Gastrointestinal stromal tumors (GIST) constitue the largest category of primary non-epithelial neoplasms of the stomach and small bowel. They are characterized by a remarkable cellular variability and their malignant potential is sometimes difficult to predict. Very recent studies, using mitotic count and tumor size as the best determinants of biological behavior, divide GISTs into three groups: benign, borderline and malignant tumors. We report on a male patient who underwent a right hepatectomy for a large metastasis 11 years after the surgical treatment of an antral-pyloric gastric neoplasm, histologically defined as leiomyoblastoma and with clinical, morphological and immunohistochemical features of benignity (low mitotic count, tumor size < 5 cm, low cellular proliferation index). Histological and immunohistochemical analysis of the hepatic metastasis showed the cellular proliferation index (Ki-67) to be positive in 25% of neoplastic cells, as opposed to the primary gastric tumor in which Ki-67 was positive in only 5% of neoplastic cells. In conclusion, although modern immunohistochemical techniques are now available to obtain useful prognostic information, the malignant potential of GISTs is sometimes difficult to predict: neoplasms clinically and histologically defined as benign could metastasize a long time after oncologically correct surgical treatment. Therefore, benign GISTs also require consistent, long-term follow-up.
Budd-Chiari syndrome (BCS) is an uncommon form of portal hypertension caused by obstruction of the hepatic venous outflow. From 1969 to 1997 we treated 19 patients (7 men, 12 women; mean age 37.6 years) affected by primary BCS. In most of the cases no etiologic factors were identified; in the remaining cases the etiology was associated with polycythemia vera, use of oral contraceptives, presence of endoluminal membranes, and repeated episodes of sepsis. Three patients with membranous occlusion of the major hepatic veins were treated by percutaneous placement of a self-expanding metallic stent inserted via a transjugular or transhepatic approach. The remaining 16 patients underwent a side-to-side portacaval shunt, which required interposition of a graft in five cases. In two patients with a significant caval obstruction, a metallic vascular stent was placed in the narrowed tract of the inferior vena cava, before shunting, by means of a transfemoral venous approach. One patient died within the first 30 postoperative days. The 18 survivors were followed for a mean of 66.7 months. The 5-year survival was 83%. Primary BCS requires different therapies depending on the stage of the disease. The fulminant or chronic forms with irreversible hepatic damage require definitive treatment, such as orthotopic liver transplantation. For the acute or subacute forms, characterized by reversible hepatic injury, a portasystemic shunt represents the most effective treatment. The patients at poor hepatic risk can be treated by interventional radiology. In both cases preliminary caval stenting is necessary if the syndrome is complicated by significant obstruction of the inferior vena cava.
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