1/14. Right atrial extension of Wilms' tumor.BACKGROUND: Right atrial involvement in children with Wilms' tumor (nephroblastoma) is a very rare phenomenon. CASE REPORT: The authors present four children with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. In two children the intracardiac tumor extension was asymptomatic, while the other two had budd-chiari syndrome. Therapeutic management included initial three-drug chemotherapy in three patients, and primary tumor excision in one child, since the venous involvement had been not diagnosed. In each child, the therapeutic strategy was individualized and tailored to the course of the disease. Two patients were cured. The child with bilateral Wilms' tumor died due to disease progression. Another child died suddenly at home in the course of an intermission between consecutive cycles of successful chemotherapy. CONCLUSIONS: The extension of Wilms' tumor to the great vessels and the right atrium indirectly affects the final outcome. Preoperative chemotherapy in children with Wilms' tumor invading the inferior vena cava and the right atrium is the method of choice. The extent of surgery depends on the preliminary chemotherapy results.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/14. Superior mesenteric artery injury during nephrectomy for Wilms' tumor.Iatrogenic injury to the aorta or its major branches during nephrectomy for Wilms' tumor in children is rarely reported but may be more common than is currently acknowledged. We identified four patients with ligation of the superior mesenteric artery (SMA) that occurred during nephrectomy for nephroblastoma and another child in whom SMA thrombosis developed postoperatively. All of the tumors were on the left side. Interruption of the SMA was recognized intraoperatively in all four children, and primary repair was done. In three patients, appearance of the bowel remained normal before repair of the injury. Three of the arteries were repaired by primary reanastomosis, and one was joined with an interpositioned hypogastric artery graft. None of these patients had gastrointestinal complications postoperatively. In the fifth patient, SMA thrombosis developed after repair of an aortic tear during nephrectomy. This patient required subsequent small bowel resection for bowel infarction and died in the perioperative period. Every surgeon treating children with Wilms' tumor should be aware of the possibly distorted vascular anatomy and take precautions to avoid such a significant injury. Attempts at early ligation of the vessels may not be justified until the renal vasculature is clearly identified.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/14. Fine-needle aspiration cytology of clear-cell sarcoma of the kidney: study of eight cases.The largest series, to date, of fine-needle aspiration cytology (FNAC) findings in clear-cell sarcoma of the kidney (CCSK) is presented. All fine-needle aspirates of pediatric renal masses over a 17-yr period were reviewed. Eight out of 119 aspirates from late-stage childhood renal tumors (6.72%) were found to be CCSK. Ten aspirates from these eight patients and histopathological confirmation in six patients were available. Aspirates were cellular with three cell types: cord cells, septal cells, and small pyknotic cells. Cord cells, seen in all aspirates, were large polygonal cells with abundant eccentrically placed wispy cytoplasm, round to oval nuclei, and fine dusty chromatin. Occasional bare nuclei and frequent nuclear grooves were also seen. Small pyknotic cells were a degenerative change identified in 9 out of 10 aspirates. Stromal fragments with branching vascular cores were seen in 8 out of 10 aspirates, 6 of which had myxoid substance surrounding the vessel. Septal cells were spindle shaped and usually embedded in the stromal fragments. On the basis of cytology and histology, cases were classified into classical CCSK (5 cases), spindle-cell CCSK (1 case), and anaplastic CCSK (2 cases). Classical CCSK showed mostly cord cells with few stromal fragments. Spindle-cell CCSK showed preponderance of myxoid stromal fragments and septal cells. Anaplastic CCSK showed bizarre pleomorphic nuclei, coarse chromatin, and atypical mitosis. Cytology of CCSK is a spectrum with varying proportions of cord cells, septal cells, and mucopolysaccharide substance. Anaplastic CCSK is liable to misdiagnosis as wilms tumor (WT) with unfavourable histology. Presence of eccentric cytoplasm in cord cells and nuclear grooves are the key to differentiation from wilms tumor, including anaplastic variants.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/14. Cavectomy for the treatment of wilms tumor with vascular extension.PURPOSE: Vascular extension to the vena cava occurs in 4% of wilms tumor cases and can reach the right atrium in up to 1%. When this happens the thrombus is usually not adherent to the vessel wall, and there is blood flow around it. Preoperative chemotherapy can cause thrombus regression and even resolution. If the thrombus persists after chemotherapy, surgery will be a challenge. On the other hand, if the thrombus invades the vessel wall, its removal may not be feasible. In this situation cavectomy is a good surgical strategy because it provides complete resection. The prerequisite for cavectomy is the absence of blood flow in the vena cava on preoperative Doppler ultrasonography. We report 3 cases of wilms tumor with vena caval invasion in which cavectomy was performed, and discuss the principles, indications and operative technique. MATERIALS AND methods: A total of 171 patients with wilms tumor were treated at our institution between 1984 and 2004. Of these patients 6 with intravascular extension of thrombus within the right atrium were treated with extracorporeal circulation, cardiac arrest and profound hypothermia, and 3 were treated with cavectomy. RESULTS: There were no instances of surgical complications or postoperative renal failure in our patients who underwent cavectomy. All remain well and free of disease. CONCLUSIONS: Cavectomy is a safe procedure for treating pediatric patients with wilms tumor when there is extension and invasion of the vena cava wall without blood flow.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/14. Teratoid Wilms' tumor: a case report with literature review.Teratoid Wilms' tumor is an unusual histologic variant of nephroblastoma in which the heterologous tissue predominates. The treatment for this tumor has not been established because of its rarity and varying tumor components. The authors report a case of localized teratoid Wilms' tumor in a 4-month-old male infant successfully treated with nephrectomy only. The tumor originated from the right kidney with multiple cysts and calcifications; renal teratoma was suspected preoperatively, and the operation was performed. The tumor was excised completely, and the specimen weighed 340 g. Histopathologic examination showed that various teratoid elements occupied most of the tumor with sparse islands of triphasic nephroblastoma, which was consistent with teratoid Wilms' tumor. Anaplastic elements, tumor capsule invasion, renal sinus soft tissue invasion, or tumors in intrarenal vessels were not observed. The patient was disease free for 3 years after surgery without receiving chemotherapy. We selected this therapeutic approach because of the chemoresistance of this tumor and the excellent prognosis in small (<550 g) stage I/favorable-histology classical Wilms' tumor diagnosed at less than 24 months of age. Therefore, nephrectomy alone may be an acceptable treatment of this stage of tumor in patients with teratoid Wilms' tumor.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/14. Postradiation renovascular hypertension.radiation injury to arteries can represent a significant complication of therapeutic irradiation, even when the dosage used has not been excessive as judged by approved protocols. Children in whom therapeutic abdominal irradiation has been used should be monitored indefinitely for the development of hypertension. The presence of hypertension in such children with normal blood urea nitrogen (BUN) and creatinine, and without proteinuria, should prompt investigation for a renovascular lesion. Standard bypass procedures are usually effective, although the long-term success may be compromised by continuing changes in affected vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/14. adult nephroblastoma.Clinical, pathologic, and ultrastructural features of two cases of adult nephroblastoma (Wilms' tumor) are presented, one in a 77-year-old man who had a large tumor with extracapsular extension and blood vessel invasion. Histologically, it consisted mainly of blastema. The patient died during the first course of postoperative chemotherapy with dactinomycin. The second patient was a 47-year-old woman with a tumor limited to the renal parenchyma. Histologically, the tumor was mainly epithelial. This tumor was discovered incidentally and remained unchanged for 5 years before surgery. The patient underwent nephrectomy and refused further treatment. She was free of disease 2 years after surgery.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/14. renin in mesoblastic nephroma: an immunohistochemical study.Congenital mesoblastic nephroma (CMN) is the most common renal tumor of early infancy. It is usually evident at birth as an abdominal mass and is composed of spindle-shaped cells resembling smooth muscle cells and fibroblasts. There is macroscopic and microscopic infiltration of the surrounding kidney and entrapped tubules and glomeruli are common at the edge of the tumor. In this report, we describe a case of CMN associated with hyperreninaemia and hypertension. We examined the tumor from this case and 11 other cases for the presence of renin using a polyclonal antibody to human kidney renin. hypertension was present in three of four additional cases for which records were available. Immunoreactive renin was present in ten of the 12 cases studied. In all of these cases, intense staining was present in vessels within the areas of the trapped cortex. In seven cases, renin was identified in the walls of vessels within the tumor itself without identifiable adjacent cortical structures. These findings indicate that CMN may often be associated with primary reninism in early infancy.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
9/14. Fetal rhabdomyomatous nephroblastoma. Pathologic histology and special clinical and biologic features.Fetal rhabdomyomatous nephroblastoma is a very rare variant of Wilms' tumor. The special clinical and histologic features of this variant are presented on the basis of a case seen in a boy who was 13 months old at nephrectomy 8 months ago and has remained healthy since then. The tumor chiefly consists of fetal striated muscle; it occurs predominantly in infancy and early childhood and is often bilateral. angiography shows a relative paucity of vessels. The volume of the tumor (determined by ultrasonography) decreases only slightly after preoperative radio- and chemotherapy because of a predominance of mesenchymal structures. The finding of nodular renal blastema in renal parenchyma with an apparently normal gross appearance is an argument in favor of classifying this type of tumor in the group of nephroblastomas and may explain the tendency of fetal rhabdomyomatous nephroblastoma to occur bilaterally.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/14. Laser-assisted partial nephrectomy in children.The authors present three case reports showing the efficacy of laser surgery in performing partial nephrectomies in children who had bilateral Wilms' tumors and duplicated systems with obstructed nonfunctioning upper pole moieties. Using both the KTP laser (for cutting) and the YAG laser (for coagulation of large vessels) allows for fast removal of kidney tissue, with minimal blood loss and minimal loss of renal parenchyma.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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