Survival After Adrenalectomy for Metastatic Hepatocellular Carcinoma: A 25-year Institutional Experience.

BACKGROUND: Extrahepatic manifestation of hepatocellular carcinoma (HCC) is rare and primarily affects lung, lymph nodes and bone. Metastases to the adrenal glands are relatively infrequent. This 25-year institutional experience aimed for an analysis of factors influencing survival in patients undergoing surgery for HCC adrenal metastasis. METHODS: A retrospective analysis of the institutional database of the Clinic for General-, Visceral- and Transplantation Surgery of the University Medical Center Mainz, Germany, was performed. Patients who underwent surgery for HCC adrenal metastases from January 1995 to June 2020 were included. Pre-, peri- and postoperative factors with potential influence on survival were assessed. RESULTS: In 16 patients (14 males, two females), one bilateral and 15 unilateral adrenalectomies were performed (13 metachronous, three synchronous). Thirteen operations were carried out via laparotomy, and three adrenalectomies were minimally invasive (two laparoscopic, one retroperitoneoscopic). Median overall survival (after HCC diagnosis) was 35 months, range: 5-198. Median post-resection survival (after adrenalectomy) was 15 months, range: 0-75. Overall survival was longer in patients with the primary HCC treatment being liver transplantation (median 66 months) or liver resection (median 51 months), compared to only palliative intended treatment of the primary with chemotherapy (median 35 months) or local ablation (median 23 months). CONCLUSIONS: Surgery is a feasible treatment option for patients with adrenal metastases originating from HCC. In patients who underwent adrenalectomy for HCC adrenal metastasis, overall survival was superior, if primary HCC treatment was potentially curative (liver transplantation or resection).

Robot-assisted minimally invasive esophagectomy (RAMIE) compared to conventional minimally invasive esophagectomy (MIE) for esophageal cancer: a propensity-matched analysis.

Robot-assisted minimally invasive esophagectomy (RAMIE) is increasingly being applied as treatment for esophageal cancer. In this study, the results of 50 RAMIE procedures were compared with 50 conventional minimally invasive esophagectomy (MIE) operations, which had been the standard treatment for esophageal cancer prior to the robotic era. Between April 2016 and March 2018, data of 100 consecutive patients with esophageal carcinoma undergoing modified Ivor Lewis esophagectomy were prospectively collected. All operations were performed by the same surgeon using an identical intrathoracic anastomotic reconstruction technique with the same perioperative management and pain control regimen. Intra-operative and postoperative complications were graded according to definitions stated by the Esophagectomy Complications Consensus Group. Data analysis was carried out with and without propensity score matching. Baseline characteristics did not show significant differences between the RAMIE and MIE group. Propensity score matching of the initial group of 100 patients resulted in two equal groups of 40 patients for each surgical approach. In the RAMIE group, the median total lymph node yield was 27 (range 13-84) compared to 23 in the MIE group (range 11-48), P = 0.053. Median intensive care unit (ICU) stay was 1 day (range 1-43) in the RAMIE group compared to 2 days (range 1-17) in the MIE group (P = 0.029). The incidence of postoperative complications was not significantly different between the two groups (P = 0.581). In this propensity-matched study comparing RAMIE to MIE, ICU stay was significantly shorter in the RAMIE group. There was a trend in improved lymphadenectomy in RAMIE.

Surgical resection of metastases to the adrenal gland: a single center experience.

BACKGROUND: Only limited data exist on the treatment and outcome of adrenal metastases that derive from different primary tumor entities. Due to the lack of evidence, it is difficult to determine the indication for surgical resection. METHODS: We assessed the outcome of 45 patients (28 men, 17 women) with adrenal metastases who underwent surgery (1990-2014). The median age at the time of adrenal surgery was 62 years (range 44-77 years). We were able to evaluate follow-up data of 41 patients. RESULTS: Primary tumor types were liver n = 12 (hepatocellular carcinoma n = 9, cholangiocellular carcinoma n = 2, sarcoma n = 1), upper GI tract n = 5 (esophagus n = 2, stomach n = 3), lung n = 9, kidney n = 6, neuroendocrine tumors n = 3, colon n = 2, ovarial n = 2, melanoma n = 2, others n = 4. The overall median survival time was 14 months (95 % CI 8.375-19.625). The survival rates at 1, 2, 5, and 10 years were 60, 31, 21, and 11 %, respectively. There were statistically significant differences in the survival time according to the resection status (R0 vs. R1/R2) (p < 0.001) and the type of the primary tumor (p = 0.009), while the metachronous or synchronous occurrence of adrenal metastases did not affect the prognosis. CONCLUSIONS: Resection of adrenal metastases can improve the survival if patients are carefully selected, the tumor is completely resected, and the intervention is integrated into a multidisciplinary oncologic treatment strategy.

Preoperative left ventricular hypertrophy is associated with reduced patient survival after liver transplantation.

UNLABELLED: Left ventricular hypertrophy (LVH) has been described in the context of cirrhotic cardiomyopathy. The influence of LVH on survival of liver transplant (LT) recipients has not been clarified. Therefore, we evaluated the effect of LVH on survival in LT recipients. In total, data from 352 LT patients were analyzed. LVH was diagnosed by echocardiographic measurement of left ventricular wall thickness before LT. Patients were followed up for a mean of 4.2 yr. LVH was diagnosed in 135 (38.4%) patients. Patients with LVH had significantly more frequently male gender (p = 0.046), diastolic dysfunction (p < 0.001), and hepatocellular carcinoma (HCC; p = 0.004). Furthermore, LVH patients were older (p < 0.001) and had a higher body mass index (BMI; p = 0.001). There was no difference in frequency of arterial hypertension, pre-transplant diabetes mellitus, or etiology of liver cirrhosis. Patients without LVH had a better survival (log rank: p = 0.05) compared with LVH patients. In a multivariate Cox regression LVH (p = 0.031), end-stage renal disease (ESRD; p = 0.003) and lack of arterial hypertension (p = 0.004) but not MELD score (p = 0.885) were associated with poorer survival. CONCLUSION: LVH is frequently diagnosed in patients on the waiting list and influences survival after LT.

[Hilar cholangiocarcinoma: right versus left hepatectomy]. / Hiläres Cholangiokarzinom: rechts- versus linksseitige En-bloc-Resektion.

INTRODUCTION: Right-sided hepatectomy including segment 1 and right trisectionectomy are typical approaches to surgical treatment of hilar cholangiocarcinoma. In this study we have compared the oncological capacity of this approach to left-sided hepatectomy. PATIENTS AND PROCEDURES: In 223 patients referred to our institution 150 hepatic resections were performed: 14 hilar resections, 68 right and 68 left hepatectomies. RESULTS: Survival after curative (R0) and palliative surgery was significantly superior to that in patient with exploration or no surgery at all (p < 0.0001). 5- and 10-year survival after right versus left hepatectomy was 29 and 22 % versus 21 and 7 % (p = 0.204). If hospital mortality was eliminated, survival after right hepatectomy was marginally significantly superior to that after left-sided hepatectomy (p = 0.041). Hospital mortality was 13 % after right compared to 4 % after left hepatectomy (p = 0.069). The R situation was of prognostic importance following right and the N situation after left hepatectomy (p = 0.038 and 0.01, respectively). Vascular resection - in right-sided procedures performed as "hilar en bloc resection" - did not influence the outcome. CONCLUSIONS: Low perioperative mortality after left-sided resection and, obviously, inferior oncological radicality are features of left hepatectomy. These features do not detract from the importance of left hepatectomy which is an indispensable approach to surgically treated patients with hilar cholangiocarcinoma.

Liver transplantation for hepatocellular carcinoma--is there a risk of recurrence caused by intraoperative blood salvage autotransfusion?

BACKGROUND/AIMS: The use of intraoperative blood salvage autotransfusion (IBSA) during surgical approaches may contribute to tumour cell dissemination. Therefore, IBSA should be avoided in cases of malignancy. However, the risks of IBSA might be acceptable in liver transplantation (LT) for selected small hepatocellular carcinoma (HCC). METHODS: In total, 136 recipients of LT with histologically proven HCC in the explanted liver were included in this analysis. With regard to tumour recurrence, 40 patients receiving IBSA despite HCC (IBSA group) were compared to 96 patients without IBSA (non-IBSA group). RESULTS: Milan criteria as assessed in the explanted liver were fulfilled in 24 of 40 IBSA patients and 58 of 96 non-IBSA patients (p = 0.85). Five of 40 patients in the IBSA group and 18 of 96 patients in the non-IBSA group experienced tumour recurrence (p = 0.29). In spite the theoretical risk of tumour cell dissemination, the recurrence rate was not increased in the IBSA group. CONCLUSION: Our results indicate that IBSA does not modify the risk of HCC recurrence. Therefore, in highly selected HCC patients undergoing LT, the use of IBSA appears to be justified.

Klatskin tumour: meticulous preoperative work-up and resection rate.

BACKGROUND: Surgery represents the only potentially curative treatment of hilar cholangiocarcinoma (hilCC). It may be suggested that meticulous preoperative work-up in Asian countries leads to higher resection rates. METHOD: One hundred and eighty-two patients treated in our department between 1998 and 2008 were included in an analysis based on our prospectively recorded database. Among them, 75 % had a percutaneous transhepatic cholangiography as part of their diagnostic work-up. A total of 160 patients underwent explorative surgery and 123 patients were resected (77 % of patients undergoing exploration, 68 % of all patients). RESULTS: Ninety-one percent of the patients were diagnosed to have Bismuth III and IV tumours. En-bloc resection of the tumour and the adjacent liver including segment 1 was the standard procedure in 109 of these patients, while hilar resection was performed in 14 patients. Upon tumour resection, hospital mortality was 5.7 %. Five-year survival in patients without surgery or with mere exploration was 0 %, after resection it reached 26 %. Patients with R 1 resection experienced longer survival than patients without resection (p < 0.001). Right and left hemihepatectomies were performed with identical frequency resulting in identical survival. Lymph node involvement proved to be the only significant predictor of prognosis (p = 0.006). CONCLUSION: Resection should be performed whenever possible since even after palliative resection survival is substantially increased compared to patients without resection. Meticulous preoperative work-up may contribute to a high resection rate in patients with hilCC by providing additional information allowing the surgeon to perform more aggressive approaches.

Hepatitis E Is a Rare Finding in Liver Transplant Patients With Chronic Elevated Liver Enzymes and Biopsy-Proven Acute Rejection.

BACKGROUND: In past decades, liver transplant (LT) patients were not routinely screened for hepatitis E virus (HEV) infection, and thus it might have been misdiagnosed as an acute rejection episode. Our aim was to analyze a real-world cohort of LT patients who presented with at least 1 episode of biopsy-proven acute rejection (BPAR) and suffered from persistent elevated transaminases, to evaluate the frequency of HEV infection misdiagnosed as a rejection episode. METHODS: Data from 306 patients transplanted between 1997 and 2017, including 565 liver biopsies, were analyzed. Biopsies from patients suffering from hepatitis C (n = 79; 25.8%) and from patients who presented with a Rejection Activity Index <5 (n = 134; 43.8%) were excluded. A subgroup of 74 patients (with 134 BPAR) with persistently elevated liver enzymes was chosen for further HEV testing. RESULTS: Positive HEV IgG was detectable in 18 of 73 patients (24.7%). Positive HEV RNA was diagnosed in 3 of 73 patients with BPAR (4.1%). Patients with HEV infection showed no difference in etiology of the liver disease, type of immunosuppression, or median Rejection Activity Index. CONCLUSION: Few HEV infections were misdiagnosed as acute rejection episodes in this real-world cohort. Thus, HEV infection is an infrequent diagnosis in cases with persistent elevated liver enzymes and BPAR after LT.

Pretransplant coronary artery disease is a predictor for myocardial infarction and cardiac death after liver transplantation.

BACKGROUND: Cardiovascular disease is a serious problem of liver transplant (LT) recipients because of increased cardiovascular risk due to immunosuppressive therapy, higher age, intraoperative risk and comorbidities (such as diabetes and nicotine abuse). Reported frequency of cardiovascular events after LT shows a high variability between different LT cohorts. Our aim was to analyze a cohort of LT recipients from a single center in Germany to evaluate frequency of the cardiovascular endpoints (CVE) myocardial infarction and/or cardiac death after LT and to investigate correlations of CVE post LT with pretransplant patient characteristics. PATIENTS: In total, data from 352 LT patients were analyzed. Patients were identified from an administrative transplant database, and all data were retrieved from patients' charts and reports. RESULTS: During the median follow-up of 4.0 (0-13) years, 10 cases of CVE were documented (six myocardial infarctions and four coronary deaths). The frequency of CVE did not differ according to classic cardiovascular risk factors such as body mass index (p=0.071), total cholesterol (p=0.533), hypertension (p=0.747), smoking (p=1.000) and pretransplant diabetes mellitus (p=0.146). In patients with pretransplant coronary heart disease (n=24; 6.8%) CVE were found more frequently (p=0.024). CONCLUSION: In summary, we found a rate of 2.8% CVE after LT in a German transplant cohort. Pretransplant CHD was the only risk factor for CVE, but showed no significant impact on overall survival.

Transarterial chemoembolization before liver transplantation in 60 patients with hepatocellular carcinoma.

Tumor recurrence is a major problem after orthotopic liver transplantation (OLT) in patients with hepatocellular carcinoma (HCC). In 60 patients OLT was performed for HCC after pretreatment by repeated transarterial chemoembolization (TACE). Forty-four recipients exceeded the Milan criteria. Recurrence-free 5-year survival was 65.2% and 5-year freedom from recurrence was 73.2%. During the waiting time, 14 patients experienced minimal change, which did not fulfill the definition of tumor progression according to official oncological criteria. Five-year freedom from recurrence among patients with stable compared with progressive disease was 93.3% versus 28.1%, respectively (P = .0001). A strict TACE pretreatment protocol may select patients with obviously biologically less aggressive tumors, who are suitable for OLT even if the HCC exceeds the commonly accepted listing criteria.

New onset of diabetes after transplantation is associated with improved patient survival after liver transplantation due to confounding factor.

BACKGROUND: The influence of NODAT on survival of liver transplant recipients has not been clarified. Therefore, we evaluated the effect of NODAT on survival in LT recipients. METHODS: Data from 352 LT patients were totally analyzed. 97 patients with pretransplant diabetes mellitus were excluded, and 255 patients without diabetes mellitus at time of transplantation were included. RESULTS: NODAT was diagnosed in 41 patients (16.1%). There was no difference in frequency of NODAT according to the etiology of liver cirrhosis. NODAT was associated with a higher body weight (p=0.004) and BMI (p=0.002) 5years after LT, but not with weight gain (p=0.201) or increase in BMI (p=0.335) 5years after LT. HbA1c 5years after LT was significantly higher in patients with NODAT (p=0.001), but mean HbA1c still remained lower than 6.5% (6.4(±1.2) %). Patients with NODAT showed better survival rates (log rank: p=0.002) compared to LT recipients without diabetes. According to all existing knowledge of diabetes mellitus (DM) better survival cannot be a direct effect of this disease. Our results are rather influenced by an not known confounding factor (possibly recovery from cachexia) associated with better survival and NODAT, while complications of NODAT will not appear during the relatively short postoperative time and observation period (mean follow up 6.08 (±2.67) years). CONCLUSION: NODAT is frequently diagnosed in LT recipients and is associated with an improved 5year survival after LT due to a not exactly known confounding factor.

[Hilar cholangiocarcinoma -- results of en bloc resection of tumor and liver]. / Hiläres CholangiokarzinomErgebnisse bei En-bloc-Resektion von Tumor und Leber.

The increasingly performed en bloc resection of liver and hilar tumor has contributed to the improvement of long-term survival in patients with hilar cholangiocarcinoma. Based on preoperative definition of operative strategy we tried to avoid any traumatization of the hilar region. Between September 1997 and September 2002, 82 patients with hilar cholangiocarcinoma were treated at our department. Three patients were excluded from any surgery. The resection rate was 75% (59 of 79); 79% (38 of 48) of en bloc resections of the hilar tumor and adjacent liver were formally curative. The hospital mortality was 7%. The 1- and 3-year survival rates of patients after explorative laparotomy, palliative and curative resection was 27 and 7%, 67 and 26%, 89 and 45% ( p<0.001), respectively. The 1- and 3-year survival rates of patients after en bloc resection were 78 and 49%, respectively. In patients with formally curative en bloc resection ( n=38), the 3-year survival rate was 63%; in patients with N0/R0 resection ( n=31) it was 71%. Lymph node involvement proved to be the only independent prognostic marker if patients who underwent hilar and en bloc resection were included in the multivariate analysis. The R situation was the only significant predictor for patients after en bloc resection. These data justify the extended diagnostic work-up and the principal liver resection in hilar cholangiocarcinoma.

Tacrolimus effects and side effects after liver transplantation: is there a difference between immediate and extended release?

BACKGROUND: Immunosuppressive therapy after orthotopic liver transplantation (OLT) requires a high degree of patient compliance to guarantee safety and avoid side effects. In 2007, prolonged-release tacrolimus was launched in Europe to improve compliance. In this prospective observational crossover single-center trial, we analyzed effects and side effects of prolonged-release tacrolimus in OLT patients. METHODS: LT patients at our center were included if they were older than l8 years of age, had had the procedure at least 6 months prior, and were outpatients currently on twice-daily tacrolimus. Patients were observed for 6 months before switching to once-daily tacrolimus. Patient history, clinical examination, and laboratory examinations were recorded on inclusion as well as after 3, 6, 9, 12, and 18 months. RESULTS: The rates of rejection, hypertension, hypercholesterolemia, and diabetes mellitus were compared during twice-daily and once-daily tacrolimus. Similarly, laboratory parameters were identical during both periods with the exception of glycated hemoglobin, which was significantly elevated under once-daily tacrolimus (P = .00l). CONCLUSION: Converting patients to extended-release tacrolimus with was safe in terms of rejection, hypertension, and hypercholesterolemia as well as renal and liver functions. Further investigations concerning pharmacokinetics and glucose metabolism will be needed to evaluate prolonged-release tacrolimus.

Comparison of resection and transarterial chemoembolisation in the treatment of advanced intrahepatic cholangiocarcinoma--a single-center experience.

AIMS: The aim of this study is to evaluate factors associated with the outcome after surgical resection and to compare the efficacy of surgery to transarterial chemoembolisation (TACE) in patients with advanced intrahepatic cholangiocarcinoma (IHC). MATERIALS AND METHODS: 273 patients with IHC treated in our department between 1997 and 2012 were included in our study. Patients were divided according to therapy into surgical (n = 130), TACE (n = 32), and systemic chemotherapy/best supportive care (n = 111) groups. Clinicopathological characteristics and survival were reviewed retrospectively. RESULTS: The 1-, 3-, and 5-year survival rates in patients after surgical resection were 60%, 40%, and 23%, respectively. Recurrence occurred in 63 percent of patients after R0 resection. Median time of recurrence-free survival was 14 months. Univariate analysis revealed nine significant risk factors for overall survival in the resection group: major surgery, extrahepatic resection, vascular and bile duct resection, lymph node invasion, poor tumour differentiation, positive surgical margin, multiple lesions, tumour diameter, and UICC-Stage. Multivariate analysis showed that lymph node metastasis (P < 0.001), poor tumour differentiation (P = 0.002), and positive resection margins (P = 0.001) were independent prognostic factors for survival. Median survival as well as overall survival rates of TACE patients were comparable to those of lymph node positive patients and patients with tumour positive surgical margins. CONCLUSIONS: R0 resection in patients with negative lymph node status remains the best chance for long-term survival in patients with IHC. There is no significant survival benefit of surgery in lymph node positive patients or patients with positive resection margin over TACE.

Ten years of simultaneous pancreas-kidney transplantation: a retrospective single-center analysis of prospectively obtained data.

INTRODUCTION: Simultaneous pancreas-kidney transplantation (SPK) is a standardized and life-saving procedure for a patient suffering from both insulin-dependent diabetes mellitus type 1 (IDDM 1) and end-stage diabetic nephropathy. To expand the donor pool and to determine the influence of the preprocurement pancreas suitability scoring system (P-PASS) on pancreas graft survival we retrospectively analyzed our data on SPK. PATIENTS AND METHODS: From 1999 to 2010 we performed 55 SPKs, using systemic-enteric drainage as surgical approach. The immunosuppressive therapy was induced with basiliximab; maintenance therapy was based on tacrolimus, mycophenolate mofetil, and steroids. Data were prospectively obtained, analyzed, and correlated to the P-PASS. RESULTS: The overall 10-year patient survival rate was 78% with a 10-year pancreas survival rate of 53%. Three patients needed retransplantation of SPK and 6 patients needed singular pancreas retransplantation. Seventeen patients showed acute rejection episodes and 14 patients suffered from cytomegalovirus (CMV) infections. We compared 43 patients receiving organs from an "ideal" donor (P-PASS <17) with 12 patients receiving grafts from "marginal" donors (P-PASS ≥17). Neither P-PASS nor donor age demonstrated significant influence on pancreas graft survival. However, the body mass index (BMI) of the donor showed a negative tendency (P = .059). CONCLUSION: The P-PASS showed no significant prediction of pancreas graft survival. In view of our data, expansion of the German donor pool is possible. A multicenter study of SPK using "marginal" pancreas grafts is mandatory to define a realistic "cut-off" value for P-PASS.

Organ recipients suffering from undifferentiated neuroendocrine small-cell carcinoma of donor origin: a case report.

BACKGROUND: Transmission of donor-derived cancer by organ transplantation is rare, but the risk has been increasing due to the aging donor pool. Undifferentiated neuroendocrine small-cell carcinoma is an aggressive tumor with the tendency to spread. Herein we have demonstrated different approaches to treat organ recipients with transmitted tumors. METHODS AND RESULTS: Grafts were retrieved from a decreased donor without any history of previous diseases. Autopsy was not performed after donation. The recipient of the liver graft presented with suspected nodules on routine abdominal ultrasound. After computed tomography (CT) scan, biopsy confirmed the diagnosis of a small-cell carcinoma. Donor origin was unequivocally identified by DNA fingerprinting. Despite chemotherapy the patient died 7 months after orthotopic liver transplantation (OLT). All involved transplantation centers were informed immediately following diagnosis. The male kidney recipient underwent detailed diagnostic work-up to exclude tumor transmission. One year after transplantation, liver metastases caused by a histologically proven small-cell carcinoma from the same donor were apparent. Chemotherapy was immediately started and the graft was removed. Despite continued treatment the tumor progressed and the patient died after repeated intestinal complications. The pathological examination of the explanted second kidney graft did not show any tumor infiltration. CONCLUSION: Therapeutic regimens in recipients suffering from donor-derived carcinoma differ depending on the transplanted organ. Graft removal of non-life-sustaining organs and discontinuation of immunosuppressive medication should result in complete tumor rejection. Minimizing the risk of tumor transmission, a CT scan might be advisable in donors of more advanced age.

Risk factors in patients with rapid recurrent hepatitis C virus-related cirrhosis within 1 year after liver transplantation.

BACKGROUND: Recurrent cirrhosis (RC) due to pretransplant underlying disease leads to organ failure and subsequent death after orthotopic liver transplantation (OLT). RC occurs in up to 30% of patients with recurrent hepatitis C (HCV) within 5 years after OLT. We sought to identify early risk factors for rapid RC within the first year after OLT in HCV-positive patients. METHODS: Among 404 liver transplanted patients at the University of Mainz between 1998 and 2008, 90 were HCV-RNA positive. To identify predictive factors for rapid RC, we compared HCV-positive patients with advanced fibrosis stages within 1 year after OLT (n = 13) with these without RC at 5 years after OLT (n = 23). RESULTS: Overall, poorer patient survival was associated with advanced fibrosis scores in the 1-year protocol biopsy and nonresponse to interferon treatment before OLT. The strongest predictive factors for rapid RC were persistently high levels of alkaline phosphatase, bilirubin, viral load at 6 months after OLT, and multiple steroid pulse therapies. The CCR2-V64I polymorphism was not associated with rapid RC. CONCLUSION: We presented a group of patients with HCV-related rapid RC within the first year after OLT to identify predictive factors for rapid fibrosis progression.

[The solid pseudopapillary tumor (SPT)--a rare neoplasm of the pancreas]. / Der solid pseudopapilläre Pankreastumor (SPT)--eine seltene Raumforderung der Bauchspeicheldrüse.

BACKGROUND: In general, the rare SPT is a tumour of low malignancy predominantly affecting young women. The outcome after radical resection is favourable. In exceptional cases the tumour presents as solid pseudopapillary carcinoma (SPC) with typical malignant features and even metastases. Unresectable liver metastases can be treated with RFA, TACE or chemotherapy. METHODS: We retrospectively reviewed the surgical approach, immunohistochemistry and clinical outcome in five female patients (1998--2007). RESULTS: The mean age was 16 years (range: 13-47 years). For radical tumour removal a pancreato-duodenectomy (n = 3), a distal pancreatectomy (n = 1) and an enucleation (n = 1) were performed. We encountered a mean tumour diameter of 8 cm (range: 6-15 cm), an angioinvasion (3/5) and a lymphatic infiltration (1/5). After 30 to 101 months follow-up four patients were free of recurrence. Chemotherapy has resulted in a survival of over 98 months in a case of SPC with liver metastases. CONCLUSION: SPT is a tumour of low malignancy. Radical resection is recommended for long-term recurrence-free survival. Chemotherapy may prolong survival in SPC with unresectable metastases.