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.

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.

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 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.

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.

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.

Hepatic resection for large hepatocellular carcinoma in the era of UCSF criteria.

BACKGROUND: Treating patients with hepatocellular carcinoma (HCC) remains a challenge, especially when the disease presents at an advanced stage. The aim of this retrospective study was to determine the efficacy of liver resection in patients who fulfil or exceed University of California San Francisco (UCSF) criteria by assessing longterm outcome. METHODS: Between 2002 and 2008, 59 patients with large HCC (>5 cm) underwent hepatectomy. Thirty-two of these patients fulfilled UCSF criteria for transplantation (group A) and 27 did not (group B). Disease-free survival and overall survival rates were compared between the two groups after resection and were critically evaluated with regard to patient eligibility for transplant. RESULTS: In all patients major or extended hepatectomies were performed. There was no perioperative mortality. Morbidity consisted of biliary fistula, abscess, pleural effusion and pneumonia and was significantly higher in patient group B. Disease-free survival rates at 1, 3 and 5 years were 66%, 37% and 34% in group A and 56%, 29% and 26% in group B, respectively (P < 0.01). Survival rates at 1, 3 and 5 years were 73%, 39% and 35% in group A and 64%, 35% and 29% in group B, respectively (P= 0.04). The recurrence rate was higher in group B (P= 0.002). CONCLUSIONS: Surgical resection, if feasible, is suggested in patients with large HCC and can be performed with acceptable overall and disease-free survival and morbidity rates. In patients eligible for transplantation, resection may also have a place in the management strategy when waiting list time is prolonged for reasons of organ shortage or when the candidate has low priority as a result of a low MELD (model for end-stage liver disease) score.

Surgical management of large adrenal tumours: the University of Miami experience using liver transplantation techniques.

OBJECTIVE: To report our experience in the surgical management of patients with large adrenal masses and describe the key steps in performing radical resections, which are especially demanding where thrombi extend into the inferior vena cava (IVC). PATIENTS AND METHODS: From 2003 to 2007, 14 patients presented with large adrenal mass, and underwent surgical extirpation. In five patients thrombi extended into the IVC, causing Budd-Chiari syndrome in one. RESULTS: The median (range) patient age was 48 (40-58) years. The excision was radical with negative tumour margins in all cases. Cardiopulmonary bypass (CPB) was required in one case with adherent intra-atrial thrombus. The mean blood loss was 500 (250-1500) mL except in the patient who required CPB. Morbidity included pleural effusion (three patients) and postoperative pneumonia (two), which responded to conservative management. All patients were alive and free of disease at a median follow-up of 12 (5-42) months. CONCLUSIONS: Surgical extirpation of large adrenal masses requires technical experience to optimize outcome. Total tumour excision is the only therapeutic option in such cases and provides acceptable results in survival and quality of life.

Radiofrequency-assisted liver resection.

BACKGROUND: Surgical resection remains the treatment of choice for primary, secondary liver cancer and a number of benign liver lesions. Complications are mainly related to blood loss. Radiofrequency-assisted liver resection (RF-LR) has been proposed in order to achieve minimal blood loss during parenchymal transection. PATIENTS AND METHODS: Between May 2005 and April 2007, 46 consecutive patients with various hepatic lesions underwent RF-LR using Radionics, Cool-Tip System. There were 28 men and 18 women with median age 65 years (range 54-76 years). Twelve major and 34 minor hepatectomies were performed for various diseases: hepatocellular carcinoma (n=19), metastatic carcinoma (n=23), focal nodal hyperplasia (n=2) and intrahepatic cholangiocarcinoma (ICC) (n=2). Hepatic inflow occlusion was not used. RESULTS: No perioperative death was documented. Median blood loss was 100ml (range 30-300cm(3)). Blood transfusion was required postoperatively in one patient. Median transection time was 35min (15-60min). Three patients developed biliary fistulas, four patients pleural effusions, one patient hyperbilirubinemia, two pneumonia and four wound infection. The median postoperative hospital stay was 6 days (range 4-10 days). In a median 12 month follow-up (range 3-24 months), four patients with colorectal metastases (CRM) and one patient with ICC developed recurrence. CONCLUSIONS: Cool-Tip RF device provides a unique, simple and safe method of bloodless liver resections and is indicated in cirrhotic patients with challenging hepatectomies (segment VIII, central resections).

Intrahepatic biliary cystadenoma: a need for radical resection.

BACKGROUND: Intrahepatic biliary cystadenoma (IBC) is a rare liver tumour, which has strong tendency to recur and malignant potential as it can progress to cystadenocarcinoma (IBCa). METHODS: From June 2003 to December 2006, four patients diagnosed with hepatic cystadenoma were operated on our Liver Surgical Unit. All patients were females with median age of 51 years (range 45-63 years). Liver resections included three left and one right hepatectomies. In two patients, IBC was diagnosed by abdominal imaging and serum tumour markers but the rest of the patients were initially misdiagnosed as simple cysts, treated by laparoscopic fenestration and referred to our unit after cyst recurrence. RESULTS: In all cases, the pathology report was consistent with liver cystadenomas. The postoperative course was uneventful and the median hospital stay was 8 days (range 5-12 days). In a median 18-month follow-up (range 2-40 months), all patients are alive and free of recurrence. CONCLUSION: Liver cystadenomas can be easily misdiagnosed with other hepatic cystic lesions. An aggressive surgical approach is recommended, due to their malignant potential and high recurrence rate after fenestration.

Selection criteria for liver resection in patients with hepatocellular carcinoma and chronic liver disease.

Hepatocellular carcinoma (HCC) is one of the most common malignancies worldwide with an annual occurrence of one million new cases. An etiologic association between HBV infection and the development of HCC has been established with a relative risk 200-fold greater than in non-infected individuals. Hepatitis C virus is also proving an important predisposing factor for this malignancy with an incidence rate of 7% at 5 years and 14% at 10 years. The prognosis depends on tumor stage and degree of liver function, which affect the tolerance to invasive treatments. Although surgical resection is generally accepted as the treatment of choice for HCC, new treatment strategies, such as local ablative therapies, transarterial embolization and liver transplantation, have been developed nowadays. With increasing detection of small HCCs from screening programs for cirrhotic patients, it is foreseen that locoregional therapy will play an important role in the near future.

Leiomyosarcoma of the infrarenal portion of the inferior vena cava in a cirrhotic patient with hepatitis C.

Leiomyosarcoma of the inferior vena cava is a rare primary tumor. We present a case of a 65-year-old man with the history of cirrhosis and non-specific symptoms. The patient underwent computed tomography and magnetic resonance imaging that revealed a leiomyosarcoma of the infrarenal portion of inferior vena cava. Clinical and imaging features, as well as the surgical technique of resection are discussed.

Rare localizations of the hydatid disease. Experience from a single center.

Hydatid disease is a rare entity that mostly affects the liver and lung, but almost any organ, forming cysts. Although diagnosis is confirmed by serology and imaging studies, a high index of suspicion is required based on the epidemiological background of the patient. However, rare locations of the cysts remain a diagnostic and therapeutic dilemma. Surgical exploration with special attention to avoid parasite spillage is justified in these situations because diagnostic puncture is usually contraindicated. Pericystectomy or fenestration and omentoplasty is strongly recommended, along with the excision of involved organs when feasible.

Multiple primary liver lipomas in a patient with chronic hepatitis B: a case report.

True liver lipomas are extremely rare. We present a case of a female patient with a history of chronic hepatitis B and multiple liver lipomas. The patient was completely asymptomatic. Liver ultrasound was suggestive of lipomas, whereas the diagnosis was confirmed by computed tomography and magnetic resonance imaging. To our knowledge, this is the first report of multiple liver lipomas in the literature.

Current role of bloodless liver resection.

Liver resections are demanding operations which can have life threatening complications although they are performed by experienced liver surgeons. Recently new technologies are applied in the field of liver surgery, having one goal: safer and easier liver operations. The aim of this article is to address the issue of bloodless liver resection using radiofrequency energy. Radionics, Cool-tip System and Tissue Link are some of the devices which are using radiofrequency energy. All information included in this article, refers to these devices in which we have personal experience in our unit of liver surgery. These devices take advantage of its unique combination of radiofrequency current and internal electrode cooling to perform sealing of the small vessels and biliary radicals. Dissection is also feasible with the cool-tip probe. For the purposes of this study patient sex, age, type of disease and type of surgical procedure in association with the duration of parenchymal transection, blood loss, length of hospital stay, morbidity and mortality were analyzed. Cool-tip RF device may provide a unique, simple and rather safe method of bloodless liver resections if used properly. It is indicated mostly in cirrhotic patients with challenging hepatectomies (segment VIII, central resections). The total operative time is eliminated and the average blood loss is significantly decreased. It is important to note that this technique should not be applied near the hilum or the vena cava to avoid damage of these structures.

Splenic abscess in a patient with fecal peritonitis.

Splenic abscess is a rare entity normally associated with underlying diseases. We report a case of splenic abscess with large gas formation in a non-diabetic and non-immunosuppressed patient after surgery for colon perforation. The most frequent cause of splenic abscess is septic embolism arising from bacterial endocarditis. Splenic abscess has a high rate of mortality when it is diagnosed late. Computed tomography resolved any diagnostic doubt, and subsequent surgery confirmed the diagnosis.

Asymptomatic calcitonin-secreting tumor of the pancreas. A case report.

CONTEXT: Pancreatic endocrine tumors are unusual tumors arising from cells belonging generically to the amine precursor uptake and decarboxylation system. CASE REPORT: We present a case of a calcitonin-secreting pancreatic endocrine tumor in a 59-year-old male who presented at our Center with elevated calcitonin values. The patient was asymptomatic. Further investigation revealed a tumor, 80 mm in diameter, in the pancreatic body and tail along with three metastatic lesions in segments III, V, and VIII of the liver. Following a distal pancreatectomy, splenectomy and wedge resection of segments III and V along with radiofrequency ablation of the segment VIII lesion, his serum calcitonin reached normal values. CONCLUSIONS: Calcitonin-secreting pancreatic endocrine tumors are often malignant and have a poor prognosis. We believe that an aggressive surgical approach may improve survival.

Surgical resection of a solitary liver metastasis from nasopharyngeal carcinoma: a case report.

BACKGROUND: Nasopharyngeal carcinoma (NPC) has a propensity to develop distant metastases at a high rate and with poor prognosis. Metastatic sites are usually multifocal and involve bones, lungs, liver and distant lymph nodes. Management of metastatic disease is essentially palliative and is based on chemotherapy. METHODS: A 50-year-old man with a solitary liver metastasis from a newly diagnosed NPC was treated by segmentectomy. Prior to surgery, neoadjuvant chemotherapy followed by concurrent chemoradiotherapy was administered. RESULTS: Complete remission of the primary disease was achieved, although the size of the hepatic lesion was increased. After resection of the liver metastasis, no signs of local or distant recurrence was noted during the 6-month follow up. CONCLUSION: Although surgical treatment has a limited role in metastatic NPC, there are rare cases of localized disease with a reasonable outcome after resection.

Changing concepts in the management of liver hydatid disease.

Hydatid disease is a rare entity primarily affecting the population of developing countries. The parasite shuttles between the liver and lungs. but almost any organ can be invaded, forming cysts. Septation and calcification of the cysts with a high antibody titre in the patient's serum confirm the diagnosis, although more sophisticated tests have been applied recently. Surgery constitutes the primary treatment, with a variety of techniques based on the principles of eradication and elimination of recurrence by means of spillage avoidance. Minimally invasive techniques and percutaneous drainage of the cysts are now feasible because of progress in the field. The aim of this review is to collect the experience from three different institutions and to provide practical guidelines for diagnostic and therapeutic strategies.

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.