Liver transplantation in hepatocellular carcinoma - should we perform downstaging?

AIM: To compare the long-term outcomes between liver transplant (LT) recipients with hepatocellular carcinoma (HCC) who were downstaged with transarterial-chemoembolization (TACE) to the Milan criteria (MC) and those initially meeting the MC. METHODS: This retrospective study enrolled 198 patients with HCC: 38 were downstaged and 160 patients initially met the MC. Post-LT survival and HCC recurrence-free survival were evaluated. We assessed the association of death and HCC recurrence with TACE, baseline (age, sex, disease etiology, Model of End-stage Liver Disease, tumor number and the sum of maximum tumor diameters, waiting time, alpha-fetoprotein level) and explant characteristics (tumor number and the sum of maximum tumor diameters, micro- and macrovascular invasion). RESULTS: The recipient survival rates one, three, and five years after LT were 88.2%, 80.1%, and 75.9%, respectively. HCC recurrence-free probabilities were 92.3%, 87.9%, and 85%, respectively. The outcomes were comparable between the groups. In multivariate analysis, the number of tumors on the explant, age, and tumor recurrence were independent risk factors for death. Only the sum of maximum tumor diameters on the explant was an independent risk factor for HCC recurrence. CONCLUSIONS: Patients successfully downstaged with TACE to the MC can achieve post-LT recipient and HCC recurrence-free survival comparable with patients initially within the MC. Good response to TACE as a criterion for LT may be a method of selecting patients with favorable biological characteristics.

Liver transplantation in patients with neuroendocrine tumors: a case series and literature review.

Neuroendocrine tumors (NET) are a rare and heterogeneous group of neoplasms with variable biological behavior. They frequently metastasize to the liver, requiring active, multimodality treatment. Surgical resection, possible in only a minority of cases, was until recently the only potentially curative option. For unresectable NET with liver metastases, liver transplantation (LT) emerged as a potential curative treatment due to relatively slow growth and indolent behavior of the metastases. In this case series with literature review, we retrospectively analyzed the characteristics of 12 highly selected patients with metastatic NET disease as an indication for LT treated in our center. We also summarized the proposed prognostic factors, and evaluated and compared the existing selection criteria. The main poor prognostic factors in our patients were high grade NET and primary tumor in the pancreas. Inconsistent liver transplantation outcome parameters make it difficult to standardize patient selection criteria. There is a need for further studies that would fully elucidate the curative potential of LT in patients diagnosed with NET.

THE COST OF KIDNEY TRANSPLANTATION AT THE MERKUR UNIVERSITY HOSPITAL, ZAGREB, CROATIA.

The aim was to determine the cost of hospitalization for a transplant procedure and identify the independent variables associated with the cost of transplantation. The investigation was designed as a retrospective single-center cohort study conducted at a tertiary university hospital transplant center in Zagreb, Croatia. The study included 219 consecutive kidney recipients transplanted during the 2007-2013 period at the Merkur University Hospital. There were 141 male and 78 female patients having undergone kidney transplantation during the study period. The majority of kidney transplants were from a deceased donor (n=179), while 40 were from a living donor. The mean cost of a transplantation was 86,140±42,240 HRK (11,460±5,600 €), ranging from 29,000 HRK (3,860 €) to 408,000 HRK (54,000 €). In the bivariate analysis, the variables associated with the cost of transplantation were the length of hospital stay, delayed graft function, death of the patient, graft loss, use of steroids, and death-censored graft loss. In the multivariate analysis, delayed graft function was the only statistically significant variable for the cost of transplantation. Since only delayed graft function had an impact on the cost of transplantation in this study, certain steps such as shortening of the cold ischemia time (better organization of organ transport), better education of family members for living donors, and higher percentage of patients on peritoneal dialysis should be taken to lower the percentage of delayed graft function.

The Intention-to-Treat Effect of Bridging Treatments in the Setting of Milan Criteria-In Patients Waiting for Liver Transplantation.

In patients with hepatocellular carcinoma (HCC) meeting the Milan criteria (MC), the benefit of locoregional therapies (LRTs) in the context of liver transplantation (LT) is still debated. Initial biases in the selection between treated and untreated patients have yielded conflicting reported results. The study aimed to identify, using a competing risk analysis, risk factors for HCC-dependent LT failure, defined as pretransplant tumor-related delisting or posttransplant recurrence. The study was registered at www.clinicaltrials.gov (identification number NCT03723304). In order to offset the initial limitations of the investigated population, an inverse probability of treatment weighting (IPTW) analysis was used: 1083 MC-in patients (no LRT = 182; LRT = 901) were balanced using 8 variables: age, sex, Model for End-Stage Liver Disease (MELD) value, hepatitis C virus status, hepatitis B virus status, largest lesion diameter, number of nodules, and alpha-fetoprotein (AFP). All the covariates were available at the first referral. After the IPTW, a pseudo-population of 2019 patients listed for LT was analyzed, comparing 2 homogeneous groups of untreated (n = 1077) and LRT-treated (n = 942) patients. Tumor progression after LRT was the most important independent risk factor for HCC-dependent failure (subhazard ratio [SHR], 5.62; P < 0.001). Other independent risk factors were major tumor diameter, AFP, MELD, patient age, male sex, and period of wait-list registration. One single LRT was protective compared with no treatment (SHR, 0.51; P < 0.001). The positive effect was still observed when 2-3 treatments were performed (SHR, 0.66; P = 0.02), but it was lost in the case of ≥4 LRTs (SHR, 0.80; P = 0.27). In conclusion, for MC-in patients, up to 3 LRTs are beneficial for success in intention-to-treat LT patients, with a 49% to 34% reduction in failure risk compared with untreated patients. This benefit is lost if more LRTs are required. A poor response to LRT is associated with a higher risk for HCC-dependent transplant failure.

Influence of donor age on liver transplantation outcomes: A multivariate analysis and comparative study.

BACKGROUND: The growing disparity between the rising demand for liver transplantation (LT) and the limited availability of donor organs has prompted a greater reliance on older liver grafts. Traditionally, utilizing livers from elderly donors has been associated with outcomes inferior to those achieved with grafts from younger donors. By accounting for additional risk factors, we hypothesize that the utilization of older liver grafts has a relatively minor impact on both patient survival and graft viability. AIM: To evaluate the impact of donor age on LT outcomes using multivariate analysis and comparing young and elderly donor groups. METHODS: In the period from April 2013 to December 2018, 656 adult liver transplants were performed at the University Hospital Merkur. Several multivariate Cox proportional hazards models were developed to independently assess the significance of donor age. Donor age was treated as a continuous variable. The approach involved univariate and multivariate analysis, including variable selection and assessment of interactions and transformations. Additionally, to exemplify the similarity of using young and old donor liver grafts, the group of 87 recipients of elderly donor liver grafts (≥ 75 years) was compared to a group of 124 recipients of young liver grafts (≤ 45 years) from the dataset. Survival rates of the two groups were estimated using the Kaplan-Meier method and the log-rank test was used to test the differences between groups. RESULTS: Using multivariate Cox analysis, we found no statistical significance in the role of donor age within the constructed models. Even when retained during the entire model development, the donor age's impact on survival remained insignificant and transformations and interactions yielded no substantial effects on survival. Consistent insignificance and low coefficient values suggest that donor age does not impact patient survival in our dataset. Notably, there was no statistical evidence that the five developed models did not adhere to the proportional hazards assumption. When comparing donor age groups, transplantation using elderly grafts showed similar early graft function, similar graft (P = 0.92), and patient survival rates (P = 0.86), and no significant difference in the incidence of postoperative complications. CONCLUSION: Our center's experience indicates that donor age does not play a significant role in patient survival, with elderly livers performing comparably to younger grafts when accounting for other risk factors.

Use of machine learning models for identification of predictors of survival and tumour recurrence in liver transplant recipients with hepatocellular carcinoma.

Background: Hepatocellular carcinoma (HCC) is one of the leading indications for liver transplantation (LT) however, selection criteria remain controversial. We aimed to identify survival factors and predictors for tumour recurrence using machine learning (ML) methods. We also compared ML models to the Cox regression model. Methods: Thirty pretransplant donor and recipient general and tumour specific parameters were analysed from 170 patients who underwent orthotopic liver transplantation for HCC between March 2013 and December 2019 at the University Hospital Merkur, Zagreb. Survival rates were calculated using the Kaplan-Meier method and multivariate analysis was performed using the Cox proportional hazards regression model. Data was also processed through Coxnet (a regularized Cox regression model), Random Survival Forest (RSF), Survival Support Vector Machine (SVM) and Survival Gradient Boosting models, which included pre-processing, variable selection, imputation of missing data, training and cross-validation of the models. The cross-validated concordance index (CI) was used as an evaluation metric and to determine the best performing model. Results: Kaplan-Meier curves for 5-year survival time showed survival probability of 80% for recipient survival and 82% for graft survival. The 5-year HCC recurrence was observed in 19% of patients. The best predictive accuracy was observed in the RSF model with CI of 0.72, followed by the Survival SVM model (CI 0.70). Overall ML models outperform the Cox regression model with respect to their limitations. Random Forest analysis provided several relevant outcome predictors: alpha fetoprotein (AFP), donor C-reactive protein (CRP), recipient age and neutrophil to lymphocyte ratio (NLR). Cox multivariate analysis showed similarities with RSF models in identifying detrimental variables. Some variables such as donor age and number of transarterial chemoembolization treatments (TACE) were pointed out, but these were not influential in our RSF model. Conclusions: Using ML methods in addition to classical statistical analysis, it is possible to develop sufficient prognostic models, which, compared to established risk scores, could help us quantify survival probability and make changes in organ utilization.

[Hepatocellular carcinoma initially diagnosed by fine needle aspiration cytology of the pelvic bone metastasis]. / Hepatocelularni karcinom primarno dijagnosticiran citoloskom punkcijom metastatskog procesa u zdjelicnoj kosti.

Hepatocellular carcinoma mostly develops in patients with liver cirrhosis due to chronic hepatitis C virus (HCV) infection. A case is presented of a patient with hepatorenal syndrome as a sequel of liver cirrhosis due to HCV infection. Primary tumor of the liver was not diagnosed by routine procedures, but by fine needle aspiration cytology of the extensive osteolytic lesion of the pelvic bone, performed as part of the pre-transplantation workup. Transplantation procedure was abandoned because of inappropriate donor liver (hepatic artery thrombosis), and palliative pain-relieving irradiation was recommended. However, hepatic coma developed very rapidly and the patient died within a month of the diagnosis of metastatic hepatocellular carcinoma. Although hepatocellular carcinoma metastases are not rare, massive bone infiltration from a primary tumor undetectable by routine methods is not frequently encountered.

[Multifocal epithelioid hemangioendothelioma treated by liver transplantation--case report]. / Multifokalni epiteloidni hemangioendoteliom jetre lijecen ortotopnom transplantacijom jetre--prikaz bolesnika.

Multifocal epithelioid hemangioendothelioma of the liver is a rare primary tumor with a variable course of disease. A case is presented of a 27-year-old female patient with multiple hepatic lesions on ultrasonography, suspect of metastatic tumor of the liver. Serum tumor markers were not elevated, while clinical examination of the lungs, gastrointestinal and gynecologic systems did not confirm the presence of a primary tumor process. Metastatic tumor and primary hepatocellular tumor were ruled out by fine needle aspiration cytology. Along with a characteristic immunophenotype of the vascular cell endothelium (positive for CD31 and CD34), high proliferation demonstrated by the analysis of argyrophilic nucleolar organization regions (AgNOR) and DNA aneuploidy, cytomorphological pattern suggested the diagnosis of angiosarcoma. Histopathologic finding corresponded to epithelioid hemangioendothelioma. Ten years after orthotopic liver transplantation, the patient is free from disease relapse, with regular follow up testing. Hemangioendothelioma of the liver is characterized by multifocality, which excludes resection; thus, liver transplantation is the method of choice. Therefore, preoperative diagnostic workup is of utmost importance to differentiate it from other primary and metastatic tumors of the liver.

[Post-transplant lymphoproliferative disease in liver transplant recipients--Merkur University Hospital single center experience]. / Post-transplantacijska limfoproliferativna bolest u bolesnika s transplantacijom jetre - iskustvo KB Merkur.

Post-transplant lymphoproliferative disorder (PTLD) is an increasingly recognized condition as the number of solid organ and bone marrow transplant recipients increases. It can be a life threatening fulminant disorder and affects approximately 8% of solid organ transplant recipients. Epstein-Barr virus (EBV) is closely involved in the pathogenesis of PTLD and the majority of PTLD cases arise in response to primary infection with EBV or to re-activation of previously acquired EBV. The principal risk factors underlying the development of PTLD are the degree of overall immunosuppression and EBV serostatus of the recipient. The most commonly used pathologic classification of PTLD is the World Health Organization classification, which divides PTLD into three categories: early lesions, polymorphic PTLD, and monomorphic PTLD. Early lesions are characterized by reactive plasmacytic hyperplasia. Polymorphic PTLD may be either polyclonal or monoclonal and is characterized by destruction of the underlying lymphoid architecture, necrosis, and nuclear atypia. In monomorphic PTLD, the majority of cases (>80%) arise from B cells, similar to non-Hodgkin's lymphoma in immunocompetent hosts. The most common subtype is diffuse large B-cell lymphoma, but Burkitt's/Burkitt's-like lymphoma and plasma cell myeloma are also seen. Rarely T-cell variants occur, which include peripheral T-cell lymphomas and, rarely, other uncommon types, including gamma/delta T-cell lymphoma and T-natural killer (NK) cell varieties. Hodgkin's disease-like lymphoma is very unusual. An accurate diagnosis of PTLD requires a high index of suspicion, since the disorder may present subtly and/or extranodally. Radiologic evidence of a mass or the presence of elevated serum markers (such as increased LDH levels) are suggestive of PTLD, with positive finding on ultrasonography, computed tomography, magnetic resonance and/or positron emission tomography scanning (possibly indicating metabolically active areas) also favoring the diagnosis. The management of PTLD poses a major therapeutic challenge and although there is reasonable agreement about the overall principles of treatment, there is still considerable controversy about the optimal treatment of individual patients. EBV-related PTLDs are a significant cause of mortality in patients undergoing orthotopic liver transplantation with the observed mortality rate of up to 50%. This paper presents the experience acquired at Merkur University Hospital in the diagnosis and treatment of patients with liver transplantation and PTLD.

Dual kidney transplantation: case report.

Chronic shortage of kidney transplants worldwide has led to the use of organs from so called marginal or borderline donors, now termed "expanded-criteria donors". There has been an emerging practice of dual kidney transplantation (DKT) to compensate for sub optimal nephron mass of such kidneys. We performed DKT in "Merkur" University Hospital in August 2005. The donor was a 72-year old female with a history of long-term hypertension, aneurysm of the posterior cerebral artery, cerebrovascular insult (CVI), and with normal creatinine values and kidney function at the time of explantation. Initial biopsy of donor kidneys revealed acute tubular damage, with connective changes in 22% and 11% of glomeruli in the left and the right kidney, respectively. The recipient was a 60-year old male diagnosed with the IgA nephropathy on the last biopsy in 1999, and on dialysis since November 2003. Postoperative course was uneventful without any surgical complications. A triple immunosuppressive protocol was used. On follow-up ultrasonography 4 years posttransplantation both kidneys appeared of normal size and parenchymal pattern and with no signs of dilatation of the canal system, and color Doppler examination demonstrated normal flow in both kidneys. In conclusion, the use of DKT ie. donors by the expanded-criteria will continue to increase, and further studies of the results will, with no doubt, support this method.

Fine needle aspiration cytology of adrenocortical carcinoma--case report.

A 49-year-old woman presented for hirsutism, deep voice and hypertension. Ultrasonography (US) revealed a solitary tumor mass, eight cm in size, of the right adrenal gland. Laboratory tests showed it to be a hormonally active, androgen secreting tumor (elevated testosterone level), which was consistent with the clinical picture of the disease. After histopathological analysis tumor was signed out as adrenocortical carcinoma, a low risk carcinoma according to Weiss' classification. One year later on regular follow up, US revealed a suspicious growth measuring 65 x 43 mm in the projection of the lower pole of the right kidney. The finding was verified by computerized tomography and the patient was reoperated on. Exploration revealed secondary growth in the region of greater omentum, without infiltration of adjacent organs. Histopathologic analysis confirmed metastatic ACC. 8 months after the second operation and after 6 chemotherapy cycles according to EAP protocol, control CT showed enlarged para-aortic lymph nodes and a node along the upper pole of the right kidney. Cytologic puncture was performed. Cytologic opinion was recidive of primary malignant disease. ACC is a rare malignant epithelial tumor of adrenal cortical cells, with high malignant potential. Morphologically (histopathology and cytology), differential diagnosis includes adenoma on the one hand, and renal cell carcinoma (RCC) and hepatocellular carcinoma (HCC) on the other hand. A combined evaluation of clinical features, size or weight, microscopic appearance, immunohistochemical and molecular genetic data is necessary to ensure a correct diagnosis. The purpose of this case report is to present clinical and cytomorphologic features of our case of adrenocortical carcinoma which is very rare in cytology practice.

Epitheloid hemangioendothelioma in patient with liver transplantation.

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown aetiology and a variable natural course. At the time of diagnosis, most patients present with multifocal tumours lesions that involve both liver lobes. From the therapeutic aspect, liver resection (LRx), liver transplantation (LTx), chemotherapy, radiotherapy, and/or immunotherapy have been used in the treatment of patients with HEH. However, because of the rarity of this tumor and its unpredictable natural history, it is impossible to assess the effectiveness of these respective therapies. In this report, our objective was to present clinical aspects, diagnostic options, therapeutic modalities, and the clinical outcome of single patient with LTx because of this rare tumor.

Parvovirus B 19 (PVB19) induced pure red cell aplasia (PRCA) in immunocompromised patient after liver transplantation.

Presented here is a case of human parvovirus B19 (PVB19) induced pure red-cell aplasia (PRCA) in immunocompromised patient after orthotopic liver transplantation (OLT). PVB19 is a small, single-stranded DNA whose target cell is the erythroid progenitor in bone marrow. Manifestations of PVB19 infection vary with the immunologic status of the patient, ranging from asymptomatic to severe infections and PRCA. Post-transplant PRCA is induced either by immunosuppressive agents or PVB19. In the presented case, bone marrow aspiration characterized by the absence of mature erythroid precursors and detection of PVB19 DNA in blood led to treatment with high-dose intravenous human immunoglobulins (IVIG) and subsequent recovery of erythropoiesis. Due to insufficient antibody response in immunocompromised patients, suppression of the PVB19 infection is delayed and repetitive treatments may be administrated in attempt of reversing PRCA.

Pre- and intraoperative predictors of acute kidney injury after liver transplantation.

BACKGROUND: Acute kidney injury (AKI) after liver transplantation (LT) is a frequent and multifactorial event related to increased morbidity and mortality. Risk factors for AKI after LT still need to be clarified. AIM: To identify the predictors of acute kidney injury after liver transplantation. METHODS: The frequency and pre- and intraoperative predictors of AKI within the first 7 d after LT were evaluated in adult liver transplant candidates in a single LT center in Croatia. AKI was defined according to the Kidney Disease: Improving Global Outcomes criteria. RESULTS: Out of 205 patients (mean age 57 ± 10 years; 73.7% males, 52.7% with alcohol-related liver disease) 93 (45.36%) developed AKI, and the majority of them (58.06%) had stage 1. Only 5.38% of patients required renal replacement therapy after LT. The majority of patients (82.8%) developed AKI within the first two days after the procedure. Multivariate logistic regression identified pre-LT body mass index (OR = 1.1, 95%CI: 1.05-1.24) and red blood cell transfusion (OR = 1.66, 95%CI: 1.09-2.53) as independent predictors of early post-LT AKI occurrence. 30-d survival after LT was significantly better for patients without AKI (P = 0.01). CONCLUSION: Early AKI after LT is a frequent event that negatively impacts short-term survival. The pathogenesis of AKI is multifactorial, but pre-LT BMI and intraoperative volume shifts are major contributors.

Adrenalectomy for Bilateral Metachronous Adrenal Recurrence of Hepatocellular Carcinoma After Liver Transplant: A Case Report.

Hepatocellular carcinoma is the fifth most common cancer in the world and the third leading cause of cancer-related death. It is currently one of the leading indications for liver transplant, with selected 5-year survival rates after liver transplant of about 70%. Despite excellent results of liver transplant for hepatocellular carcinoma, a number of patients develop metastases after transplant, and multifocal metastatic disease is the most frequent cause of death. In a large autopsy series of patients with hepatocellular carcinoma, adrenal glands were the third most common site of extrahepatic metastasis after lungs and bones. However, isolated metastatic disease in the adrenal glands is rare, and isolated metachronous bilateral metastasis is an even rarer occurrence. Only few reports have been published of metachronous bilateral metastasis of hepatocellular carinoma after liver transplant treated with bilateral adrenalectomy. We describe a case of a 56-year-old man who underwent liver transplant for hepatocellular carcinoma in a cirrhotic liver. Two years after liver tranplant, regular follow-up revealed metastatic disease in the left adrenal gland. Preoperative imaging showed no other metastasis, and he underwent an uneventful left adrenalectomy. A year after surgery, he presented with right flank pain and tenderness. Imaging showed hemorrhage and tumor involvement of the right adrenal gland, and he underwent right adrenalectomy. Two years after surgery, he is alive and well with no signs of disease recurrence. Apparently, in the absence of intrahepatic or other metastases, bilateral metachronous recurrence of hepatocellular carcinoma after liver tranplant can be a good surgical indication with acceptable long-term survival.

Liver re-transplantation for donor-derived neuroendocrine tumor: A case report.

BACKGROUND: Donor-origin cancer is a well-recognized but rare complication after liver transplantation (LT). The rise in the use of extended criteria donors due to the current shortage of organs increases the risk. Data on donor-origin neuroendocrine neoplasms (NENs) and the most appropriate treatment are scarce. Here, we report a case of a patient who developed a NEN confined to the liver after LT and was treated with liver re-transplantation (re-LT). CASE SUMMARY: A 49-year-old man with no other medical co-morbidities underwent LT in 2013 for alcoholic liver cirrhosis. The donor was a 73-year-old female with no known malignancies. Early after LT, a hypoechogenic (15 mm) lesion was detected in the left hepatic lobe on abdominal ultrasound. The lesion was stable for next 11 mo, when abdominal magnetic resonance identified two hypovascular lesions (20 and 11 mm) with atypical enhancement pattern. Follow-up abdominal ultrasound revealed no new lesions for the next 2.5 years, when magnetic resonance showed a progression in size and number of lesions, also confirmed by abdominal computed tomography. Liver biopsy proved a well-differentiated NEN. Genetic analysis of the NEN confirmed donor origin of the neoplasm. As NEN was confined to liver graft only, in 2018, the patient underwent his second LT. At 12 mo after re-LT the patient is well with no signs of NEN dissemination. CONCLUSION: The benefits of graft explantation should be weighed against the risks of re-LT and the likelihood of NEN dissemination beyond the graft.

Constrictive pericarditis as a cause of refractory ascites after liver transplantation: A case report.

BACKGROUND: Refractory ascites is a rare complication following orthotopic liver transplantation (OLT). The broad spectrum of differential diagnosis often leads to delay in diagnosis. Therapy depends on recognition and treatment of the underlying cause. Constrictive pericarditis is a condition characterized by clinical signs of right-sided heart failure. In the advanced stages of the disease, hepatic congestion leads to formation of ascites. In patients after OLT, cardiac etiology of ascites is easily overlooked and it requires a high degree of clinical suspicion. CASE SUMMARY: We report a case of a 55-year-old man who presented with a refractory ascites three months after liver transplantation for alcoholic cirrhosis. Prior to transplantation the patient had a minimal amount of ascites. The transplant procedure and the early postoperative course were uneventful. Standard post-transplant work up failed to reveal any typical cause of refractory post-transplant ascites. The function of the graft was good. Apart from atrial fibrillation, cardiac status was normal. Eighteen months post transplantation the patient developed dyspnea and severe fatigue with peripheral edema. Ascites was still prominent. The presenting signs of right-sided heart failure were highly suggestive of cardiac etiology. Diagnostic paracentesis was suggestive of cardiac ascites, and further cardiac evaluation showed typical signs of constrictive pericarditis. Pericardiectomy was performed followed by complete resolution of ascites. On the follow-up the patient remained symptom-free with no signs of recurrent ascites and with normal function of the liver graft. CONCLUSION: Refractory ascites following liver transplantation is a rare complication with many possible causes. Broad differential diagnosis needs to be considered.

Role of total pancreatectomy in the treatment of paraduodenal pancreatitis: A case report.

BACKGROUND: Paraduodenal pancreatitis (PP) is a rare form of chronic pancreatitis presenting with symptoms of duodenal obstruction. Conservative treatment is often unsuccessful and pancreaticoduodenectomy is the preferred surgical approach. A mini review of the outcomes of surgical therapy for PP shows that the results of pancreaticoduodenectomy are predominantly favorable. CASE SUMMARY: In our case report of PP, we describe an unusual course first presenting with the symptoms of chronic pancreatitis and a pseudocyst of the pancreatic tail. A pseudocystojejunostomy was performed and the late postoperative course was complicated with the symptoms of duodenal obstruction. At laparotomy, PP was found and the patient was treated with a total pancreatectomy. The postoperative course was uneventful and good weight gain with resolution of pain was demonstrated at follow up visits. CONCLUSION: Surgery is currently the optimal treatment option for PP. It is also the best diagnostic tool in distinguishing between pancreatitis and pancreatic adenocarcinoma.