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Xanthogranulomatous pyelonephritis in childhood
INTERNATIONAL
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was performed and showed local xanthogranulomatous pyelonephritis. The displacement of the parenchyma by xanthogranulomata and stage III morphology indicates long-standing disease, probably of prenatal origin.--AmirArmy Xanthogranulomatous Pyelonephritis in Childhood. P.M. Hughes, SC. Gupta, and N.B. Thomas. Clin Radio1 41:360-362,
(May), 1990. A case of multifocal xanthogranulomatous pyelonephritis in a 4-year-old child diagnosed by ultrasound, computed tomography, and needle biopsy was reported. Restoration of function following the use of antibiotic therapy was confirmed by ultrasound and dimercapto succinic acid renal scintiscan.-AmirAzmy
ABSTRACTS
Detection of Neuroblastoma Cells in Blood. T.J. Moss and D.G. Sanders. J Clin Oncol8:736-740, (April), 1990.
The authors present data in 23 patients with disseminated neuroblastoma (10 at diagnosis, 13 during therapy). Seven of 10 patients with newly diagnosed disease and six of 13 patients during therapy for disease were found to have circulating neuroblasts detected by immunocytologic analysis of blood specimens. Those patients on therapy also showed significantly higher likelihood of tumor relapse. The authors advocate the use of this screening test to complement the radiologic, histopathologic, and blood analyses used presently.-Michael P. Hirsh and Ian T. Cohen lmmunoscintigraphic Imaging of MIBG-Negative Metastases in Neuroblastoma. F. Berthold, W Walters, H. Sievetis, et al. Am J
Pediatr Hematol Oncol 12:61-62,199O. NEOPLASMS A Comparison of Four Staging Systems for Localized and Regional Neuroblastoma: A Report From the Children’s Cancer Study Group. A.E. Evans, G.J. D’Angio, H.N. Sather, et al. J
Clin Oncol8:678-688, (April), 1990. This Children’s Cancer Study Group (CCSG) report compares its staging system with those of St Jude’s Hospital, the Pediatric Oncology Group (POG), and the Union Internationale Contre le Cancer TNM system. The authors suggest that all staging systems did well with the low-stage patient and showed appropriate survival decreases with progressively advanced staging. The CCSG system better identified those children with local or regional disease in stage II or III that fared poorly. This apparently is related to the CCSG system being the only one that takes into account the significance of tumor crossing the midline.-Michael P. Hirsh and Ian i? Cohen N-myc Oncogene and Stage IV-S Neuroblastoma: Preliminary Observations on Ten Cases. A Nakagawara, T. Sasazuki, H. Akiyama, et al. Cancer 65:1960-1967, (May), 1990.
Genomic amplification of N-myc was studied in 103 patients with neuroblastoma, including 10 who were in clinical stage IV-S (Evans). Among 41 patients younger than 1 year of age, N-myc amplification of more than three copies was found only in patients in the stage IV-S group. Five infants in Stage IV-S who were less than 1 year of age, four of whom had 1 to 3 copies, and one 4 to 10 copies, are survivors. Among patients in stage IV-S who were less than 1 year of age at diagnosis and also found to have greater than 10 copies, two of three have had recurrence or progression of disease. Among patients greater than 1 year of age, two qualified as stage IV-S, one with amplification of N-myc and one without. Both of these have died. Two patients in clinical stage IV-S (1 and 3 months of age) had massive hepatic involvement and amplification of N-myc of more than 10 copies. Although initial major spontaneous tumor regression occurred, there was subsequent relapse and progressive disease. The increased expression of N-myc mRNA was found in nine of 10 patients in clinical stage IV-S and did not correlate with progression of disease or outcome. The VMA/HVA ratio was low in tumors with increased numbers of genomic copies of N-myc. This study suggests that although the extent of N-myc amplification may affect outcome among patients classified as stage IV-S, it does not correlate with the tumor regression seen in early infancy. In this study, amplification of the genomic N-myc was rare in patients younger than 1 year of age in stages other than IV-S. As in other stages of patients with neuroblastoma, in the stage IV-S group, N-myc amplification is correlated with a poor prognosis and immaturity of catechloamine metabolism.-DanielM Hays
This study showed a comparison of results using Y-metaiodobenzylguanidine (mIBG) scanning with -“Tc-labeled monoclonal antibody (MAb) immunoscintigraphic scanning in seven patients with recurrent stage IV neuroblastoma. Both techniques picked up the majority of tumor sites but had similar false-negative findings that the other modality detected. The authors recommend that both scans be performed to compliment and enhance the results of scanning.-Michael P. Hirsh and Ian T. Cohen Prognosis in Children With Rhabdomyosarcoma (RMS): A Report of the Intergroup RMS Studies I and II. W.M. Crist, L. Gamsey, M.S. Beltangady, et al. J Clin Oncol 8:443-452, (March),
1990. The Children’s Cancer Study Group reports its pretreatment prognosis data on patients entered into Intergroup Rhabdomyosarcoma Study I (IRS I; 19721978; #686) and Intergroup Rhabdomyosarcoma Study II (IRS II; 1978-1984; #1002). The S-year survival was 56% in IRS I and 62% in IRS II. The clinical grouping was the most important prognostic characteristic in both groups with survival being best (82%) in group I (local disease completely resected) and worst (24%) in group IV (metastatic disease). The prognostic importance of pathological features varied with clinical group. In group I, alveolar tumors fared the poorest; embryonalibotryoid tumors did best. In group II (comprised or regional resection) no characteristic had prognostic significance. In group II (incomplete resection) orbital primaries did best; in group IV, genitourinary tumors did best. The data from this analysis will help design therapy plans for IRS IV.-Michael P. Hirsh and Ian T. Cohen Soft-Tissue Sarcoma of the Perineal Region in Childhood. R.B. Raney, W Crist, D.M. Hays, et al. Cancer 6512787-2792,(June
15), 1990. This report from the Intergroup Rhabdomyosarcoma Study (IRS) concerns 36 patients less than 21 years of age with perineal rhabdomyosarcomas reported during a 12-year interval (1972 to 1984). Tumors in this site were predominantly (56%) alveolar. Thus, this constitutes the site with the highest incidence of this specific subtype in the IRS. Gross complete surgical excision (clinical groups I and II) was performed in 15 children, 15 had incomplete tumor excisions, and six had metastatic disease at diagnosis. Among children with complete tumor excision (margins confirmed) four of four are disease-free with an estimated 3-year disease-free survival of 100%. All of these patients had two initial surgical procedures, ie, a gross excision followed by a primary reexcision (PRE) because of unanticipated microscopic residual at the margin of the specimen removed at the initial procedure. They received no subsequent
120
was performed and showed local xanthogranulomatous pyelonephritis. The displacement of the parenchyma by xanthogranulomata and stage III morphology indicates long-standing disease, probably of prenatal origin.--AmirArmy Xanthogranulomatous Pyelonephritis in Childhood. P.M. Hughes, SC. Gupta, and N.B. Thomas. Clin Radio1 41:360-362,
(May), 1990. A case of multifocal xanthogranulomatous pyelonephritis in a 4-year-old child diagnosed by ultrasound, computed tomography, and needle biopsy was reported. Restoration of function following the use of antibiotic therapy was confirmed by ultrasound and dimercapto succinic acid renal scintiscan.-AmirAzmy
ABSTRACTS
Detection of Neuroblastoma Cells in Blood. T.J. Moss and D.G. Sanders. J Clin Oncol8:736-740, (April), 1990.
The authors present data in 23 patients with disseminated neuroblastoma (10 at diagnosis, 13 during therapy). Seven of 10 patients with newly diagnosed disease and six of 13 patients during therapy for disease were found to have circulating neuroblasts detected by immunocytologic analysis of blood specimens. Those patients on therapy also showed significantly higher likelihood of tumor relapse. The authors advocate the use of this screening test to complement the radiologic, histopathologic, and blood analyses used presently.-Michael P. Hirsh and Ian T. Cohen lmmunoscintigraphic Imaging of MIBG-Negative Metastases in Neuroblastoma. F. Berthold, W Walters, H. Sievetis, et al. Am J
Pediatr Hematol Oncol 12:61-62,199O. NEOPLASMS A Comparison of Four Staging Systems for Localized and Regional Neuroblastoma: A Report From the Children’s Cancer Study Group. A.E. Evans, G.J. D’Angio, H.N. Sather, et al. J
Clin Oncol8:678-688, (April), 1990. This Children’s Cancer Study Group (CCSG) report compares its staging system with those of St Jude’s Hospital, the Pediatric Oncology Group (POG), and the Union Internationale Contre le Cancer TNM system. The authors suggest that all staging systems did well with the low-stage patient and showed appropriate survival decreases with progressively advanced staging. The CCSG system better identified those children with local or regional disease in stage II or III that fared poorly. This apparently is related to the CCSG system being the only one that takes into account the significance of tumor crossing the midline.-Michael P. Hirsh and Ian i? Cohen N-myc Oncogene and Stage IV-S Neuroblastoma: Preliminary Observations on Ten Cases. A Nakagawara, T. Sasazuki, H. Akiyama, et al. Cancer 65:1960-1967, (May), 1990.
Genomic amplification of N-myc was studied in 103 patients with neuroblastoma, including 10 who were in clinical stage IV-S (Evans). Among 41 patients younger than 1 year of age, N-myc amplification of more than three copies was found only in patients in the stage IV-S group. Five infants in Stage IV-S who were less than 1 year of age, four of whom had 1 to 3 copies, and one 4 to 10 copies, are survivors. Among patients in stage IV-S who were less than 1 year of age at diagnosis and also found to have greater than 10 copies, two of three have had recurrence or progression of disease. Among patients greater than 1 year of age, two qualified as stage IV-S, one with amplification of N-myc and one without. Both of these have died. Two patients in clinical stage IV-S (1 and 3 months of age) had massive hepatic involvement and amplification of N-myc of more than 10 copies. Although initial major spontaneous tumor regression occurred, there was subsequent relapse and progressive disease. The increased expression of N-myc mRNA was found in nine of 10 patients in clinical stage IV-S and did not correlate with progression of disease or outcome. The VMA/HVA ratio was low in tumors with increased numbers of genomic copies of N-myc. This study suggests that although the extent of N-myc amplification may affect outcome among patients classified as stage IV-S, it does not correlate with the tumor regression seen in early infancy. In this study, amplification of the genomic N-myc was rare in patients younger than 1 year of age in stages other than IV-S. As in other stages of patients with neuroblastoma, in the stage IV-S group, N-myc amplification is correlated with a poor prognosis and immaturity of catechloamine metabolism.-DanielM Hays
This study showed a comparison of results using Y-metaiodobenzylguanidine (mIBG) scanning with -“Tc-labeled monoclonal antibody (MAb) immunoscintigraphic scanning in seven patients with recurrent stage IV neuroblastoma. Both techniques picked up the majority of tumor sites but had similar false-negative findings that the other modality detected. The authors recommend that both scans be performed to compliment and enhance the results of scanning.-Michael P. Hirsh and Ian T. Cohen Prognosis in Children With Rhabdomyosarcoma (RMS): A Report of the Intergroup RMS Studies I and II. W.M. Crist, L. Gamsey, M.S. Beltangady, et al. J Clin Oncol 8:443-452, (March),
1990. The Children’s Cancer Study Group reports its pretreatment prognosis data on patients entered into Intergroup Rhabdomyosarcoma Study I (IRS I; 19721978; #686) and Intergroup Rhabdomyosarcoma Study II (IRS II; 1978-1984; #1002). The S-year survival was 56% in IRS I and 62% in IRS II. The clinical grouping was the most important prognostic characteristic in both groups with survival being best (82%) in group I (local disease completely resected) and worst (24%) in group IV (metastatic disease). The prognostic importance of pathological features varied with clinical group. In group I, alveolar tumors fared the poorest; embryonalibotryoid tumors did best. In group II (comprised or regional resection) no characteristic had prognostic significance. In group II (incomplete resection) orbital primaries did best; in group IV, genitourinary tumors did best. The data from this analysis will help design therapy plans for IRS IV.-Michael P. Hirsh and Ian T. Cohen Soft-Tissue Sarcoma of the Perineal Region in Childhood. R.B. Raney, W Crist, D.M. Hays, et al. Cancer 6512787-2792,(June
15), 1990. This report from the Intergroup Rhabdomyosarcoma Study (IRS) concerns 36 patients less than 21 years of age with perineal rhabdomyosarcomas reported during a 12-year interval (1972 to 1984). Tumors in this site were predominantly (56%) alveolar. Thus, this constitutes the site with the highest incidence of this specific subtype in the IRS. Gross complete surgical excision (clinical groups I and II) was performed in 15 children, 15 had incomplete tumor excisions, and six had metastatic disease at diagnosis. Among children with complete tumor excision (margins confirmed) four of four are disease-free with an estimated 3-year disease-free survival of 100%. All of these patients had two initial surgical procedures, ie, a gross excision followed by a primary reexcision (PRE) because of unanticipated microscopic residual at the margin of the specimen removed at the initial procedure. They received no subsequent