Low-grade fibromyxoid sarcoma of the liver: A case report.

BACKGROUND: Low grade fibromyxoid sarcoma (LGFMS) is a rare and benign mesenchymal tumor with indolent course, most commonly found in young or middle-aged men. The majority of the LGFMSs are located in the trunk and deep soft tissue of the lower extremities. They appear as well circumscribed, although not encapsulated, which often leads to incomplete surgical resection. Despite their seemingly benign appearance, these tumors have aggressive behavior with high metastatic and recurrence rates. Accurate histopathologic examination of the specimen and its immunohistochemical analysis are mandatory for a precise diagnosis. CASE SUMMARY: We report a case of a 38 year-old-man who presented with jaundice and upper abdominal discomfort. Multi-detector computed tomography and magnetic resonance imaging showed a large left liver tumor mass, extending to the hepatoduodenal ligament. Left hepatectomy was performed with resection and reconstruction of hepatic artery and preservation of middle hepatic vein. Histopathologic examination confirmed the tumor being a low-grade fibromyxoid sarcoma. Three and a half years after surgery, the patient died after being diagnosed with spine metastasis. CONCLUSION: Due to poor response to all modalities of adjuvant treatment, we consider that the focus of treatment should be on surgery as the only option for curing the disease.

Standard versus extended lymphadenectomy in radical surgical treatment for pancreatic head carcinoma.

PURPOSE: The primary aim of this study was to evaluate the benefit of extended lymphadenectomy in pancreaticoduodenectomy (PD) and to estimate its impact on long-term survival in patients with pancreatic head carcinoma. Secondary endpoints included perioperative mortality, postoperative morbidity and predictors of survival in patients undergoing standard versus extended lymphadenectomy for pancreatic head carcinoma. METHODS: From January 2007 to December 2010, 60 patients with potentially resectable pancreatic head carcinoma were operated using pylorus-preserving pancreatoduodenectomy (PPPD) at the Clinic for Digestive Surgery, Clinical Center of Serbia, Belgrade. Intraoperatively patients were randomly stratified into two groups: the first group (N1=30) underwent PPPD with standard lymphadenectomy whilst the second group (N2=30) was operated with PPPD with extended lymphadenectomy. None of the patients received adjuvant treatments. RESULTS: The number of retrieved lymph nodes, mean operating time and postoperative hospital stay were greater in patients with extended lymphadenectomy . Cox regression analysis showed that stage and lymph node metastasis were independent prognostic factors for survival. CONCLUSION: Extended lymphadenectomy in PPPD did not improve long-term survival in patients with resectable pancreatic head carcinoma and led to comparable and similar morbidity and mortality rates to those after standard lymphadenectomy.

A Case of Autoimmune Cholangitis Misdiagnosed for Cholangiocarcinoma: How to Avoid Unnecessary Surgical Intervention?

INTRODUCTION: Autoimmune cholangitis or immunoglobulin G4-associated cholangitis (IAC) has been recently regarded as a new clinical and histopathological entity and is a part of a complex autoimmune disorder--IgG4-related systemic disease (ISD). ISD is an autoimmune disease with multi-organic involvement, characterized with IgG4-positive plasmocytic infiltration of various tissues and organs with a consequent sclerosis, which responds well to steroid therapy. Most commonly affected organs are the pancreas (autoimmune pancreatitis, [AIP]) and the common bile duct (IAC). IAC and cholangiocarcinoma (CCA) share many clinical, laboratory and imaging findings. CASE OUTLINE: We present a case of a 60-year-old male with a biliary stricture of a common bile duct, which was clinically considered as a bile duct carcinoma and treated surgically. Definite histopathological findings and immunohistochemistry revealed profound chronic inflammation, showing lymphoplasmacytic IgG-positive infiltration of a resected part of a common bile duct, highly suggestive for the diagnosis of IAC. In addition, postoperative IgG4 serum levels were also increased. CONCLUSION: It is of primary clinical importance to make a difference between IAC and CCA, in order to avoid unnecessary surgical intervention. Therefore, IAC should be considered in differential diagnosis in similar cases.

Primary retroperitoneal mucinous cystadenoma: A case report.

Primary retroperitoneal mucinous cystic tumors are extremely rare. These tumors can be classified as a primary retroperitoneal mucinous cystadenoma with or without borderline malignancy or primary retroperitoneal mucinous cystadenocarcinoma. The most common of these is primary retroperitoneal mucinous cystadenoma, which almost always occurs in female patients; only ten cases have been reported in males. The most common clinical findings for this tumor type include nonspecific abdominal pain and a palpable abdominal mass. A definitive diagnosis is usually obtained from histopathology after surgical excision. Here, we report the case of a 60-year-old female patient who complained of abdominal pain that had been present for 3 mo and presented with a palpable abdominal mass. Multidetector computed tomography scanning revealed a large, unilocular cystic mass in the left retroperitoneal space. Surgical intervention was performed and the tumor was completely removed. Histopathologic examination confirmed that the tumor was a primary retroperitoneal mucinous cystadenoma. Two years after surgery, the patient remains disease free.

Laparoscopic distal pancreatectomy for intrapancreatic accessory spleen: case report.

INTRODUCTION: Accessory spleens can be found in up to 10% of the population, and their intrapancreatic occurrence is considered uncommon. When present, the intrapancreatic accessory spleen is usually found in the tail of the pancreas in about 1.7% of adult individuals. The infrequent presence of the accessory spleen in the pancreatic tissue could lead to inappropriate diagnosis and hence therapeutic approach, as they are commonly presented as a hypervascular node in the tail of the pancreas on abdominal CT and MRI, mimicking a well differentiated tumor of the pancreas or non-functioning pancreatic neuroendocrine tumor. CASE OUTLINE: We present a 70-year-old female in whom a preoperative evaluation finding was highly suggestive of a non-functioning neuroendocrine tumor of the pancreatic tail. We performed spleen preserving laparoscopic distal pancreatectomy, and histopathological examination revealed the intrapancreatic accessory spleen. CONCLUSION: Although infrequent, the presence of the intrapancreatic accessory spleen must be considered in the differential diagnosis of pancreatic tail tumors.

Gastric adenomyoma.

BACKGROUND: Gastric adenomyoma is a rare, hamartomatous tumor localized most frequently in the gastric antrum. Review of the available literature shows only sporadic reports or smaller series. CASE REPORTS: We presented a 72-year-old woman admitted due to epigastric pain with dyspeptic difficulties. Biochemical parameters and tumor markers were within the referential limits. Diagnostic procedures (upper endoscopy, endoscopic ultrasonography and computerized tomography) revealed an intramural tumor prominence with intact mucosa on the posterior wall of gastric antrum, not accessible for biopsy. Surgical treatment was performed with total extirpation of the tumor. Histopathological examination verified adenomyoma with focal low grade epithelial dysplasia. Cytologic immunophenotype was consistent with smooth muscle stromal and epithelial tumor (CK7 and CK20 ++ immunophenotype). Stromal component revealed low proliferative index (Ki-67 protein immunoexpression level 3%), and p53 less than 0.1% in both epithelial and stromal components. Following the operation, the patient remained in good condition. CONCLUSION: Uncertain malignant potential of the gastric adenomyoma in the presented case indicates that timely diagnostics with adequate surgical treatment is crucial for an adequate treatment.