421/1062. Contribution of angiography to the diagnosis, staging and assessment of radiation and chemotherapy of solid abdominal malignancies in children. Sequential angiographic studies were done in six children to stage and assess the results of radiation and/or chemotherapy of solid abdominal malignancies: one bilateral Wilms' tumor, two neuroblastomas, two hepatoblastomas and one hepatocarcinoma. angiography was of value in demonstrating the tumor, its location, extent and vascular characteristics, as well as its regression and recurrence. Wilms' tumor and neuroblastoma responded and well to radiation and chemotherapy with substantial decrease in tumor size and regression or disappearance of tumor neovasculature. Resceted tumors revealed this to be due to tumor necrosis, hemorrhage and/or cystic degeneration. hepatoblastoma and hepatocarcinoma did not respond as well to chemotherapy, with only mild decrease in size and neovasculature of the tumor. ( info) |
| We present cytogenetic and molecular genetic analyses of two cases of alveolar rhabdomyosarcoma. The characteristic translocation between chromosomes 2 and 13, t(2;13)(q35;q14), has been identified in both cases. Using cell lines derived from these tumor specimens, we have performed Southern blot analysis to investigate the possibility of rearrangement of 14 candidate genes mapping to the relevant regions of 2q and 13q. These candidate genes can be divided into 5 groups: signal transduction proteins (RB1, inhibin alpha, FLT1, and HOX4B), muscle-specific products [myosin light chain, desmin, and nicotinic cholinergic receptor subunits gamma and delta (CHRNG and CHRND)], extracellular matrix proteins (collagen type vi alpha 3 chain, elastin, and fibronectin), transformation-associated products (intestinal alkaline phosphatase and L-plastin), and other genes (esterase D). Conventional gel electrophoresis followed by Southern blot analysis indicated no evidence of rearrangement within or near these genes except for a rearrangement in the CHRNG-CHRND locus, which occurred only in a subpopulation of the late recurrence tumor cells of one patient. In addition, we employed pulsed-field gel electrophoresis-Southern blot analysis to demonstrate the absence of detectable rearrangements within a larger region around each of these genes. ( info) |
423/1062. Constitutional translocation t(1;17)(p36;q12-21) in a patient with neuroblastoma. A constitutional chromosome translocation t(1;17)(p36;q12-21) is reported in a boy with neuroblastoma. This is the first description of a cytogenetic abnormality possibly predisposing to the development of neuroblastoma. ( info) |
424/1062. Autologous tumor-specific cytotoxic t-lymphocytes in a child with neuroblastoma. Activation of peripheral blood lymphocytes from a neuroblastoma patient by co-cultivation with autologous neuroblastoma cells in a mixed lymphocyte-tumor cell culture (A-MLTC) resulted in the generation of cytotoxic activity against the autologous neuroblastoma cell line HNB-MS. A-MLTC was set up in the presence of recombinant human interleukin-2 (IL-2). HNB-MS stimulator was treated with recombinant human interferon-gamma (IFN-gamma) prior to A-MLTC. CTL generated in short-term culture effectively lysed HNB-MS, while they had no effect on an Epstein-Barr virus transformed autologous B-cell line EB-MS. Moreover, CTL lysed 3 different allogeneic neuroblastoma cell lines, but not a rhabdomyosarcoma cell line RBB. Recombinant human tumor necrosis factor-alpha (TNF-alpha) and interleukin-4 (IL-4) enhanced and suppressed CTL generation, respectively, when added to the A-MLTC from the beginning of culture. CD3 CD4- CD8 T cells were the major anti-tumor effectors. Furthermore, 111indium-labeled CTL clearly accumulated in metastatic sites. These results indicate that CTL can be used for adoptive immunotherapy in neuroblastoma. ( info) |
425/1062. Tumor invasion of the upper inferior vena cava: the use of profound hypothermia and circulation arrest as a surgical adjunct. Two children presented with abdominal tumors invading the upper inferior vena cava. To facilitate removal of the tumors, cardiopulmonary bypass with profound hypothermia and circulation arrest were utilized. This technique simplifies tumor excision and is worthwhile even in children with advanced malignant disease. ( info) |
| A rare case of primary renal ganglioneuroblastoma in a 68 year old female is presented. The authors believe this to be the first case report of this tumour arising in the kidney of an adult patient. ( info) |
427/1062. Ultrasound guided fine-needle aspiration biopsy of abdominal masses. Twenty-six cases of ultrasonically guided percutaneous fine-needle aspiration biopsies of abdominal masses are reported. Included are masses in the pancreas, retroperitoneum, liver, kidney, and pelvis. Aspiration biopsies accurately diagnosed or excluded malignancy in 21 patients (81%). The procedure, performed under local anesthesia, is rapid, simple and almost painless. No complications occurred; specifically, there was no hemorrhage, peritonitis, or spread of tumor along the needle tract. ( info) |
428/1062. splenosis simulating an abdominal lymphoma. splenosis, an autograft of splenic tissue, may occur after a traumatic spleen rupture or splenectomy. Usually asymptomatic, this pathology may uncommonly produce symptoms which justify radiologic investigations. We are presenting the case of a young boy with a Wiskott Aldrich syndrome and who developed splenosis simulating an abdominal lymphoma. ( info) |
429/1062. Malakoplakia. Two unusual cases which presented diagnostic problems. Two unusual cases of malakoplakia are presented. Both cases occurred outside the urinary tract (retroperitoneum and anus) and were diagnostic problems due to the paucity of Michaelis-Guttman bodies. One case mimicked a retroperitoneal malignant fibrous histiocytoma and the other a common perianal abcess. ( info) |
430/1062. VIP secreting tumours in infancy. A review of radiological appearances. Vasoactive intestinal polypeptide (VIP) secreting neural crest tumours are an uncommon but important treatable cause of intractable childhood diarrhoea. The radiological appearances of two cases are presented with a review of radiological findings in childhood VIP secreting neural crest tumours. Twenty eight cases of childhood VIP secreting neural crest tumours were reviewed. Nineteen (68%) were ganglioneuroblastomas and nine (32%) were ganglioneuromas. The majority of tumours (66%) were in a paravertebral location in the abdomen indicating that a search for such a tumour should be initiated at this site. Eighteen of the twenty-eight cases reviewed discussed relevant radiological investigations. Calcification was detected in 50% of abdominal radiographs. Gut dilatation was often a prominent feature. A mass was detected in 5 of 5 cases where ultrasound findings were reported, and seven of seven cases with CT findings reported. Prior to the availability of CT and ultrasound the most useful investigation was IVU which demonstrated evidence of a mass in 5 of 9 cases. The presence of paravertebral calcification and gut dilatation on the plain radiograph of a child with intractable diarrhoea suggests the presence of a VIP secreting neural crest tumour. If an abdominal tumour is not found in the appropriate clinical setting and VIP levels are elevated, a widespread search of the paravertebral region is indicated. ( info) |