does invasive pancreatic carcinoma. The current understanding of precursors to pancreatic cancer can be illustrated by progressive pathways from noninvasive MCN, IPMN, and PanIN toward invasive carcinoma. MCNs consist of ovarian-type stroma and epithelial lining with varying grades of atypia, and are occasionally associated with invasive adenocarcinoma. The epithelium of noninvasive IPMNs shows a variety of different directions of differentiation, including gastric, intestinal, pancreatobiliary (PB), and oncocytic types. IPMNs can also harbor varying grades of architectural and cytologic atypia. IPMNs confined to branch ducts are mostly the gastric type, and IPMNs involving the main ducts are often intestinal type, while PB and oncocytic types are rare. Small (<1 cm) IPMNs of the gastric type are not always morphologically distinguishable from low-grade PanINs. Mucin expression profiles suggest intestinal-type IPMNs progress to mucinous noncystic (colloid) carcinoma, while PB-type IPMNs progress toward ductal adenocarcinoma. It is a well-described paradigm that PanIN lesions progress toward ductal adenocarcinoma through step-wise genetic alterations. The activation of Hedgehog and Notch signaling pathways in PanIN lesions as well as in pancreatic adenocarcinoma suggest that developmental pathways may be disregulated during carcinogenesis of the pancreas. Further study is needed to elucidate the pathways from precursors toward invasive carcinoma of the pancreas.     

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??Diagnosis and treatment of cystic neoplasms of pancreas: retrospective analysis of 212 cases WANG Dan-song??JIN Da-yong, KUANG Tian-tao, et al. Zhongshan Hospital of Fudan University, General Surgery Research Institute of Fudan University, Shanghai200032, China
Corresponding author??LOU Wen-hui,E-mail: wenhuilou@aliyun.com
Abstract Objective To investigate the diagnosis and treatment of cystic neoplasms of the pancreas. Methods Retrospective analysis was made on the clinical data of 212 cases of pancreatic cystic neoplasms from January 2000 to December 2010 in Zhongshan Hospital. Results There were 101 cases of IPMNs(48%), 49 cases of MCNs(23%) and 62 cases of SCNs(29%) . No special clinical manifestation was observed. 90%, 93% and 94% accuracy can be achieved by ultrasound, CT and MRI on the diagnosis of cystic tumor respectively. The postoperative incidence of pancreatic fistula is 34.3%. All the cases of SCN, non-invasive MCN and IPMN survived five years or longer, while the five-year survival rate of invasive MCNs and IPMNs is just 31% and 35% respectively. Conclusion Both CT and MRI are accurate on preoperative diagnosis of pancreatic cystic tumors. Priority should be given to function-saving-operation for SCN, non-invasive MCN and IPMN cases, while the invasive PNCs should receive standard pancreatectomy plus regional lymph nodes dissection. The overall prognosis of pancreatic cystic tumor is satisfactory.     

Intraductal papillary mucinous neoplasms of the pancreas: an increasingly recognized clinicopathologic entity

OBJECTIVE: To assess the authors' experience with intraductal papillary mucinous neoplasms of the pancreas (IPMNs). SUMMARY BACKGROUND DATA: Intraductal papillary mucinous neoplasms of the pancreas are being recognized with increasing frequency. METHODS: All patients who underwent pancreatic resection for an IPMN at the Johns Hopkins Hospital between January 1987 and December 2000 were studied. The data were compared with those of 702 concurrent patients with infiltrating ductal adenocarcinoma of the pancreas not associated with an IPMN resected by pancreaticoduodenectomy. RESULTS: In the 13-year time period, 60 patients underwent pancreatic resection for IPMNs, with 40 patients undergoing resection in the past 3 years. Mean age at presentation was 67.4 +/- 1.4 years. The most common presenting symptom in patients with IPMNs was abdominal pain (59%). Most IPMNs were in the head of the pancreas or diffusely involved the gland, with 70% being resected via pancreaticoduodenectomy, 22% via total pancreatectomy, and 8% via distal pancreatectomy. Twenty-two patients (37%) had IPMNs with an associated infiltrating adenocarcinoma. In a subset of IPMNs immunohistochemically stained for the Dpc4 protein (n = 50), all of the intraductal or noninvasive components strongly expressed Dpc4, whereas 84% of associated infiltrating cancers expressed Dpc4. The 5-year survival rate for all patients with IPMNs (n = 60) was 57%. CONCLUSION: Intraductal papillary mucinous neoplasms represent a distinct clinicopathologic entity being recognized with increasing frequency. IPMNs are clinically, histologically, and genetically disparate from pancreatic ductal adenocarcinomas. The distinct clinical features, the presumably long in situ or noninvasive phase, and the good long-term survival of patients with IPMNs offer a unique opportunity for early diagnosis, curative resection, and further studies of the molecular genetics and natural history of these unusual neoplasms.     

Cystic Neoplasia of the Pancreas: Pathology and Biology

In contrast with solid tumors, most of which are invasive ductal adenocarcinoma with dismal prognosis, cystic lesions of the pancreas are often either benign or low-grade indolent neoplasia. Those that are mucinous, namely, intraductal papillary mucinous neoplasms (IPMNs) and mucinous cystic neoplasms (MCNs), constitute the most important category, not only because they are the most common, but more importantly because they have well-established malignant potential, representing an adenomacarcinoma sequence. While many are innocuous adenomas — in particular, those that are small and less complex, and in the case of IPMN, those that are branch-duct type are more commonly benign, some harbor or progress into in situ or invasive carcinomas. For this reason, pancreatic cysts with mucinous differentiation ought to be evaluated carefully, preferably by experts familiar with subtle evidences of malignancy in these tumors. In the past few years, the definition of IPMNs and MCNs has become more refined. The presence of ovarian-type stroma has now almost become a requirement for the diagnosis of MCN, and when defined as such, MCN is seen almost exclusively in women of perimenopausal age group as thick-walled multilocular cystic mass in the tail of the pancreas in contrast with IPMN which afflicts an elder population, both genders in almost equal numbers, and occur predominantly in the head of the organ. While mucinous lesions have well-established pre-malignant properties, most of the entities that fall into the nonmucinous true cyst category such as serous tumors, lymphoepithelial cysts, congenital cysts, and squamoid cyst of ducts have virtually no malignant potential. In contrast, the rare cystic tumors that occur as a result of degenerative/necrotic changes in otherwise solid neoplasia such as the rare cystic ductal adenocarcinomas, cystic endocrine neoplasia, and most importantly, solid-pseudopapillary tumor (SPT) in which cystic change is so common that it used to be incorporated into its name (“solid-cystic,” “papillary-cystic”) are malignant neoplasia, albeit variable degrees of aggressiveness. SPT holds a distinctive place among pancreatic neoplasia because of its highly peculiar characteristics, undetermined cell lineage, occurrence almost exclusively in young females, association with β-catenin pathway, and also by being a very low-grade curable malignancy. In conclusion, cystic lesions in the pancreas constitute a biologically and pathologically diverse category most (but not all) of which are either benign or treatable diseases; however, a substantial subset, especially mucinous ones, has malignant potential that requires careful analysis. This paper was originally presented as part of the SSAT/AGA/ASGE State-of-the-Art Conference on Management of Cystic Lesions of the Pancreas at the SSAT 48th Annual Meeting, May 2007 in Washington, DC. The other articles presented in the conference were Scheiman JM, Management of Cystic Lesions of the Pancreas: Diagnosis: Radiographic Imaging, EUS and Fluid Analysis; Tseng JF, Management of Serous Cystadenoma of the Pancreas; Fernández-del Castillo C, Mucinous Cystic Neoplasms; and Farnell MB, Surgical Management of Intraductal Papillary Mucinous Neoplasm (IPMN) of the Pancreas.     

Pancreatic mucinous cystic tumor in Turner syndrome: How a tumor bends to a genetic disease

INTRODUCTIONMucinous cystic neoplasms (MCN) are uncommon tumors of the pancreatic corpus/tail occurring mostly in middle-aged women, with a variable clinico-biological behavior. On histology, MCNs concurrently show an epithelial mucosecreting component with ovarian-type stromal cells.PRESENTATION OF CASEThis report describes the first case of a pancreatic MCN with no ovarian-type stroma in a patient with Turner syndrome (TS).DISCUSSIONThe mesenchymal component of MCN presumably results from the intra-pancreatic entrapment of ovarian stroma during embryogenesis. In our case, the absence of such stromal component may relate to the “dysgenetic” changes in the ovary involved in TS.CONCLUSIONThe present case of primary pancreatic MCN arising in a TS-patient triggers some original speculation on the morphogenesis of pancreatic MCN, also expanding the current clinico-pathological knowledge of this extremely rare entity.     

A Review of Mucinous Cystic Neoplasms of the Pancreas Defined by Ovarian-type Stroma: Clinicopathological Features of 344 Patients

Introduction Despite formal definitions of mucinous cystic neoplasms (MCNs) and intraductal papillary neoplasms (IPMNs) by the World Health Organization (WHO) and Armed Forces Institute of Pathology (AFIP), several controversies with regard to MCNs remain. The aim of this review was to determine the clinicopathological features of MCNs defined by ovarian-type stroma (OS) as proposed by the WHO and AFIP and to compare them with MCNs defined by less stringent criteria. Methods A MEDLINE search was conducted to identify English-language articles on pancreatic MCNs from 1996 to 2005. Twenty-five studies were identified. The studies were divided into 2 groups: group A included 10 studies with 344 patients whereby the presence of OS was a criteria for the diagnosis of MCNs, and group B, included 15 studies comprising 761 patients whereby the presence of OS was not mandatory for the diagnosis of MCNs. Results Patients in group A (MCNs as defined by OS) were almost always female (99.7%), with a mean age of 47 (range, 18–95) years. MCNs were located predominantly in the body or tail of the pancreas (94.6%) and had a mean size of 8.7 cm (range, 0.6–35 cm); 76% were symptomatic, 6.8% demonstrated ductal communication, and 27% were malignant. At a mean follow-up of 57.5 (range, 1–264) months and 43 (range, 2–257) months after surgery, 97.9% of benign and 61.9% of malignant neoplasms were disease free, respectively. Patients in group B were older and had a higher proportion of males. Neoplasms were more evenly distributed in the pancreas, were smaller, communicated more frequently with the pancreatic duct, and were composed of a higher proportion of malignant tumors compared with group A. Their clinicopathological features were intermediate between those of group A and patients with IPMN. Conclusion Pancreatic MCNs with OS have unique and distinct clinicopathological features. MCNs should be defined by the presence of OS, as it is the most reliable way of distinguishing MCNs from IPMN. Adoption of “looser” criteria will result in misclassification of some IPMNs as MCNs. Presented in part at the 7th World Congress of the International Hepato-Pancreato-Biliary Association, Edinburgh, 3-7 Sept 2006 (oral presentation)     

High incidence of extrapancreatic neoplasms in patients with intraductal papillary mucinous neoplasms

BACKGROUND: Intraductal papillary mucinous neoplasms (IPMNs) are associated with a high incidence of extrapancreatic neoplasms. DESIGN: Retrospective study. SETTING: Tertiary care referral center. PATIENTS: Sixty-one patients underwent surgical resection for IPMN between January 1, 1993, and June 30, 2004. Thirty-eight patients with mucinous cystic neoplasms and 50 patients with pancreatic ductal adenocarcinoma also were examined for development of extrapancreatic neoplasms. MAIN OUTCOME MEASURES: The incidence and clinicopathological features of extrapancreatic neoplasms with IPMNs were compared with those with mucinous cystic neoplasm and pancreatic ductal adenocarcinoma. RESULTS: Of the 61 patients with IPMNs, 24 (39%) developed 26 extrapancreatic neoplasms, and 18 (30%) had extrapancreatic malignancies. Gastric adenocarcinoma (33%) and colorectal adenocarcinoma (17%) were the most common neoplasms in the 24 patients. During postoperative follow-up, 3 patients died of malignant IPMNs, 3 of associated malignancies, and 1 of a nonmalignancy-related cause. Comparisons of the clinicopathological features in patients with IPMNs with and without associated malignancies revealed no significant differences in age, sex, family history of malignancy, history of cigarette smoking or alcohol abuse, or type of IPMN. The incidence of extrapancreatic neoplasms in patients with IPMN was significantly higher than in those with other pancreatic diseases such as mucinous cystic neoplasm (8%) or pancreatic ductal adenocarcinoma (10%). CONCLUSIONS: Frequently, IPMNs are associated with the development of extrapancreatic neoplasms. Considerable attention should be paid to the possible occurrence of other associated malignancies in patients with IPMNs, either concurrently or postoperatively. Further molecular studies may be necessary to elucidate the unusual association between IPMN and other primary neoplasms.     

Intraductal papillary mucinous neoplasms of the pancreas: an updated experience

OBJECTIVE: To update the authors' experience with intraductal papillary mucinous neoplasms (IPMNs) of the pancreas. BACKGROUND DATA: IPMNs are intraductal mucin-producing cystic neoplasms of the pancreas with clear malignant potential. Since the authors' 2001 report, the number of IPMNs resected at our institution has more than doubled, providing an opportunity to define the clinical features of this distinct neoplasm. METHODS: All patients undergoing pancreatic resection for an IPMN at the Johns Hopkins Hospital between January 1987 and March 2003 were evaluated. Noninvasive IPMNs were classified as "adenoma," "borderline," or "carcinoma-in situ" (CIS) depending on the degree of dysplasia within the specimen. Invasive cancers were classified as tubular, colloid, mixed, or anaplastic types. Pathology was retrospectively reviewed to identify main-duct or branch-duct origin of the tumors. Long-term overall survival for patients having IPMNs with invasive cancer was compared with those patients having IPMNs without an invasive component. RESULTS: Between January 1987 and March 2003, inclusive, 136 pancreatic resections were performed for patients with IPMNs, with 78 resections performed since January 2001. The mean age of the patients was 66.8 +/- 1.1 years, with 57% being male and 89% white. Pancreaticoduodenectomy was performed in 71% of patients, total pancreatectomy in 15%, distal pancreatectomy in 12%, and central pancreatic resection in 2%. IPMNs without evidence of invasive cancer were identified in 62% (n = 84) of patients (17% adenoma, 28% borderline, or 55% CIS). The remaining 38% (n = 52) of patients had IPMNs with associated invasive cancer (60% tubular, 27% colloid, 7% mixed, and 6% anaplastic). The mean age of patients with IPMN adenoma was 63.2 years, 66.7 years for those with borderline/CIS IPMNs, and 68.1 years for those with invasive cancer (P = 0.08, adenomas vs. invasive cancer). In those patients with invasive cancers, 15% had invasive cancer at the final surgical margin, 23% had IPMN without invasive cancer at the margin, and 54% had lymph node metastases. Residual IPMN was identified at the neck or uncinate margin in 24% of patients with noninvasive IPMNs. The overall 5-year survival for patients having IPMNs without invasive cancer was 77% (several deaths secondary to metachronous invasive cancer), compared with 43% in those patients with an invasive component (P < 0.0001). There were no differences in survival when comparing adenomas, borderline neoplasms, and CIS. Similarly, there were no statistically significant differences in survival when comparing branch-duct, main-duct, and combined variants; however, the branch-duct variants were more often noninvasive. For those patients with invasive IPMNs, 2-year survival was 40% when margins were positive for invasive cancer or for IPMN without invasive cancer, and 60% when margins were tumor-free (P = 0.15). Those patients with colloid carcinomas (n = 14) had improved survival compared with those with tubular carcinomas (n = 31), with 5-year survival rates of 83% and 24%, respectively. IPMN recurrences and deaths from cancer occurred in patients with both invasive and noninvasive IPMNs at initial resection. CONCLUSIONS: IPMNs continue to be recognized with increasing frequency. Five-year survival for those patients following resection of IPMNs with invasive cancer (43%) is improved compared with those patients with resected pancreatic ductal adenocarcinoma in the absence of IPMN (averages 15%-25%). Survival following resection of IPMNs without invasive cancer (regardless of degree of dyplasia) is good, but recurrent disease in the residual pancreas suggests that long-term surveillance is critical. Based on the age at resection data, there appears to be a 5-year lag time from IPMN adenoma (63.2 years) to invasive cancer (68.1 years).     

Incidence of additional primary cancers in patients with invasive intraductal papillary mucinous neoplasms and sporadic pancreatic adenocarcinomas

BACKGROUND: Recent small studies have reported an incidence of 23% to 39% for additional primary cancers in patients with intraductal papillary mucinous neoplasms (IPMN) of the pancreas. There have been no population-based studies evaluating this incidence rate. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) database (1983 to 1991), we identified all patients with primary pancreatic cancers (sporadic and adenocarcinomas arising in IPMNs). We determined the incidence of additional primary cancers that developed either before or after the diagnosis of invasive IPMN and compared it to the incidence of additional primary cancers in patients with sporadic pancreatic adenocarcinoma. RESULTS: Nineteen thousand six hundred forty-seven patients were reported with pancreatic cancer. Ninety-five percent of cancers were sporadic and 5.0% were invasive IPMNs. Ten point three percent had one or more extra-pancreatic primary cancers in addition to their pancreatic primary (10.3% in patients with sporadic adenocarcinoma and 10.1% in patients with invasive IPMNs, p = NS). The most common sites of additional primary cancers were colorectal (20.1%), breast (19.9%), prostate (16.6%), urinary system (11.1%), and lung (9.8%). In the 2,017 patients with additional primary cancer, 86% occurred before the diagnosis of pancreatic cancer and 14% occurred after the diagnosis of pancreatic cancer. CONCLUSIONS: Our population-based analysis shows that the incidence of additional primary malignancies in patients with invasive IPMNs is 10%. Although not as high as previously reported in smaller studies, the incidence is significant and comparable to the incidence seen in patients with adenocarcinoma. Surveillance for other common malignancies in patients with IPMNs and pancreatic adenocarcinomas should be performed.     

An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms

Invasive pancreatic ductal adenocarcinoma is an almost uniformly fatal disease. Several distinct noninvasive precursor lesions can give rise to invasive adenocarcinoma of the pancreas, and the prevention, detection, and treatment of these noninvasive lesions offers the potential to cure early pancreatic cancers. Noninvasive precursors of invasive ductal adenocarcinoma of the pancreas include pancreatic intraepithelial neoplasias (PanINs), intraductal papillary mucinous neoplasms (IPMNs), and mucinous cystic neoplasms. Diagnostic criteria, including a distinct ovarian-type stroma, and a consistent nomenclature are well established for mucinous cystic neoplasms. By contrast, consistent nomenclatures and diagnostic criteria have been more difficult to establish for PanINs and IPMNs. Because both PanINs and IPMNs consist of intraductal neoplastic proliferations of columnar, mucin-containing cells with a variable degree of papilla formation, the distinction between these two classes of precursor lesions remains problematic. Thus, considerable ambiguities still exist in the classification of noninvasive neoplasms in the pancreatic ducts. A meeting of international experts on precursor lesions of pancreatic cancer was held at The Johns Hopkins Hospital from August 18 to 19, 2003. The purpose of this meeting was to define an international acceptable set of diagnostic criteria for PanINs and IPMNs and to address a number of ambiguities that exist in the previously reported classification systems for these neoplasms. We present a consensus classification of the precursor lesions in the pancreatic ducts, PanINs and IPMNs.     

Predictive Factors of Malignant or Invasive Intraductal Papillary-Mucinous Neoplasms of the Pancreas

The aim of this study was to identify useful preoperative diagnostic findings indicative of malignant or invasive intraductal papillary-mucinous neoplasms (IPMN) of the pancreas to determine an optimal operative procedure for IPMN. Sixty-two IPMNs, which consisted of 29 adenomas, 10 borderline tumors, 11 adenocarcinomas in situ, and invasive adenocarcinomas were reviewed from 1990 to 2003. Preoperative predictive factors of malignant or invasive IPMN were analyzed among 10 factors by univariate and multivariate analysis. Diameter of the main pancreatic duct (≧6 mm) and cytological examination of the pancreatic juice (the presence of malignant cells) were identified as independent predictive factors of malignant IPMN, and only cytological examination of the pancreatic juice (the presence of malignant cells) was identified as an independent predictor of invasive IPMN by multivariate analysis (P < 0.05). There was no recurrent disease in patients with adenoma and adenocarcinoma in situ, whereas recurrences occurred in 6 of 12 patients with invasive IPMN. Patient survival in noninvasive IPMN was significantly (P = 0.018) better than that in invasive IPMN (The overall 5-year survival rates were 87.2% and 49.2%, respectively). These results might be useful for selecting an optimal surgical procedure for IPMN. Presented at the 2005 Congress of the American Hepato-Pancreato-Biliary Association, Fort Lauderdale, Florida, April 14-17, 2005 (free paper presentation).     

Mucinous cystic neoplasms of the pancreas: pathology and molecular genetics

Mucinous cystic neoplasm (MCN) of the pancreas is a distinct clinicopathological entity characterized by mucin-producing epithelial and cyst-forming neoplasm with “ovarian-type” stroma beneath the epithelial component. It is clearly distinguished from ductal adenocarcinoma and intraductal papillary mucinous neoplasm (IPMN). However, MCN can progress to infiltrating carcinoma, and frequently shows a similar histological pattern to ductal adenocarcinoma. Several genetic alterations such as K-ras oncogene mutation, and epigenetic alterations such as hypermethylation of p16 in the invasive component of MCN are also common with ductal adenocarcinoma. Furthermore, recent technologies, including a laser-assisted microdissection system for histological slides and global gene expression profilings using DNA microarrays, made possible to identify more information about molecular abnormalities of MCNs. It is important to diagnose the lesions before they progress to an invasive carcinoma. MCN is one of the precursors of invasive pancreatic carcinoma.     

Proposed new score predicting malignancy of intraductal papillary mucinous neoplasms of the pancreas

BACKGROUND: Our objective was to predict malignancy for intraductal papillary mucinous neoplasms of the pancreas (IPMN) before operation. METHODS: Sixty-four resected patients with IPMN were examined and 17 parameters were investigated for their relation to malignancy by univariate and multivariate analysis. RESULTS: Multivariate logistic regression analysis showed that IPMN type, the size of main pancreatic duct, and serum carbohydrate antigen 19-9 were significant for malignancy. Size of the main pancreatic duct > or = 6.5 mm and serum carbohydrate antigen 19-9 > or = 35 U/mL scored 3 points, main duct type scored 2 points, and patulous papilla, jaundice, diabetes mellitus, and tumor size > or = 42 mm scored 1 point. When IPMNs with 3 and more than 3 points using the new score were diagnosed as malignant, accuracy was 90.6%. CONCLUSION: This scoring system for IPMN is feasible to detect malignancy and useful for selecting an appropriate treatment.     

Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients

OBJECTIVE: Mucinous cystic neoplasms (MCNs) of the pancreas have often been confused with intraductal papillary mucinous neoplasms. We evaluated the clinicopathologic characteristics, prevalence of cancer, and prognosis of a large series of well-characterized MCNs in 2 tertiary centers. METHODS: Analysis of 163 patients with resected MCNs, defined by the presence of ovarian stroma and lack of communication with the main pancreatic duct. RESULTS: MCNs were seen mostly in women (95%) and in the distal pancreas (97%); 25% were incidentally discovered. Symptomatic patients typically had mild abdominal pain, but 9% presented with acute pancreatitis. One hundred eighteen patients (72%) had adenoma, 17 (10.5%) borderline tumors, 9 (5.5%) in situ carcinoma, and 19 (12%) invasive carcinoma. Patients with invasive carcinoma were significantly older than those with noninvasive neoplasms (55 vs. 44 years, P = 0.01). Findings associated with malignancy were presence of nodules (P = 0.0001) and diameter > or =60 mm (P = 0.0001). All neoplasms with cancer were either > or =40 mm in size or had nodules. There was no operative mortality and postoperative morbidity was 49%. Median follow-up was 57 months (range, 4-233); only patients with invasive carcinoma had recurrence. The 5-year disease-specific survival for noninvasive MCNs was 100%, and for those with invasive cancer, 57%. CONCLUSIONS: This series, the largest with MCNs defined by ovarian stroma, shows a prevalence of cancer of only 17.5%. Patients with invasive carcinoma are older, suggesting progression from adenoma to carcinoma. Although resection should be considered for all cases, in low-risk MCNs (< or =4 cm/no nodules), nonradical resections are appropriate.     

76例胰腺导管内乳头状黏液性肿瘤的外科治疗及预后分析

目的 分析胰腺导管内乳头状黏液性肿瘤患者的临床特征及手术疗效.方法 收集1999年1月至2008年12月复旦大学附属中山医院手术切除的76例胰腺导管内乳头状黏液性肿瘤的病史资料,并进行随访,分析其临床特征及手术疗效.结果 76例患者中,男性49例,女性27例;肿瘤位于胰头者63例,胰体尾10例,全胰3例;32例为非浸润性肿瘤(腺瘤16例,交界性肿瘤6例,原位癌10例),44例为浸润癌,两者在发病年龄及黄疸、消瘦、无症状患者、CA199升高等方面差异有统计学意义(P＜0.05);胰十二指肠切除59例,联合门静脉切除重建4例,胰体尾切除6例,局部切除2例,节段性胰腺切除2例,全胰切除3例;总体并发症发生率为28.9%,无手术相关死亡病例;非浸润性及浸润性肿瘤患者5年生存率分别为100%及35%;非浸润性肿瘤患者7例切缘阳性,其中1例术后67个月复发转移;多因素分析显示肿瘤直径及淋巴结状况是影响浸润性癌患者预后的独立因素.结论 非浸润性胰腺导管内乳头状黏液性肿瘤手术疗效极佳,而浸润癌患者的预后较差;及早手术是防止病变进展及改善预后的关键;术后必须进行长期随访.     

76例胰腺导管内乳头状黏液性肿瘤的外科治疗及预后分析

目的 分析胰腺导管内乳头状黏液性肿瘤患者的临床特征及手术疗效.方法 收集1999年1月至2008年12月复旦大学附属中山医院手术切除的76例胰腺导管内乳头状黏液性肿瘤的病史资料,并进行随访,分析其临床特征及手术疗效.结果 76例患者中,男性49例,女性27例;肿瘤位于胰头者63例,胰体尾10例,全胰3例;32例为非浸润性肿瘤(腺瘤16例,交界性肿瘤6例,原位癌10例),44例为浸润癌,两者在发病年龄及黄疸、消瘦、无症状患者、CA199升高等方面差异有统计学意义(P＜0.05);胰十二指肠切除59例,联合门静脉切除重建4例,胰体尾切除6例,局部切除2例,节段性胰腺切除2例,全胰切除3例;总体并发症发生率为28.9%,无手术相关死亡病例;非浸润性及浸润性肿瘤患者5年生存率分别为100%及35%;非浸润性肿瘤患者7例切缘阳性,其中1例术后67个月复发转移;多因素分析显示肿瘤直径及淋巴结状况是影响浸润性癌患者预后的独立因素.结论 非浸润性胰腺导管内乳头状黏液性肿瘤手术疗效极佳,而浸润癌患者的预后较差;及早手术是防止病变进展及改善预后的关键;术后必须进行长期随访.

Abstract：

Objective To investigate the outcome of intraductual papillary mucious neoplasms (IPMN) of the pancreas after surgical resection. Method Clinical data of 76 patients with intraductal papillary neoplasms of the pancreas undergoing surgical resection at Zhongshan Hospital, Fudan University between January 1999 and December 2008 were retrospectively analyzed. Results Among the 76 patients,49 were male, 37 were female. 32 had noninvasive IPMNs, including adenomas( n = 16), borderline tumors (n =6 ), carcinomas in situ (n = 10 ). 44 had invasive IPMNs. Lesions were present in the head in 63 cases, in the body or tail in 10, in the whole pancreas in 3. There were significant difference in age,jaundice, weight loss, asymptomatic cases and CA199 value between noninvasive and invasive IPMNs.Three patients underwent total pancreatectomy, 59 patients underwent pancreaticoduodenectomy, 4 patients underwent pancreaticoduodenectomy with portal vein resection and reconstruction, six patients underwent distal pancreatectomy, two patients each underwent central pancreatectomy or enucleation. The overall postoperative morbidity rate were 28.9%, there was no operative mortality. Positive pancreatic margin was identified in seven patients of noninvasive neoplasms, among thoee one developed recurrence after 67 months. The five-year survival rate for patients with noninvasive and invasive neolpasms was 100% and 35% ,respectively. Size and lymph node metastasis were significant prognostic factors after surgical resection of the invasive IPMNs. Conclusions Surgical resection provides a favorable outcome for patients with noninvasive IPMNs. In contrast, invasive IPMNs was associated with a poor survival. Early resection is essential for improving survival. Long-term follow-up is necessary for all patients with IPMNs after resection.     

76例胰腺导管内乳头状黏液性肿瘤的外科治疗及预后分析

目的 分析胰腺导管内乳头状黏液性肿瘤患者的临床特征及手术疗效.方法 收集1999年1月至2008年12月复旦大学附属中山医院手术切除的76例胰腺导管内乳头状黏液性肿瘤的病史资料,并进行随访,分析其临床特征及手术疗效.结果 76例患者中,男性49例,女性27例;肿瘤位于胰头者63例,胰体尾10例,全胰3例;32例为非浸润性肿瘤(腺瘤16例,交界性肿瘤6例,原位癌10例),44例为浸润癌,两者在发病年龄及黄疸、消瘦、无症状患者、CA199升高等方面差异有统计学意义(P＜0.05);胰十二指肠切除59例,联合门静脉切除重建4例,胰体尾切除6例,局部切除2例,节段性胰腺切除2例,全胰切除3例;总体并发症发生率为28.9%,无手术相关死亡病例;非浸润性及浸润性肿瘤患者5年生存率分别为100%及35%;非浸润性肿瘤患者7例切缘阳性,其中1例术后67个月复发转移;多因素分析显示肿瘤直径及淋巴结状况是影响浸润性癌患者预后的独立因素.结论 非浸润性胰腺导管内乳头状黏液性肿瘤手术疗效极佳,而浸润癌患者的预后较差;及早手术是防止病变进展及改善预后的关键;术后必须进行长期随访.     

Pancreatic Serous Oligocystic Adenomas: Clinicopathologic Features and a Comparison with Serous Microcystic Adenomas and Mucinous Cystic Neoplasms

Introduction The preoperative distinction between serous cystic neoplasms (SCNs) and mucinous cystic neoplasms (MCNs) is essential, as all MCNs are considered malignant or potentially malignant and should be surgically resected, whereas SCNs are almost always benign. However, the radiologic distinction between SCNs and MCNs is frequently difficult especially with serous oligocystic adenoma (SOA), a morphologic variant of SCN, as both SOA and MCN appear on cross-sectional imaging as a solitary macrocystic lesion in the pancreas. We reviewed all SOAs managed at our institution to determine if any clinicopathologic features would prove useful for establishing a preoperative diagnosis. Methods Over a 15-year period, 64 patients with a pathologically confirmed diagnosis of a pancreatic cystadenoma or cystadenocarcinoma treated at Singapore General Hospital were retrospectively reviewed. There were 27 MCNs and 37 SCNs including 12 SOAs. In addition, 40 cases of SOA previously reported in the literature were reviewed and analyzed together with the 12 patients, making this a series of 52 SOAs. Results In our experience, SOAs comprised 32.4% of the SCNs, and females predominated (7/12). The median age of the patients was 42.5 years (range 22–74 years), and only 4 of the 12 patients were symptomatic. Most of the cysts were located in the body or tail of the pancreas (9/12), and the median cyst size was 52.5 mm (range 10–190 mm). When the clinicopathologic features of SOAs and serous microcystic adenomas (SMAs) were compared, there was no difference between the patients with SOAs and SMAs in terms of age, sex, presence of symptoms, cyst size, or site of the lesion. However, SOAs occurred in the women less frequently (67.3% vs. 96.3%, P = 0.004), were smaller [40 mm (range 10–190 mm) vs. 95 mm (range 25–180 mm), P < 0.001], and occurred more commonly in the head of the pancreas [25 (48.1%) vs. 2(7.4%)] compared to MCNs. None of the SOAs were frankly malignant compared to the 29.6% of MCNs that were. Conclusions SOAs and SMAs have similar clinicopathologic features. On the other hand, SOAs differ from MCNs by their relatively higher male/female ratio, higher frequency of tumors occurring in the head of the pancreas, and smaller cyst size. Knowledge of these distinguishing clinical features when used in combination with other diagnostic modalities such as endoscopic ultrasonography/fine-needle aspiration will enable clinicians to better differentiate these two pathologic entities preoperatively.