Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocythemia: Case Report. [Corrected].

Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)-like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, and abnormal liver function tests and coagulation profile. Imaging revealed hepatic vein thrombosis, confirming Budd-Chiari syndrome, and a 6.2 × 6.1 × 6.8 cm lesion in segment 8 of the liver. Primary cause of Budd-Chiari syndrome was essential thrombocythemia according to bone marrow biopsy and molecular testing results. The patient was treated medically and underwent transjugular intrahepatic portosystemic shunt insertion. The lesion in segment 8 continued to enlarge. Cadaveric liver transplantation was carried out. On gross and histologic examination of the explanted liver, the lesion was found to be a true FNH.

GIST in Saudi Arabia: multicentric histopathological genetic study of 75 surgically excised cases.

The availability of CD117 (Ckit) immunohistochemical testing in most hospital laboratories has facilitated the diagnosis of GIST in cKit positive cases. The aim of this study was to describe the histopathological variations of GIST in Saudi Arabia. The study involved analysis of paraffin blocks of 75 surgically excised GIST and GIST-like stromal tumors obtained from four major referral hospitals in Saudi Arabia (Riyadh Military Hospital, King Abdulaziz Medical City, King Faisal Specialist Hospital & Research Center and Aramco's hospital) between 1998 and 2009. Patient sex and age were noted in addition to the following tumor variables: size, site, histological grade and type, immunohistochemical profile and genetic analysis of the cKit mutation. The stomach was the commonest site for tumors and spindle cell type was the most frequently seen variant. Further sub classification of the histopathological type was made in order to recognize the morphological pattern of the tumor. The most common tumor grades seen were of low-risk and high-risk groups. There was one case of familial GISTparaganglioma syndrome and the youngest subject was an 8-year-old girl with high-risk spindle cell gastric GIST. Genetic study in 34 cases revealed exon 11 mutations in all of the cKit genes.

Clinical features, treatment, and outcome in 102 adult and pediatric patients with localized high-grade synovial sarcoma.

Background. There remains controversy on the routine use of chemotherapy in localized SS. Methods. The records of 87 adult (AP) and 15 pediatric (PP) patients with localized SS diagnosed between 1986 and 2007 at 2 centres in Toronto were reviewed. Results. Median age for AP and PP was 37.6 (range 15-76) and 14 (range 0.4-18) years, respectively. 65 (64%) patients had large tumours (>5 cm). All patients underwent en bloc surgical resection resulting in 94 (92.2%) negative and 8 (7.8%) microscopically positive surgical margins. 72 (82.8%) AP and 8 (53%) PP received radiotherapy. Chemotherapy was administered to 12 (13.8%) AP and 13 (87%) PP. 10 AP and 5 PP were evaluable for response to neoadjuvant chemotherapy, with response rate of 10% and 40%, respectively. 5-year EFS and OS was 69.3 ± 4.8% and 80.3 ± 4.3%, respectively, and was similar for AP and PP, In patients with tumors >5 cm, in whom chemotherapy might be considered most appropriate, relapse occurred in 9/19 (47%) with chemotherapy, compared to 17/46 (37%) In those without. Conclusions. Patients with localized SS have a good chance of cure with surgery and RT. Evidence for a well-defined role of chemotherapy to improve survival In localized SS remains elusive.

Cytological clues in the distinction between phyllodes tumor and fibroadenoma.

BACKGROUND: Fine-needle aspiration (FNA) sensitivity in discriminating between phyllodes tumor and its benign mimicker fibroadenoma (FA) remains low. Because a preoperative categorization of phyllodes tumor is crucial for their appropriate management, the authors undertook this study in an effort to improve the outcome of FNA by identifying reliable distinguishing features. METHODS: FNA smears from 15 and 12 histologically proven cases of phyllodes tumor and FA, respectively, were reviewed. The stromal and epithelial components were qualitatively and quantitatively analyzed. A group of cytological features that may distinguish between phyllodes tumor and FA were identified. Their value and reproducibility in categorizing phyllodes tumor cases were tested. RESULTS: Three major cytological features were exclusively seen in all of, or the vast majority of, the phyllodes tumor cases; fibromyxoid stromal fragments with spindle nuclei (90%), fibroblastic pavements (93%), and appreciable number of spindles cells of fibroblastic nature among dispersed cell population (100%). The identification of these features improved the pick-up rate of phyllodes tumor from 40% to 100% and had substantial (kappa = 0.56-0.73) interobserver and almost perfect (kappa = 0.83) intraobserver reproducibility. There was no significant difference in the epithelial component characteristics between phyllodes tumor and FA. The frequency and degree of atypia in the dispersed cell population correlated well to the histological grade of phyllodes tumor. CONCLUSIONS: FNA has proven to be a reliable test in differentiating between phyllodes tumor and FA with high sensitivity and good reproducibility. The importance of training and continuing education is emphasized.

Mesenteric panniculitis of the sigmoid colon.

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