[Extraintestinal GIST--case report]. / Extraintestinální GIST--kazuistika.

Gastrointestinal stromal tumors (GISTs) represent the majority of mesenchymal neoplasms of the gastrointestinal tract. Extragastrointestinal stromal tumors (EGISTs), which are neoplasms with histological and immunohistological features overlapping with those of GISTs, are found in the abdomen outside of the gastrointestinal tract with no connection to the gastric or intestinal wall. We present a case of EGIST arising in the mesentery of a 68 years old patient.

[GIST of the small bowel in neurofibromatosis terrain as a source of massive bleeding]. / GIST tenkého streva v terénu neurofibromatózy jako zdroj masivního krvácení

Neurofibromatosis type I (Morbus Von Recklinghausen) is an autosomal dominant disorder. The major diagnostic criteria include multiple cutaneous neurofibromas, café au lait spots, that are rarely found in the gastrointestinal tract. 5-25% of these lesions, however, may develop into gastrointestinal stromal tumours. We report the case of a 69-year-old woman with Von Recklinghausen disease. She was admitted due to gastrointestinal bleeding. During surgery we found, among multiple neurofibromatic intestinal lesions, macroscopically different bleeding tumours. They were completely removed. Histological examination revealed gastrointestinal stromal tumour. Using an immunohistological assay, we examined stored specimens from previous operations on the same patient: one anal polyp removed a year ago and tumours removed 32 years ago and regarded as polyps then were re-classified as gastrointestinal stromal tumours. In the discussion, the authors address the issue of which examination of the intestine would be appropriate to find out the source of bleeding in the small intestine and how to distinguish intestinal stromal tumours in the terrain of intestinal neurofibromatosis. Another issue addressed is a screening examination in patients with skin forms of Recklinghausens disease. To successfully manage intestinal bleeding, close cooperation among the surgeon, the endoscopist and the radiologist is indispensable. In order to quickly establish the right diagnosis and subsequently target treatment promptly, it is very helpful to know the patients exact personal medical history and also the possible complications of chronic diseases.

Small cell variant of renal oncocytoma--a rare and misleading type of benign renal tumor.

Here we report 3 cases of renal oncocytoma with a dominating small cell component arising in adult women. These 3 cases of small cell variant of renal oncocytoma were found among 134 cases of oncocytomas of the kidney in the routine and consultation files of the authors. Two of 3 cases were initially thought to be a malignant tumor by the referring pathologists. In all 3 cases minor areas of the tumors, which were identical to the classical renal oncocytomas were found. The small cell areas were arranged in a characteristic organoid pattern different from all the known types of the renal benign and malignant tumors. We summarize all morphologic, immunohistochemical, and ultrastructural features of this unusual oncocytoma variant with regard to its differential diagnosis. Int J Surg Pathol 9(3):215-222, 2001

[Oncocytoma of the kidney--morphologic variation in 102 cases]. / Onkocytom ledviny--morfologická variabilita 102 prípadu.

From the collection of 2500 cases of renal epithelial tumors in our files, 102 renal oncocytomas were analyzed for size, multifocality and a morphologic spectrum of the growth pattern. The size of the tumors ranged from 1.5 to 13 cm in diameter, with a mean of 6.3 cm. Three cases were multifocal, four cases were combined with another primary renal tumor (1x angiomyolipoma, 1x conventional renal carcinoma, 2x papillary renal cell carcinoma). A central fibrosis or a scar was noted in 13 cases, and there was a gross area of hemorrhage in 11 cases. In 4 cases extensive necroses were recognized. Histologically, an alveolar pattern was noted in 70 cases. A tubular pattern was revealed in 31 cases and an unusual tubopapillar ("glomeruloid") pattern was noted in one case. Foci of atypical nuclei were identified in 58 cases. In 4 oncocytomas broad areas of clearance of the oncocytes were found. Psammoma bodies were recognized in 9 tumors and foci of ossification were present in 4 cases. Intracellular and extracellular hyaline globules were noted in two cases. Renal oncocytoma has a variable morphologic spectrum, and its diagnosis should be based on an analysis of structural and cytologic features. Differential diagnosis of renal oncocytomas with various tumors of the kidney which contain granular cytoplasm is discussed. These tumors with granular cytoplasm include conventional renal cell carcinomas, chromophobe cell carcinomas, and rare examples of papillary renal carcinomas.

[Thrombosis in the area of the superior mesenteric vein in a female patient positive for APC resistance]. / Trombóza v povodí vena mesenterica superior u pacientky s pozitivní APC rezistencí.

Thrombosis of the superior mesenteric vein (VMS) is a frequent cause of ischaemia of the gut (5% to 15% acute intestinal ischaemias). The authors summarize the causes of venous thrombosis, the clinical picture, diagnostic procedure and treatment. They describe the case-history of a 79-year-old patient with thrombosis of the VMS. They revised the abdominal cavity and assessed the correct diagnosis on operation. On account of gangrene of the small intestine resection was performed. For a brief period anticoagulant treatment was administered, food was administered soon. The patient does not require any further revisions of the abdominal cavity. The authors examined thrombophil markers and assessed APV resistance. Relapse of TEN in the form of bilateral massive pulmonary embolization with subsequent anticoagulation.

[Angiosarcoma of the liver as a cause of massive hemoperitoneum]. / Angiosarkom jater jako prícina masivního hemoperitonea.

The authors give an account of an uncommon cause of haemoperitoneum--spontaneous rupture of an angiosarcoma-afflicted liver. During the urgent operation haemostatic stitches, partial devascularisation of the liver and tamponade were used. The patient died during the postoperative period due to prolonged haemorrhagic shock and developing multiorgan failure. The authors discuss possible methods of haemostasis of hepatic origin, and emphasize perihepatic tamponade (packing). Failure of the surgery of this case-history is discussed.