The impact of resection margin distance on survival and recurrence in pancreatic ductal adenocarcinoma in a retrospective cohort analysis.

BACKGROUND: The prognostic effect of resection margin status following pancreatoduodenectomy for pancreatic ductal adenocarcinoma (PDAC) remains controversial, even with the implementation of standardized pathological assessment. We therefore investigated the impact of resection margin (RM) status and RM distance in curative resected PDAC on overall survival (OS), disease-free survival (DFS) and recurrence. METHOD: 108 patients were retrieved from a prospectively maintained database of a certified pancreatic cancer center. Distribution and relationships between circumferential resection margin (CRM) involvement (CRM≤1mm; CRM>1mm; CRM≥2mm) and their prognostic impact on OS and DFS were assessed using Kaplan-Meier statistics and the Log-Rank test. Multivariate logistic regression was used explain the development of a recurrence 12 months after surgery. RESULTS: 63 out of 108 patients had medial RM and 32 posterior RM involvement. There was no significant difference in OS and DFS between CRM≤1mm and CRM>1mm resections. Clearance at the medial margin of ≥2mm had an impact on OS and DFS, (RM≥2mm vs. RM<2mm: median OS 29.8 vs 16.8 months, median DFS 19.6 vs. 10.3 months). Multivariate analysis demonstrated that age, medial RM ≥2mm, lymph node status and chemotherapy were prognostic factors for OS and DFS. Posterior RM had no influence on OS or DFS. CONCLUSION: Not all RM seem to have the same impact on OS and DFS, and a clearance of 1mm for definition of a negative RM (i.e. CRM>1mm) seems not sufficient. Future studies should include more patients to stratify for potential confounders we could not account for. TRIAL REGISTRATION: This study was registered with the German Clinical Trials Registry (reference number DRKS0017425).

[Pathological processing in pancreatic ductal adenocarcinoma-What is new?] / Pathologische Aufbereitung beim duktalen Adenokarzinom des Pankreas ­ was gibt es Neues?

The macroscopic and microscopic assessments of pancreatic cancer resection specimens belong to the standard repertoire of any department of pathology. In recent years standards have been developed regarding both macroscopic and microscopic assessments, which are laid down in international and national guidelines and classifications and are regularly updated. In this way the reporting of results and interdisciplinary communication are facilitated. These classifications and guidelines are influenced by current studies and the data from them provide information on which histopathological factors are particularly relevant for the prognosis and treatment. Due to the increasing use of neoadjuvant therapy the assessment of tumor regression in histopathological specimens is also gaining in importance. Finally, individual targeted treatments are also now available for pancreatic cancer, which require extended molecular pathological diagnostics.

Pancreatic Apoplexy: Fulminant Necrotizing Pancreatitis Leading to Completion Pancreatectomy Within 3 Days After Partial Pancreaticoduodenectomy.

OBJECTIVES: Patient characteristics with postoperative acute necrotizing pancreatitis and completion pancreatectomy (CP) after pancreaticoduodenectomy (PD) remain unclear. METHODS: Data from all patients who underwent a PD with need for CP (January 2011-December 2019) at a German University Hospital were analyzed regarding the indications and timing of CP, laboratory and histopathological findings, and overall outcome. RESULTS: Six hundred twelve patients underwent PD, 33 (5.4%) of them needed a CP. Indications were grade C pancreatic fistula with or without biliary leak (46% and 12%), biliary leak (6%), and hemorrhage due to pancreatic fistula (36%). Eight patients (24%) underwent CP within 3 days after PD. These fulminant courses ("pancreatic apoplexy") were accompanied by significantly higher levels of lactate dehydrogenase, C-reactive protein, serum amylase, serum lipase, drain amylase, and drain lipase compared with patients with CP after the third day. Pancreatic apoplexy was histologically associated with higher rates of pancreatic necrosis (P = 0.044) and hemorrhage (P = 0.001). A trend toward higher mortality was observed (75% vs 36%, P = 0.058). CONCLUSIONS: Pancreatic apoplexy, defined as fulminant necrotizing pancreatitis after PD leading to CP within 3 days, is associated with characteristic laboratory and histopathological findings and a trend to higher mortality.

Recurrence of Pancreatic Ductal Adenocarcinoma after Complete Histopathological Remission Caused by FOLFIRINOX.

We previously reported 2 cases of pathologic complete response (pCR) of a pancreatic cancer (PC) following neoadjuvant FOLFIRINOX treatment. We now complete our report by a follow-up on both patients. Patient 1 achieved a disease-free survival of 12 months after initial resection until she developed a singular hepatic metastasis. Treatment by FOLFIRINOX and complete removal of the metastasis by atypical liver resection after 6 months allowed for another 12 months of disease control. After intra-abdominal tumor recurrence and development of intracerebral metastases, the patient died 34 months after primary diagnosis. Patient 2 developed hepatic tumor recurrence only 3 months after initial resection. However, treatment by FOLFIRINOX led to a stable disease for 27 months after resection and was followed by atypical liver resection of multiple segments. Six months later, another hepatic recurrence was suspected. Via next-generation sequencing (NGS) of the tumor genome, a deleterious mutation in the ataxia telangiectasia-mutated (ATM) gene, causing a BRCAness, was detected. After initial treatment by FOLFOX, maintenance therapy with the poly-ADP-ribose-polymerase (PARP) inhibitor olaparib was initiated. The patient is now alive for 54 months after initial diagnosis of metastasized pancreatic adenocarcinoma. Tumor recurrence is possible even after pCR of PC and remains challenging. In case of multifocal tumor recurrence, chemotherapy remains the standard treatment. Recently, genetic sequencing allows individualized treatments. In selected cases, highly specialized teams can offer a variety of therapeutic options leading to previously unseen clinical courses.

The Unusual Suspects of the Pancreas-Understanding Pancreatic Acinar Cell Carcinomas and Adenomas.

PURPOSE: Acinar cell carcinomas (ACC) and adenomas (ACA) of the pancreas are rare entities. Sufficient knowledge about occurrence and prognosis is scarce. METHODS: A retrospective chart review of our database was performed for all patients who had undergone pancreatic surgery between 2006 and 2018. Results were compared to recent literature findings. RESULTS: Nine patients were diagnosed with ACC and four patients with ACA of the pancreas in the study period. ACC patients were older and more often male than patients of the ACA group. ACC were mainly localized in the pancreatic head, whereas ACA were more often found in the distal pancreas. Tumor markers are not necessarily elevated, even in case of malignancy. CONCLUSIONS: ACC and ACA are very rare pancreatic tumors. Both entities account for less than 1% of all pancreatic neoplasms. Diagnosis is challenging due to unspecific radiologic features and clinical symptoms. Nevertheless, a patient complaining of abdominal discomfort and an unclear hypodense pancreatic lesion should undergo surgical exploration.

Prognostic value and impact of cerebral metastases in pancreatic cancer.

Background: Pancreatic cancer is a fatal disease most often diagnosed at an advanced stage. Most patients already suffer from irresectable tumor or distant metastases being most commonly found in the liver or the lung. However, cerebral metastases occur extremely rare.Methods: We performed a retrospective analysis of our database to identify all patients diagnosed with pancreatic cancer and cerebral metastases who underwent surgical treatment in our department from January 2004 to November 2016.Results: Only 0.2% (4 of 2492) were diagnosed with cerebral metastases. Two patients had surgical resection of the cerebral metastases. One patient underwent palliative radiation therapy and the fourth patient received only palliative therapy. Mean interval between initial diagnosis and development of brain metastases was 8.5 months (range 1-20). Mean survival period after diagnosis of brain metastases was 4.75 months (range 1-10).Conclusions: Cerebral metastases of pancreatic cancer occur extremely rare. They are associated with an advanced tumor stage, commonly liver and lung metastases. All patients presenting with neurological symptoms, multifocal metastases, and significantly elevated CA 19-9 levels are suspicious of sustaining cerebral metastases and should undergo brain imaging.

Outcome Determining Factors of Intraductal Papillary Neoplasm of the Biliary Tract (IPNB)-a Single Center Survey and Analysis of Current Literature.

PURPOSE: Intraductal papillary neoplasms of the biliary tract (IPNB) are rare tumors originating from the bile duct epithelium. Metastatic disease of IPNB is extremely rare and only reported in a small number of cases worldwide. Due to this limitation in number, the treatment of IPNB mainly relies on retrospective case series. PATIENTS AND METHODS: We reported three cases of IPNB, one benign, one carcinoma with lymph node metastasis, and one case with histologically proven metachronous pulmonary metastasis. We correlated our findings with the existing data found in the literature. All patients underwent hemihepatectomy and complete tumor resection was achieved. RESULTS: Diagnosis of IPNB can be challenging due to varying presentation. The treatment of choice is surgical oncological resection in an early tumor stage. Long-term outcome highly depends on the underlying grade of dysplasia, multiplicity, and tumor-free margins. Aggressive tumor invasion is reported in up to 72% of cases in IPNB. Furthermore, the recurrence rate of IPNB is high with up to 22%. Further factors associated with an impaired survival are incomplete resection, lymph node involvement, and MUC1 expression. CONCLUSION: High potential for dysplasia and proof of invasive carcinoma upon diagnosis are hallmarks of IPNB. Metastatic disease in IPNB is reported only in small numbers. IPNB is an aggressive tumor entity with impaired long-term outcomes. A drawback for interpretation of current data is the fact that they rely on case series and reports and are not validated through more powerful randomized multicentric trials.

Pathologic Complete Response of Pancreatic Cancer following Neoadjuvant FOLFIRINOX Treatment in Hepatic Metastasized Pancreatic Cancer.

INTRODUCTION: Pancreatic cancer is a lethal disease and often asymptomatic. Therefore, it is most often diagnosed at an advanced stage. The standard approach in a metastasized tumor stage is palliative chemotherapy. However, the prognosis remains extremely poor. CASE REPORT: We present the case of a patient who was diagnosed with a cancer of the head of the pancreas with hepatic metastases. After receiving palliative intended chemotherapy with the FOLFIRINOX regimen, staging computed tomography revealed the disappearance of the liver metastases and local resectability of the pancreatic head tumor. The patient underwent an uneventful Whipple's procedure. Surprisingly, pathohistological investigation revealed a complete pathological response. CONCLUSION: Pathological complete response after FOLFIRINOX treatment in hepatic metastasized pancreatic cancer is extremely rare. It enables surgical resection and increases the survival rate significantly.

FOLFIRINOX treatment leading to pathologic complete response of a locally advanced pancreatic cancer.

Pancreatic cancer (PC) is a lethal disease with a poor prognosis. It is typically asymptomatic and therefore most often diagnosed at an advanced stage. A primary unresectable PC can become resectable in case of tumor regression turning palliative into neoadjuvant therapy. We present a 67-year old female patient who was diagnosed with a locally advanced adenocarcinoma of the pancreatic head. After receiving palliative intended chemotherapy with the FOLFIRINOX regimen, staging computed tomography revealed local resectability of the pancreatic head tumor. The patient underwent an uneventful total pancreatectomy. Pathohistological investigation revealed a pathologic complete response (pCR). pCR after FOLFIRINOX treatment in primary unresectable PC is extremely rare. It might enable surgical resection and can increase the survival rate.

Conversion cholecystectomy in patients with acute cholecystitis-it's not as black as it's painted!

BACKGROUND: Although laparoscopic cholecystectomy is recommended as standard treatment for acute cholecystitis, in 10-30 % a conversion to open cholecystectomy is required. Among some surgeons, this is still perceived as a "complication." The aim of our study was to define characteristics and outcome of patients with acute cholecystitis undergoing conversion cholecystectomy. METHODS: Over a 9-year period, 464 consecutive patients undergoing cholecystectomy for acute cholecystitis were analyzed for demographic, preoperative, intraoperative, histopathological, and laboratory findings and surgical outcome parameters. RESULTS: Patients with conversion cholecystectomy were characterized by younger age, lower American Society of Anesthesiologists (ASA) score, and less cardiac comorbidities compared to patients with primary open cholecystectomy. Severity of inflammation on the clinical and histopathological level was similar and comparable. Overall complication rate, mortality, and median hospital stay were significantly lower compared to those of primary open cholecystectomy group. CONCLUSIONS: There are no disadvantages for patients undergoing conversion cholecystectomy compared to primary open cholecystectomy. The outcome is influenced by general condition and comorbidities rather than by the surgical approach. Underlying fear of conversion should not avoid a laparoscopic approach in patients with acute cholecystitis.

[Curative endoscopic therapy: which lesions can be addressed]. / Kurative endoskopische Therapie: Welche Läsionen eignen sich?

Since the implementation of a nationwide screening colonoscopy the incidence of colorectal adenocarcinoma is decreasing, while the amount of early endoscopically resectable tumours is increasing. Similarly malignant tumors of the upper gastrointestinal tract are diagnosed and resected endoscopically more often. In part, this also depends on improvements in technology.The current guidelines of diagnosis and treatment of squamous carcinoma and adenocarcinoma of the esophagus, gastric cancer and colorectal cancer exactly define, which lesions can be endoscopically resected curatively. This mostly depends on the risc of metastases in regional lymph nodes. The risc of a lymphatic spread can be asessed by histopathological characteristics depending on the localisation of the lesion. Substantially, these characteristics comprise the depth of invasion in the resection specimen and the status at the resection margins after the endoscopic resection as well as the tumor subtype and the histological grading of the tumor. In addition, an infiltration in lymphatic or blood vessels has to be taken into account.

False-Positive PET/CT After Cyanoacrylate Sealing of a Pancreaticojejunostomy.

In 2013, a 68-year-old male had a pancreaticoduodenectomy for pancreatic cancer. The pancreaticojejunostomy was sealed with cyanoacrylate (Dermabond) to prevent postoperative pancreatic fistula. Local recurrence of malignancy at the anastomosis was suspected 18 months later in PET/CT. Surgical revision was performed and anastomosis resected. However, histology showed no tumor recurrence, but strong inflammation and foreign-body reaction towards Dermabond. The sealant caused false-positive PET/CT findings, so its use in oncologic surgery should be abandoned.

A microRNA-based test improves endoscopic ultrasound-guided cytologic diagnosis of pancreatic cancer.

BACKGROUND & AIMS: Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) in combination with cytopathology is the optimal method for diagnosis and staging of pancreatic ductal adenocarcinoma (PDAC) and other pancreatic lesions. Its clinical utility, however, can be limited by high rates of indeterminate or false-negative results. We aimed to develop and validate a microRNA (miRNA)-based test to improve preoperative detection of PDAC. METHODS: Levels of miRNAs were analyzed in a centralized clinical laboratory by relative quantitative polymerase chain reaction in 95 formalin-fixed paraffin-embedded specimens and 228 samples collected by EUS-FNA during routine evaluations of patients with solid pancreatic masses at 4 institutions in the United States, 1 in Canada, and 1 in Poland. RESULTS: We developed a 5-miRNA expression classifier, consisting of MIR24, MIR130B, MIR135B, MIR148A, and MIR196, that could identify PDAC in well-characterized, formalin-fixed, paraffin-embedded specimens. Detection of PDAC in EUS-FNA samples increased from 78.8% by cytology analysis alone (95% confidence interval, 72.2%-84.5%) to 90.8% when combined with miRNA analysis (95% confidence interval, 85.6%-94.5%). The miRNA classifier correctly identified 22 additional true PDAC cases among 39 samples initially classified as benign, indeterminate, or nondiagnostic by cytology. Cytology and miRNA test results each were associated significantly with PDAC (P < .001), with positive predictive values greater than 99% (95% confidence interval, 96%-100%). CONCLUSIONS: We developed and validated a 5-miRNA classifier that can accurately predict which preoperative pancreatic EUS-FNA specimens contain PDAC. This test might aid in the diagnosis of pancreatic cancer by reducing the number of FNAs without a definitive adenocarcinoma diagnosis, thereby reducing the number of repeat EUS-FNA procedures.

Epidemiology of Colorectal Adenomas and Histopathological Assessment of Endoscopic Specimens in the Colorectum.

BACKGROUND: Colorectal cancer is one of the most frequently observed neoplasms in the world. It develops from intraepithelial neoplasia of the colorectal mucosa, and these precursor lesions are also known as adenoma. As the precursor lesion is known and can be detected easily, efficient screening strategies are available for a reliable prevention of colorectal adenocarcinoma, e.g. by colonoscopy. METHODS: Literature databases (PubMed) were searched selectively for the keywords 'colorectal adenoma', 'epidemiology', and 'resection techniques'. The results are presented in the following text, also taking into account our own experience and the current S3 guidelines. RESULTS: Endoscopic resection samples are one of the specimens most frequently assessed by pathologists. Therefore, gastroenterologists expect standardized and well-structured pathology reports, stating relevant information concerning the removed lesions and recommendations for clinical management. These aspects are summarized in the evidence-based S3 guideline. CONCLUSION: As a consequence of colorectal adenoma resection during screening procedures, the carcinoma incidence is decreasing. For further advancements in successful prevention, knowledge of different precursor lesions (conventional adenoma, serrated adenoma) is important, but also structured communication between the different disciplines engaged in colorectal cancer screening.

Influence of MRE11, RAD50 and NIBRIN protein expression on survival in pancreatic carcinoma after curative resection.

The MRE11/RAD50/NIBRIN complex, a protein complex that repairs DNA double-strand breaks, could serve as an early marker for new lesions in pancreatic cancer. We determined the expression of MRE11, RAD50 and NIBRIN, and their possible prognostic value regarding survival. Forty-one patients with ductal adenocarcinoma of the pancreas were included. All underwent curative surgery. Immunohistochemistry was performed for MRE11, RAD50 and NIBRIN. Subsequent analyses were based on a modified immunoreactive score. Statistical analysis was conducted using the statistics program "R". The mean follow-up period was 509 days. The mean age of the patients was 67±8 years, male=56%, female=44%. Eighty-seven percent underwent a Kausch-Whipple procedure, whereas a left side resection was performed in 22% of patients. Positive lymph nodes were found in 80% of cases, and patients were staged UICC IIa (12%), IIb (56%) and IV (29%). Overall significant results were found for MRE11 (p=0.02) and NIBRIN (p=0.01) expression and postoperative survival. We found a significant relation between the expression of MRE11, NIBRIN and the postoperative survival of patients with ductal adenocarcinoma. The link between the expression of the MRN complex, ATM and pancreatic cancer can be used to develop new treatment options for pancreatic carcinoma.

Quantitative assessment and determinants of suture-holding capacity of human pancreas.

BACKGROUND: Hard pancreas is welcome by surgeons performing resective pancreatic surgery, because it is believed to offer better suture holding capacity (SHC), thus decreasing the risk for a postoperative leak. However, neither the actual SHC of pancreatic tissue in humans nor its determinants have been studied. METHODS: We directly measured SHC for polydioxanone 5-0 suture and tissue hardness at the pancreatic isthmus in 53 human pancreata using a dynamometer and a durometer. A histologic score based on fibrosis grade, fat content, pancreatic duct size, and signs of chronic pancreatitis was calculated for every sample. We tested the hypothesis that SHC of the pancreas was proportional to tissue hardness, and evaluated the role of different possible histomorphologic determinants of SHC. RESULTS: Suture-holding capacity correlated perfectly with tissue hardness (r = 0.98; P < 0.001; 95% confidence interval, 0.96-0.99). The histologic score showed a stronger correlation with both parameters than any single histologic parameter. The SHC of transductal sutures was significantly higher than that of pure transparenchymal sutures. The SHC and hardness were significantly lower in patients who developed a clinically relevant pancreatic fistula postoperatively. CONCLUSIONS: A mixture of histomorphologic features of human pancreas determines its tissue hardness and SHC. Involvement of the main pancreatic duct in the suture line appears to increase the mechanical strength of the pancreatic anastomosis.

ß-Catenin, Cox-2 and p53 immunostaining in colorectal adenomas to predict recurrence after endoscopic polypectomy.

BACKGROUND: Endoscopic polypectomy significantly reduces the incidence of colorectal cancer, but recurrence rates are high, especially for adenomas with advanced histology. The present guidelines recommend re-colonoscopy 3 to 5 years later. Due to limited resources, more precise predictions of adenoma recurrence are required. DESIGN: Lesions from 109 patients with colorectal adenomas recruited into a randomized, placebo-controlled chemoprevention trial with mesalazine were included. Formalin-fixed paraffin-embedded tissue sections were stained for ß-catenin, cyclooxygenase-2 (Cox-2), and p53 and scored. Adenoma recurrence rates were recorded after 3 years and associated with clinical and immunohistochemical parameters by contingency table analysis. RESULTS: After 3 years, adenomas recurred in 51.4% of patients. Out of 109 adenomas, 95 met at least one criterion of advanced adenoma (size >1 cm, villous histology, high-grade intraepithelial neoplasia). There was no influence of age, sex, size or villous histology on adenoma reappearance, whilst the number of adenomas at baseline was positively associated with recurrence (p = 0.003). In contrast, ß-catenin nuclear localisation, Cox-2 expression and p53 nuclear expression were significantly associated with adenoma recurrence after 3 years (ß-catenin: p = 0.002; Cox-2: p = 0.001; p53: p = 0.001). Combining these three markers led to a negative predictive value of 88.5% and a sensitivity of 94.6%. (OR = 13.54) CONCLUSIONS: Scoring each single parameter and, more strongly, the combination of all three parameters of the expression of ß-catenin, Cox-2 and p53 in colorectal adenoma tissue may be a useful negative predictor for adenoma recurrence in patients with advanced colorectal adenomas.

Circulating U2 small nuclear RNA fragments as a novel diagnostic biomarker for pancreatic and colorectal adenocarcinoma.

Improved non-invasive strategies for early cancer detection are urgently needed to reduce morbidity and mortality. Non-coding RNAs, such as microRNAs and small nucleolar RNAs, have been proposed as biomarkers for non-invasive cancer diagnosis. Analyzing serum derived from nude mice implanted with primary human pancreatic ductal adenocarcinoma (PDAC), we identified 15 diagnostic microRNA candidates. Of those miR-1246 was selected based on its high abundance in serum of tumor carrying mice. Subsequently, we noted a cross reactivity of the established miR-1246 assays with RNA fragments derived from U2 small nuclear RNA (RNU2-1). Importantly, we found that the assay signal discriminating tumor from controls was derived from U2 small nuclear RNA (snRNA) fragments (RNU2-1f) and not from miR-1246. In addition, we observed a remarkable stability of RNU2-1f in serum and provide experimental evidence that hsa-miR-1246 is likely a pseudo microRNA. In a next step, RNU2-1f was measured by qRT-PCR and normalized to cel-54 in 191 serum/plasma samples from PDAC and colorectal carcinoma (CRC) patients. In comparison to 129 controls, we were able to classify samples as cancerous with a sensitivity and specificity of 97.7% [95% CI = (87.7, 99.9)] and 90.6% [95% CI = (80.7, 96.5)], respectively [area under the ROC curve 0.972]. Of note, patients with CRC were detected with our assay as early as UICC Stage II with a sensitivity of 81%. In conclusion, this is the first report showing that fragments of U2 snRNA are highly stable in serum and plasma and may serve as novel diagnostic biomarker for PDAC and CRC for future prospective screening studies.

Osteonecrosis of the mandible due to anti-angiogenic agent, bevacizumab.

BACKGROUND: Osteonecrosis of the jaw (ONJ) is defined by areas of tissue breakdown and exposure of bone in the maxillofacial region that fail to heal within 8 weeks after identification by a health provider in a patient who has not received radiation of the jaws. The disease affects the quality of life and produces significant morbidity in afflicted patients. ONJ is correlated with such risk factors as treatment with bisphosphonates, dental extraction-related trauma, chemotherapy, corticosteroids, renal osteodystrophy and infections. Although the use of bisphosphonates is associated with osteonecrosis of the jaw, the pathophysiology of bisphosphonate-associated ONJ is still unknown. It has been assumed that bisphosphonates lead to the inhibition of capillary angiogenesis and disturbances in the activities of both osteoblasts and osteoclasts, thereby impairing bone remodelling. Currently, inhibitors of angiogenesis used in the treatment of cancer patients are implicated in isolated cases of ONJ. CASE REPORT: This manuscript reports a case of ONJ in a female patient who received bevacizumab (Avastin®, Roche), a humanised monoclonal antibody that recognises and blocks vascular endothelial growth factor (VEGF)-A. CONCLUSION: The anti-angiogenic agent, bevacizumab, may increase the risk of osteonecrosis of the jaw. This agent inhibits VEGF and, therefore, also presumably represses the vascularisation of the jaw, which leads to healing complications. Due to increasing use of bevacizumab, patients receiving this agent should be closely monitored for possible side effects.

Rare solid tumors of the pancreas as differential diagnosis of pancreatic adenocarcinoma.

CONTEXT: Rare solid tumors of the pancreas can be misinterpreted as primary pancreatic cancer. OBJECTIVE: The aim of this study was to report our experience in the treatment of patients with rare tumor lesions of the pancreas and to discuss clinical and pathological characteristics in the context of the role of surgery. DESIGN: Data from patients of our prospective data-base with rare benign and malignant tumors of the pancreas, treated in our division from January 2004 to August 2010, were analyzed retrospectively. RESULTS: One-thousand and ninety-eight patients with solid tumors of the pancreas underwent pancreatic surgery. In 19 patients (10 women, 9 men) with a mean age of 57 years (range: 20-74 years) rare pancreatic tumors (metastasis, solid pseudopapillary tumor, teratoma, hemangioma, accessory spleen, lymphoepithelial cyst, hamartoma, sarcoidosis, yolk sac tumor) were the reason for surgical intervention. CONCLUSION: If rare benign and malignant pancreatic tumors, intrapancreatic metastasis, as well as pancreatic malformations or other abnormalities, present themselves as solid masses of the pancreas, they constitute an important differential diagnosis to primary pancreatic neoplasia, e.g. pancreatic ductal adenocarcinoma. Clinical imaging techniques cannot always rule out malignancy, thus operative exploration often remains the treatment of choice to provide the correct diagnosis and initiate adequate surgical therapy.