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.

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.

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.