A Rare Inflammatory Myofibroblastic Tumor of the Spleen: A Case Report.

BACKGROUND/AIM: Inflammatory myofibroblastic tumors (IMTs) are rare, solid, potentially malignant lesions of uncertain etiology. Histologically, IMTs exhibit a combination of lymphocytes and inflammatory cells within a fibroblastic myxoid layer. The diagnosis of IMTs poses a challenge for various medical specialties, including surgeons, pathologists, and oncologists, due to their non-specific clinical presentation. Furthermore, radiologists face difficulties in interpreting computed tomography (CT) or magnetic resonance imaging (MRI) results, which often yield polymorphic and inconclusive findings. Ultimately, histopathologists play a crucial role in reaching a definitive diagnosis based on the tumor's histological characteristics. They are detected in every system of the human body, most commonly in the lungs. Here, we report an uncommon occurrence of IMT in the spleen of a patient with nonspecific abdominal pain. CASE REPORT: A 56-year-old Caucasian female presented to Konstantopouleio General Hospital of Nea Ionia, Athens, Greece, with abdominal pain and discomfort. The patient had no significant medical history and normal laboratory tests. An abdominal CT revealed a large mass in the spleen. A splenectomy was performed. Histopathological analysis of the tumor revealed IMTS. CONCLUSION: Splenic IMT is a rare benign tumor with moderate malignant potential. It lacks a distinct clinical presentation and is typically identified either incidentally or during the examination of abdominal pain.

Intraductal Papillary Mucinous Neoplasm of the Pancreas: A Challenging Diagnosis.

Intraductal papillary mucinous neoplasm of the pancreas (IPMN) was classified as a distinct entity from mucinous cystic neoplasm by the WHO in 1995. It represents a mucin-producing tumor that originates from the ductal epithelium and can evolve from slight dysplasia to invasive carcinoma. In addition, different aspects of tumor progression may be seen in the same lesion. Three types are recognized, the branch duct variant, the main duct variant, which shows a much higher prevalence for malignancy, and the mixed-type variant, which combines branch and main duct characteristics. Advances in cross-sectional imaging have led to an increased rate of IPMN detection. The main imaging characteristic of IPMN is the dilatation of the pancreatic duct without the presence of an obstructing lesion. The diagnosis of a branch duct IPMN is based on the proof of its communication with the main pancreatic duct on MRI-MRCP examination. Early identification by imaging of the so-called worrisome features or predictors for malignancy is an important and challenging task. In this review, we will present recent imaging advances in the diagnosis and characterization of different types of IPMNs, as well as imaging tools available for early recognition of worrisome features for malignancy. A critical appraisal of current IPMN management guidelines from both a radiologist's and surgeon's perspective will be made. Special mention is made of complications that might arise during the course of IPMNs as well as concomitant pancreatic neoplasms including pancreatic adenocarcinoma and pancreatic endocrine neoplasms. Finally, recent research on prognostic and predictive biomarkers including radiomics will be discussed.

Duplicated inferior vena cava-trifurcated portal vein: a rare anatomical variation encountered during Whipple procedure.

The inferior vena cava (IVC) is the largest single vein in humans. However, during embryogenesis, abnormalities can occur resulting in a duplicated IVC. The portal vein (PV) offers the main blood flood to the liver, forming by the left and right PV. A number of anatomical variations are noticed, underlying the great importance of the pre-operative imaging workup. This case report presents a duplicated IVC and a trifucated PV that were incidentally found in an 82 year-old Caucasian male with pancreatic ductal adenocarcinoma who underwent pancreatoduodenectomy (Whipple procedure). Although some anatomical variations, including the duplication of the IVC and the trifurcation of PV, may be rare to the general population, the suspicion of their existence should always be taken under consideration from surgeons during hepatobiliary or retroperitoneal operations.

Duplicated gallbladder: an incidental anatomical variation in a patient with symptomatic cholelithiasis.

Congenital malformations of the biliary tract represent a relatively rare entity with which surgeons, radiologists and clinicians are not adequately familiarized. We present a rare case of gallbladder duplication in a 40-year-old female, with the accessory cystic duct entering the left hepatic duct, which depicts the fifth reported case in the international bibliography. Our case illustrates the importance of detailed knowledge of anatomical malformations of the biliary tree, serving the purpose of a preoperative diagnosis of symptomatic cholelithiasis. It is also of paramount importance to take under consideration biliary tract malformations to avoid inadvertent complications such as biliary duct injuries in case of laparoscopic cholecystectomy.

Capecitabine, Oxaliplatin, Irinotecan, and Bevacizumab Combination Followed by Pazopanib Plus Capecitabine Maintenance for High-Grade Gastrointestinal Neuroendocrine Carcinomas.

OBJECTIVES: Gastrointestinal neuroendocrine carcinoma (NEC) is a lethal, uncommon, and understudied neoplasm. We present the efficacy and safety of first-line capecitabine (CP), oxaliplatin, irinotecan, and bevacizumab (CAPOXIRI-BEV) combination followed by pazopanib plus CP maintenance therapy in patients with advanced high-grade poorly differentiated gastrointestinal NEC. METHODS: This was a two-stage phase II study conducted at multiple institutions. Patients were consecutively enrolled and had advanced NEC of the colon or small bowel. Patients received irinotecan 125 mg/m, oxaliplatin 80 mg/m on day 1, CP 1000 mg/m twice daily on days 1 to 14, plus bevacizumab 8 mg/kg on day 1 for six 21-day cycles. Maintenance therapy was given to those who responded (complete response/partial response) or had stable disease after 6 cycles with CAPOXIRI-BEV with pazopanib 800 mg daily plus CP 1600 mg/m daily on days 1 to 14 every 3 weeks until disease progression or unacceptable toxicity. Patients who progressed on CAPOXIRI-BEV received standard etoposide-carboplatin. The primary endpoint was overall response rate. RESULTS: Twenty-two patients were enrolled of whom 19 were evaluable. The median age was 60 years. The overall response rate (3 complete response/6 partial response) was 47.4% (95% confidence interval: 29.5-76.1), the overall disease control rate was 78.9% (95% confidence interval: 62.6-99.6), and, at median 30 (11 to 41 mo) months' follow-up, 5 patients (26.3%) were still alive. Median progression-free survival was 13 months, and the 1-year progression-free survival rate was 52.6%. The median overall survival was 29 months. The median overall survival of the 9 patients who responded versus those with stable disease/progressive disease was 30.5 versus 14 months, respectively. The median duration of response was 16 months. Predictable toxicity was observed. CONCLUSIONS: First-line CAPOXIRI-BEV followed by pazopanib plus CP maintenance therapy for advanced NEC demonstrates promising efficacy and predictable toxicity. Further investigation is warranted.

Adult pancreatoblastoma: A case report and review of the literature.

The present study describes the case of a 24-year-old patient who presented with obstructive jaundice and weight loss, and was diagnosed with pancreatoblastoma (PB). Abdominal imaging studies revealed a heterogenous lesion of the pancreatic head with dilatation of the common bile duct. The patient underwent pancreaticoduodenectomy, however, three months after surgery multiple liver and bone metastases were identified on follow-up computed tomography scans. Despite treatment with four cycles of systemic chemotherapy and five courses of radiofrequency ablation, the patient succumbed due to tumour dissemination 13 months after initial diagnosis. PB is a malignant tumour of the pancreas that typically occurs in the pediatric population. The aim of the present study was to highlight the aggressive behavior of this rare clinical entity, focusing on the pitfalls of pre-operative diagnosis and the lack of management strategy guidelines in adults. Preoperative diagnosis of PB based on radiographic features may be difficult, as the imaging characteristics are non-specific. Furthermore, cytology may also be misleading, as the neoplasm consists of multiple cell lines (acinar, ductal and neuroendocrine cells) and diagnosis depends largely on the identification of the distinctive histological characteristic of squamoid corpuscles, which present as nests of flattened cells with a squamous appearance. Despite the use of surgical resection and adjuvant chemoradiotherapy for the treatment of this malignancy, its aggressive nature means that PB is associated with a poor prognosis in adult patients.

Rare cystic liver lesions: a diagnostic and managing challenge.

Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity's nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution.

Computed tomography-guided radiofrequency ablation for palliation of a painful supraclavicular soft-tissue metastasis invading the brachial plexus.

The present study describes a case of a painful supraclavicular soft-tissue metastasis of a skin melanoma invading the brachial plexus in a 38-year-old male. The patient was treated twice with radiofrequency ablation (RFA) under computed tomography (CT) guidance, which caused tumoral necrosis. The patient was originally referred with a 7-cm metastasis in the right supraclavicular fossa, which caused intractable pain and a degree of numbness. These symptoms were unresponsive to chemotherapy and radiotherapy and the pain was not controlled using narcotic analgesics. The lesion was treated with CT-guided RFA causing necrosis, relieving the pain and sparing the patient from using analgesics. The pain recurred 19 months thereafter and a CT scan revealed an 8-cm mass in the right supraclavicular space. The patient underwent repeat CT-guided RFA, which reduced the pain to a level that was controlled with minor oral analgesics. In conclusion, in this case of a painful supraclavicular soft-tissue metastasis invading the brachial plexus, which was intractable to chemotherapy and radiotherapy, RFA was feasible and offered substantial palliation of the symptoms, freedom from the use of narcotic analgesics and improvements to the quality of life.

Pathophysiology of acute and infected pancreatitis.

Two key pathologic acinar cell responses of acute pancreatitis are vacuole accumulation and trypsinogen activation. Degradation of long-lived proteins, a measure of autophagic efficiency, is markedly inhibited in pancreatitis. Further, processing of the lysosomal proteases cathepsin L (CatL) and CatB into their fully active, mature forms is reduced in pancreatitis, as are their activities in the lysosome-enriched subcellular fraction. These findings indicate that autophagy is retarded in pancreatitis due to deficient lysosomal degradation caused by impaired cathepsin processing. Trypsinogen activation occurred in pancreatitis and is prevented by inhibiting autophagy. A marker of trypsinogen activation partially localized to autophagic vacuoles, and pharmacologic inhibition of CatL increased the amount of active trypsin in acinar cells. The results suggest that retarded autophagy is associated with an imbalance between CatL, which degrades trypsinogen and trypsin, and CatB, which converts trypsinogen into trypsin, resulting in intra-acinar accumulation of active trypsin in pancreatitis. Thus, deficient lysosomal degradation may be a dominant mechanism for increased intra-acinar trypsin in pancreatitis. Proinflammatory cytokines and oxidative stress play a pivotal role in the early pathophysiological events of the disease. Cytokines such as interleukin 1beta and tumor necrosis factor alpha initiate and propagate almost all consequences of the systemic inflammatory response syndrome. On the other hand, depletion of pancreatic glutathione is an early hallmark of acute pancreatitis and reactive oxygen species are also associated with the inflammatory process. Changes in thiol homeostasis and redox signaling decisively contribute to amplification of the inflammatory cascade through mitogen activated protein kinase (MAP kinase) pathways.

The interventional radiological procedures of the infections of pancreas.

Infected necrotizing pancreatitis is the most severe form of acute pancreatitis and is related with high rates of morbidity and mortality. The close cooperation and communication, working as a team, among interventional radiologists surgeons and gastroenterologists improves the successful treatment considerably. Therapeutic modalities such as percutaneous CT-guided catheter drainage can be helpful to save lives, changing dramatically the clinical aspect of the patient. The objective of this paper is to review the indications and techniques of image-guided percutaneous treatment of pancreatic infected pseudocysts and to report our clinical experience and observations made during primary CT-guided percutaneous catheter drainage of infected abscesses.

Current trends in the management of infected necrotizing pancreatitis.

Severe acute pancreatitis is a potentially life-threatening disease. Pancreatic necrosis is associated with an aggravated prognosis, while superimposed infection is almost always lethal without surgery. Bacterial translocation mainly from the gut is the most widely accepted mechanism in the pathogenesis of infected pancreatic necrosis. Infected pancreatic necrosis should be suspected in the presence of the usual markers of systemic inflammation (i.e., fever and leukocytosis), organ failure, or a protracted severe clinical course. The diagnostic method of choice to confirm the diagnosis of pancreatic necrosis is contrast-enhanced computed tomography, where necrotic areas are evidenced as regions without enhancement. The presence of pancreatic necrotic infection should be based on a combination of clinical manifestations, results of laboratory investigation (mainly increased levels of CRP and / or procalcitonin), and can be confirmed by image-guided fine-needle aspiration and gram stain /culture of the aspirates. Surgery remains the treatment of choice for the management of infected pancreatic necrosis and involves open necrosectomy (debridement) and wide drainage of the peripancreatic areas, often in association with continuous irrigation. Planned reoperations may be required to achieve complete removal of the necrotic / infected material. The timing of surgery is of paramount importance; ideally, surgery should be performed after 2 or 3 weeks from the onset of pancreatitis. Recently, various minimally invasive approaches have been described, but they have not been compared in prospective trials with the classical open surgery. Antibiotic therapy is routinely used in patients with infected necrotizing pancreatitis, in conjunction with surgical debridement; its role, however, in the management of patients with sterile necrosis is recently questioned. Nutritional support should be taken into consideration in these patients; enteral nutrition should be preferred over total parenteral nutrition to improve the anatomical and functional integrity of the gut mucosa, thereby preventing bacterial translocation.

Imaging evaluation of post-pancreatitis infection.

Acute pancreatitis affects around 40 per 100.000 of the general population and 20-30% of attacks are severe. Mortality is usually associated to septic multiorgan dysfunction syndrome caused by secondary infection of pancreatic or peripancreatic necrosis. The diagnosis of acute pancreatitis is generally based on clinical and laboratory findings. However CT is the imaging technique of choice for detecting complications. Patients with complicated pancreatitis require multiple follow-up examinations. Substitution of US or MRI for CT in certain cases would reduce the radiation dose considerably. Complicated pseudocysts and other pancreatic collections may contain solid debris, which is best depicted by MRI. Abscesses are suggested when gas is present in a pancreatic or peripancreatic collection. MRI can reveal air-fluid levels or large pockets of gas, but CT is more sensitive for small gas collections. US or CT-guided percutaneous drainage of pancreatic abscesses or infected collections is a useful therapeutic approach in acute cases obviating the need for unnecessary surgery. On the other hand infected necrosis can not be successfully treated percutaneously due to its thicker consistency. In this review, the role of different imaging modalities in the evaluation of post-pancreatitis infection as well as in the treatment planning will be discussed.

Laparoscopic cholecystectomy in cirrhotic patients: the value of MELD score and Child-Pugh classification in predicting outcome.

BACKGROUND: Laparoscopic cholecystectomy is a challenging procedure in patients with cirrhosis. This study aims to evaluate the safety and outcome of laparoscopic cholecystectomy in patients with cirrhosis and examines the value of model for end-stage liver disease (MELD) score and Child-Pugh classification in predicting morbidity. MATERIALS AND METHODS: From January 1995 to July 2008, 220 laparoscopic cholecystectomies were performed in cirrhotic, Child-Pugh class A and B patients. Indications included symptomatic gallbladder disease and cholecystitis. MELD score ranged between 8 and 27. Child-Pugh class and MELD score were preoperatively calculated and associated with postoperative results. Data regarding patients and surgical outcome were retrospectively analyzed. RESULTS: No deaths occurred. Postoperative morbidity occurred in 19% of the patients and included hemorrhage, wound complications, and intra-abdominal collections controlled conservatively. Intraoperative difficulty due to liver bed bleeding was experienced in 19 patients. Conversion to open cholecystectomy was necessary in 12 cases. Median operative time was 95 min. Median hospital stay was 4 days. Patients with preoperative MELD score above 13 showed a tendency for higher complication rate postoperatively. Child-Pugh classification did not seem to predict morbidity effectively. CONCLUSION: Laparoscopic cholecystectomy can be performed safely in selected patients with cirrhosis Child-Pugh A and B and symptomatic cholelithiasis with acceptable morbidity. Some of its advantages are shorter operative time and reduced hospital stay. MELD score seems to predict morbidity more accurately than Child-Pugh classification system.

Hepatic resection for hepatocellular carcinoma exceeding Milan criteria.

BACKGROUND: Many hepatocellular carcinomas (HCCs) are discovered at an advanced stage. The efficacy of transplantation for such tumors is doubtable. The aim of this retrospective study was to determine liver resection efficacy in patients with large HCC regarding long term and disease- free survival. METHODS: Between 2002 and 2008, sixty six patients with large HCC (>5cm) underwent hepatectomy. Fifty nine patients had background cirrhosis due to hepatitis B, C or other reason and preserved liver function (Child A). Liver function was assessed by both Child's-Pugh grading and MELD score. Conventional approach of liver resection was performed in most cases. RESULTS: The 5-year overall survival was 32% with a median follow up of 33 months. The three year disease-free survival was 33% in our cohort. On multivariate analysis, only tumor size and grade remained independent predictors of adverse long term outcome. Multivariate analysis identified size of the primary tumour and degree of differentiation as risk factors for recurrence. Median blood loss was 540ml and median transfusion requirements were two units of pack red blood cells. Morbidity included pleural effusion (n=18), biliary fistula (n=4), peri-hepatic abscess (n=4), hyperbilirubinemia (n=3), pneumonia (n=5) and wound infection (n=6). No peri-operative mortality was reported in our study. CONCLUSION: Partial hepatectomy is safe in selective patients with large HCC. Surgical resection if feasible is suggested in patients with large HCC because it prolongs both overall and disease-free survival with low morbidity.

Pleurobiliary fistula, a rare complication of hepatocellular carcinoma after locoregional chemotherapy: a case report.

A rare complication of the compilation of high intrahepatic biliary pressure and the formation of a subdiaphragmatic abscess is that of pleurobiliary fistula. We present a case of 67-year-old male who presented with pleurobiliary fistula following transarterial chemoembolization in a patient with a large hepatocellular carcinoma, as well as the course of the diagnostic procedures and the therapeutics interventions which took place.

WITHDRAWN: Liver resection vs radio-frequency ablation in the treatment of small hepatocellular carcinoma.

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Clamp-crush technique vs. radiofrequency-assisted liver resection for primary and metastatic liver neoplasms.

BACKGROUND: Several techniques for liver resection have been developed. We compared radiofrequency-assisted (RF) and clamp-crush (CC) liver resection (LR) in terms of blood loss, operating time and short-term outcomes in primary and metastatic tumour resection. METHODS: From 2002 to 2007, 196 consecutive patients with primary or metastatic hepatic tumours underwent RF-LR (n= 109; group 1) or CC-LR (n= 87; group 2) in our unit. Primary endpoints were intraoperative blood loss (and blood transfusion requirements) and total operative time. Secondary endpoints included postoperative complications, mortality and intensive care unit (ICU) and hospital stay. Data were collected retrospectively on all patients with primary or secondary liver lesions. RESULTS: Blood loss was similar (P= 0.09) between the two groups of patients with the exception of high MELD score (>9) cirrhotic patients, in whom blood loss was lower when RF-LR was used (P < 0.001). Total operative time and transection time were shorter in the CC-LR group (P= 0.04 and P= 0.01, respectively), except for high MELD score (>9) cirrhotic patients, in whom total operation and transection times were shorter when RF-LR was used (P= 0.04). Rates of bile leak and abdominal abscess formation were higher after RF-LR (P= 0.04 for both). CONCLUSIONS: Clamp-crush LR is reliable and results in the same amount of blood loss and a shorter operating time compared with RF-LR. Radiofrequency-assisted LR is a unique, simple and safe method of resection, which may be indicated in cirrhotic patients with high MELD scores.

Model for end-stage liver disease (MELD) score, as a prognostic factor for post-operative morbidity and mortality in cirrhotic patients, undergoing hepatectomy for hepatocellular carcinoma.

BACKGROUND/AIMS: To evaluate the ability of the model for end-stage liver disease (MELD) in predicting the post-hepatectomy outcome for hepatocellular carcinoma (HCC). METHODS: Between 2001 and 2004, 69 cirrhotic patients with HCC underwent hepatectomy and the results were retrospectively analysed. MELD score was associated with post-operative mortality and morbidity, hospital stay and 3-year survival. RESULTS: Seventeen major and 52 minor resections were performed. Thirty-day mortality rate was 7.2%. MELD < or = 9 was associated with no peri-operative mortality vs. 19% when MELD > 9 (P < 0.02). Overall morbidity rate was 36.23%; 48% when MELD > 9 vs. 25% when MELD < or = 9 (P < 0.02). Median hospital stay was 12 days [8.8 days, when MELD < or = 9 and 15.6 days when MELD > 9 (P = 0.037)]. Three-year survival reached 49% (66% when MELD < or = 9; 32% when MELD > 9 (P < 0.01). In multivariate analysis, MELD > 9 (P < 0.01), clinical tumour symptoms (P < 0.05) and American Society of Anesthesiologists (ASA) score (P < 0.05) were independent predictors of peri-operative mortality; MELD > 9 (P < 0.01), tumour size >5 cm (P < 0.01), high tumour grade (P = 0.01) and absence of tumour capsule (P < 0.01) were independent predictors of decreased long-term survival. CONCLUSION: MELD score seems to predict outcome of cirrhotic patients with HCC, after hepatectomy.

Perioperative risk assessment for hepatocellular carcinoma by using the MELD score.

BACKGROUND/AIMS: The aim of this study was to evaluate the ability of Model for End-Stage Liver Disease (MELD) in predicting post hepatectomy outcome for hepatocellular carcinoma (HCC). METHODS: Between 2001 and 2005, 94 cirrhotic patients with HCC underwent hepatectomy and were analyzed retrospectively. MELD score associated with postoperative mortality and morbidity, hospital stay, and 3-year survival. RESULTS: Twenty-eight major and 66 minor resections were performed. Thirty-day mortality rate was 6.4%. MELD 9 (p = 0.01). Overall morbidity rate was 32%; 21% when MELD 9 (p = 0.01). Median hospital stay was 11 days (7 days, when MELD 9; p = 0.03). Three-year survival reached 48% (63% when MELD 9; p < 0.01). In multivariate analysis, MELD > 9 (p = 0.01), clinical tumor symptoms (p = 0.04), and American Society of Anesthesiologists score (p = 0.04) were independent predictors of perioperative mortality; MELD > 9 (p = 0.01), tumor size >5 cm (p = 0.01), presence of tumor symptoms (p = 0.02), high tumor grade (p = 0.01), and absence of tumor capsule (p = 0.01) were independent predictors of decreased long-term survival. CONCLUSION: MELD score seems to predict outcome of cirrhotic patients with HCC after hepatectomy.