{"title":"Rhabdomyosarcoma of the hand.","authors":"R. Milanović, M. Žganjer, A. C̆izmić, A. Pajić","doi":"10.1002/MPO.1236","DOIUrl":"https://doi.org/10.1002/MPO.1236","url":null,"abstract":"","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"30 1","pages":"481"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82541901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
R. Sposto, A. Meadows, R. Chilcote, P. Steinherz, C. Kjeldsberg, M. Kadin, M. Krailo, A. Termuhlen, M. Morse, S. Siegel
BACKGROUND Early Children's Cancer Group (CCG) trials indicated that the cyclophosphamide, vincristine, methotrexate, and prednisone (COMP) regimen was superior to the LSA2L2 regimen for non-lymphoblastic (NLB) non-Hodgkin lymphoma (NHL). Studies by other groups suggested that addition of anthracyclines to standard therapies could improve outcome. Therefore, in 1983 CCG initiated study CCG-503, a randomized trial of COMP vs. daunomycin-COMP (D-COMP) in children and adolescents with disseminated NLB NHL. PROCEDURES Between December 1983 and April 1990, 404 eligible patients were entered. Patients without central nervous system (CNS) or marrow involvement were randomized to receive COMP (N = 139) or D-COMP (N = 145). Randomization was stratified by histology and site of disease. Patients with CNS or marrow involvement (stage IV) were non-randomly treated with D-COMP (N = 120). RESULTS Ten-year event-free survival in COMP and D-COMP patients was similar: 55 +/- 4.3% (Estimate +/- SE) vs. 57 +/- 4.2% (not significant). Stage I-III patients with large-cell (LC) NHL had worse 10-year event-free survival (EFS) (48 +/- 4.9%) than those with small non-cleaved cell (SNCC) NHL disease (61 +/- 3.5%, P < 0.05 in multivariate analysis), but equivalent survival (65 +/- 4.7% vs. 63 +/- 3.5%) due to significantly higher salvage rates in LC patients, especially those failing more than 12 months from diagnosis. Ten-year EFS in stage IV patients was 39 +/- 5.2%. Addition of daunomycin resulted in higher rates of grade 3/4 hematologic toxicity and stomatitis, as well as late cardiac-related deaths. The incidence of second malignant neoplasms was 1.0% at 10 years. CONCLUSIONS Addition of daunomycin to standard COMP therapy did not improve outcome in pediatric disseminated NLB NHL. Patients with LC disease had a significantly reduced long-term EFS, but were retrieved at a higher rate than patients with SNCC disease, resulting in equivalent long-term survival.
{"title":"Comparison of long-term outcome of children and adolescents with disseminated non-lymphoblastic non-Hodgkin lymphoma treated with COMP or daunomycin-COMP: A report from the Children's Cancer Group.","authors":"R. Sposto, A. Meadows, R. Chilcote, P. Steinherz, C. Kjeldsberg, M. Kadin, M. Krailo, A. Termuhlen, M. Morse, S. Siegel","doi":"10.1002/MPO.1226","DOIUrl":"https://doi.org/10.1002/MPO.1226","url":null,"abstract":"BACKGROUND\u0000Early Children's Cancer Group (CCG) trials indicated that the cyclophosphamide, vincristine, methotrexate, and prednisone (COMP) regimen was superior to the LSA2L2 regimen for non-lymphoblastic (NLB) non-Hodgkin lymphoma (NHL). Studies by other groups suggested that addition of anthracyclines to standard therapies could improve outcome. Therefore, in 1983 CCG initiated study CCG-503, a randomized trial of COMP vs. daunomycin-COMP (D-COMP) in children and adolescents with disseminated NLB NHL.\u0000\u0000\u0000PROCEDURES\u0000Between December 1983 and April 1990, 404 eligible patients were entered. Patients without central nervous system (CNS) or marrow involvement were randomized to receive COMP (N = 139) or D-COMP (N = 145). Randomization was stratified by histology and site of disease. Patients with CNS or marrow involvement (stage IV) were non-randomly treated with D-COMP (N = 120).\u0000\u0000\u0000RESULTS\u0000Ten-year event-free survival in COMP and D-COMP patients was similar: 55 +/- 4.3% (Estimate +/- SE) vs. 57 +/- 4.2% (not significant). Stage I-III patients with large-cell (LC) NHL had worse 10-year event-free survival (EFS) (48 +/- 4.9%) than those with small non-cleaved cell (SNCC) NHL disease (61 +/- 3.5%, P < 0.05 in multivariate analysis), but equivalent survival (65 +/- 4.7% vs. 63 +/- 3.5%) due to significantly higher salvage rates in LC patients, especially those failing more than 12 months from diagnosis. Ten-year EFS in stage IV patients was 39 +/- 5.2%. Addition of daunomycin resulted in higher rates of grade 3/4 hematologic toxicity and stomatitis, as well as late cardiac-related deaths. The incidence of second malignant neoplasms was 1.0% at 10 years.\u0000\u0000\u0000CONCLUSIONS\u0000Addition of daunomycin to standard COMP therapy did not improve outcome in pediatric disseminated NLB NHL. Patients with LC disease had a significantly reduced long-term EFS, but were retrieved at a higher rate than patients with SNCC disease, resulting in equivalent long-term survival.","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"11 1","pages":"432-41"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77856810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Torben Ek, M. Jarfelt, L. Mellander, J. Abrahamsson
BACKGROUND Treatment with high-dose cytarabine (1-beta-D-arabinofuranosylcytosine) is often associated with an acute febrile reaction sometimes including abdominal pain, myalgia, and rash. The similarity of these symptoms to those caused by hypersecretion of cytokines in the systemic inflammatory response syndrome (SIRS) prompted us to investigate the plasma levels of proinflammatory cytokines during treatment of children with high-dose cytarabine. PROCEDURE Sixteen children treated for hematological malignancies and in clinical remission were studied during treatment with six infusions of cytarabine given every 12 hr at a dose of 2 g/m(2). Blood samples for analysis of tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), interleukin-1gamma (IL-1gamma), interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-10 (IL-10) and interleukin-1 receptor antagonist (IL-1ra) were obtained prior to treatment and subsequently at 12, 36 and 60 hr. Additional samples were collected as soon as fever occurred. RESULTS Thirteen of 16 patients developed fever at a median time of 30 hr following start of treatment. At 12 hr levels of TNF-alpha were elevated followed by a rise in IL-6, IFN-alpha, and IL-1ra, peaking at the onset of fever. Thereafter these levels slowly declined whereas low IL-10 levels became detectable. CONCLUSIONS We conclude that high-dose cytarabine treatment often induces release of TNF-alpha followed by the sequential release of other proinflammatory cytokines. Most likely these cytokines mediate the development of symptoms comprising the cytarabine syndrome.
{"title":"Proinflammatory cytokines mediate the systemic inflammatory response associated with high-dose cytarabine treatment in children.","authors":"Torben Ek, M. Jarfelt, L. Mellander, J. Abrahamsson","doi":"10.1002/MPO.1230","DOIUrl":"https://doi.org/10.1002/MPO.1230","url":null,"abstract":"BACKGROUND\u0000Treatment with high-dose cytarabine (1-beta-D-arabinofuranosylcytosine) is often associated with an acute febrile reaction sometimes including abdominal pain, myalgia, and rash. The similarity of these symptoms to those caused by hypersecretion of cytokines in the systemic inflammatory response syndrome (SIRS) prompted us to investigate the plasma levels of proinflammatory cytokines during treatment of children with high-dose cytarabine.\u0000\u0000\u0000PROCEDURE\u0000Sixteen children treated for hematological malignancies and in clinical remission were studied during treatment with six infusions of cytarabine given every 12 hr at a dose of 2 g/m(2). Blood samples for analysis of tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), interleukin-1gamma (IL-1gamma), interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-10 (IL-10) and interleukin-1 receptor antagonist (IL-1ra) were obtained prior to treatment and subsequently at 12, 36 and 60 hr. Additional samples were collected as soon as fever occurred.\u0000\u0000\u0000RESULTS\u0000Thirteen of 16 patients developed fever at a median time of 30 hr following start of treatment. At 12 hr levels of TNF-alpha were elevated followed by a rise in IL-6, IFN-alpha, and IL-1ra, peaking at the onset of fever. Thereafter these levels slowly declined whereas low IL-10 levels became detectable.\u0000\u0000\u0000CONCLUSIONS\u0000We conclude that high-dose cytarabine treatment often induces release of TNF-alpha followed by the sequential release of other proinflammatory cytokines. Most likely these cytokines mediate the development of symptoms comprising the cytarabine syndrome.","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"2 1","pages":"459-64"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84173589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Kanerva, T. Niini, K. Vettenranta, P. Riikonen, A. Mäkipernaa, R. Karhu, S. Knuutila, U. Saarinen‐Pihkala
BACKGROUND Genetic aberrations provide prognostic information in childhood ALL. The proportion of patients with detectable aberrations can be increased by combining G-banding with comparative genomic hybridization (CGH). PROCEDURE We studied 79 children with ALL by CGH and G-banding, and explored the relationship of these findings to clinical features and outcome. RESULTS CGH revealed DNA copy number changes in 57 patients (72%), 9 of whom had normal karyotype by G-banding. Gains were more frequent than losses, and changes of whole chromosomes more frequent than partial aberrations. Two frequent partial losses were found; at 9p and 12p. The 9 patients with loss at 12p were studied for the deletion of TEL (ETV6) gene and the fusion of TEL and AML1 genes by fluorescent in situ hybridization (FISH). Eight out of the 9 children with loss at 12p harbored the TEL-AML1 translocation and all 9 had the deletion of a nontranslocated TEL allele. All 9 had precursor-B phenotype and L1 morphology, and 8/9 had WBC below 50 x 10(9)/liter. All children were treated according to Nordic ALL protocols, had a good response to treatment based on day 15 bone marrow morphology, and 7 out of the 9 survived in continuous complete remission (median follow-up 74 months). CONCLUSIONS CGH is a valuable tool in screening for genetic aberrations in childhood ALL. DNA copy number losses detected at 12p associate with TEL-AML1 fusion as well as with favorable prognostic features.
{"title":"Loss at 12p detected by comparative genomic hybridization (CGH): association with TEL-AML1 fusion and favorable prognostic features in childhood acute lymphoblastic leukemia (ALL). A multi-institutional study.","authors":"J. Kanerva, T. Niini, K. Vettenranta, P. Riikonen, A. Mäkipernaa, R. Karhu, S. Knuutila, U. Saarinen‐Pihkala","doi":"10.1002/MPO.1224","DOIUrl":"https://doi.org/10.1002/MPO.1224","url":null,"abstract":"BACKGROUND\u0000Genetic aberrations provide prognostic information in childhood ALL. The proportion of patients with detectable aberrations can be increased by combining G-banding with comparative genomic hybridization (CGH).\u0000\u0000\u0000PROCEDURE\u0000We studied 79 children with ALL by CGH and G-banding, and explored the relationship of these findings to clinical features and outcome.\u0000\u0000\u0000RESULTS\u0000CGH revealed DNA copy number changes in 57 patients (72%), 9 of whom had normal karyotype by G-banding. Gains were more frequent than losses, and changes of whole chromosomes more frequent than partial aberrations. Two frequent partial losses were found; at 9p and 12p. The 9 patients with loss at 12p were studied for the deletion of TEL (ETV6) gene and the fusion of TEL and AML1 genes by fluorescent in situ hybridization (FISH). Eight out of the 9 children with loss at 12p harbored the TEL-AML1 translocation and all 9 had the deletion of a nontranslocated TEL allele. All 9 had precursor-B phenotype and L1 morphology, and 8/9 had WBC below 50 x 10(9)/liter. All children were treated according to Nordic ALL protocols, had a good response to treatment based on day 15 bone marrow morphology, and 7 out of the 9 survived in continuous complete remission (median follow-up 74 months).\u0000\u0000\u0000CONCLUSIONS\u0000CGH is a valuable tool in screening for genetic aberrations in childhood ALL. DNA copy number losses detected at 12p associate with TEL-AML1 fusion as well as with favorable prognostic features.","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"30 1","pages":"419-25"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76267591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Jadhav, B. Cushing, O. Ozdemir, A. Mohamed, Y. Ravindranath, S. Savaşan
Translocations involving the MLL gene located at 11q23 have been reported in acute lymphoblastic leukemia (ALL), in 5±10% of cases with acute myeloid leukemia (AML), frequently of the monocytic type, and in biphenotypic leukemias expressing early stem cell as well as both myeloid and lymphoid markers. MLL gene rearrangement is the most common cytogenetic abnormality in infant leukemias and it can occur in therapyrelated leukemias as well. Trisomy 11 is the fourth most common acquired trisomy, occurring in 1% of AML/MDS cases [1]. Leukemias with trisomy 11 tend to express CD34, HLA-DR, myeloid antigens CD15, CD13 or CD33, and occasionally CD19. These leukemias are associated with a poor prognosis similar to cases with MLL gene rearrangements [1]. Recently, Schnittger et al. [2], in their study of 387 patients, reported the partial tandem duplication of the MLL gene, leading to the fusion of the proto-oncogene with itself, in 5.7% of AML patients with normal karyotypes, in 37.5% of cases with trisomy 11 with other cytogenetic abnormalities, and in 79% of cases with trisomy 11 as the sole karyotypic abnormality. Patients with this duplication had varying FAB morphologies and a poor outcome. In addition to their possible contribution to malignant transformation individually, the high incidence of partial tandem duplication of the MLL gene in cases with trisomy 11 suggests a link between these two cytogenetic events. Our experience with a 13-year-old south Asian girl is relevant. She had biphenotypic leukemia and at relapse, was found to have trisomy 11 detected by FISH, but not by conventional G banding. She presented with pancytopenia in December 1998. Family history was signi®cant for several cancers (liver, lung, colon, and brain) among close family members. A maternal aunt with Fanconi anemia developed AML at 5 years of age. Physical examination of the patient was not suggestive of Fanconi anemia and her diepoxybutane (DEB)-induced chromosomal breakage studies performed at the time of relapse were negative. At diagnosis, the patient's bone marrow aspirate revealed blasts of predominant L1 morphology; other blasts were large with prominent nuclei. The blasts were strongly positive for CD10, HLA-DR, CD19, CD22, CD34 and showed milder expression of myeloid markers CD15 and CD13. Cytochemical staining revealed the blasts to be positive with Sudan black and 3±5% also were positive with peroxidase. Conventional G banding revealed a small number of metaphase cells that were 46 XX. A FISH study for the MLL gene revealed two probe signals in both metaphase and interphase. The patient had a good response to a high risk ALL protocol that included high-dose methotrexate, cytarabine, anthracyclines, and teniposide. After 17 months of treatment, while on maintenance therapy, the leukemia relapsed in the bone marrow. Analysis of relapse blasts had similar surface markers but had lost CD34 and Sudan black positivity. The blasts showed no clonal Ig-H variable reg
{"title":"Clonal trisomy 11 in a child with acute leukemia: G banding vs. FISH.","authors":"M. Jadhav, B. Cushing, O. Ozdemir, A. Mohamed, Y. Ravindranath, S. Savaşan","doi":"10.1002/MPO.1233","DOIUrl":"https://doi.org/10.1002/MPO.1233","url":null,"abstract":"Translocations involving the MLL gene located at 11q23 have been reported in acute lymphoblastic leukemia (ALL), in 5±10% of cases with acute myeloid leukemia (AML), frequently of the monocytic type, and in biphenotypic leukemias expressing early stem cell as well as both myeloid and lymphoid markers. MLL gene rearrangement is the most common cytogenetic abnormality in infant leukemias and it can occur in therapyrelated leukemias as well. Trisomy 11 is the fourth most common acquired trisomy, occurring in 1% of AML/MDS cases [1]. Leukemias with trisomy 11 tend to express CD34, HLA-DR, myeloid antigens CD15, CD13 or CD33, and occasionally CD19. These leukemias are associated with a poor prognosis similar to cases with MLL gene rearrangements [1]. Recently, Schnittger et al. [2], in their study of 387 patients, reported the partial tandem duplication of the MLL gene, leading to the fusion of the proto-oncogene with itself, in 5.7% of AML patients with normal karyotypes, in 37.5% of cases with trisomy 11 with other cytogenetic abnormalities, and in 79% of cases with trisomy 11 as the sole karyotypic abnormality. Patients with this duplication had varying FAB morphologies and a poor outcome. In addition to their possible contribution to malignant transformation individually, the high incidence of partial tandem duplication of the MLL gene in cases with trisomy 11 suggests a link between these two cytogenetic events. Our experience with a 13-year-old south Asian girl is relevant. She had biphenotypic leukemia and at relapse, was found to have trisomy 11 detected by FISH, but not by conventional G banding. She presented with pancytopenia in December 1998. Family history was signi®cant for several cancers (liver, lung, colon, and brain) among close family members. A maternal aunt with Fanconi anemia developed AML at 5 years of age. Physical examination of the patient was not suggestive of Fanconi anemia and her diepoxybutane (DEB)-induced chromosomal breakage studies performed at the time of relapse were negative. At diagnosis, the patient's bone marrow aspirate revealed blasts of predominant L1 morphology; other blasts were large with prominent nuclei. The blasts were strongly positive for CD10, HLA-DR, CD19, CD22, CD34 and showed milder expression of myeloid markers CD15 and CD13. Cytochemical staining revealed the blasts to be positive with Sudan black and 3±5% also were positive with peroxidase. Conventional G banding revealed a small number of metaphase cells that were 46 XX. A FISH study for the MLL gene revealed two probe signals in both metaphase and interphase. The patient had a good response to a high risk ALL protocol that included high-dose methotrexate, cytarabine, anthracyclines, and teniposide. After 17 months of treatment, while on maintenance therapy, the leukemia relapsed in the bone marrow. Analysis of relapse blasts had similar surface markers but had lost CD34 and Sudan black positivity. The blasts showed no clonal Ig-H variable reg","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"97 1","pages":"475-6"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76466822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The role of radiation therapy in children with acute lymphoblastic leukemia kidney infiltration.","authors":"S. Dahlbeck, M. Tome, A. Kagan, R. Cooper","doi":"10.1002/MPO.1234","DOIUrl":"https://doi.org/10.1002/MPO.1234","url":null,"abstract":"","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"14 1","pages":"477-8"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82063129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
N. Peylan‐Ramu, Bin-Nun Alona, Miri Skleir‐Levy, Arie Bibas,, B. Koplewitz, I. Anteby, J. Pe’er
BACKGROUND Orbital growth retardation, after enucleation and/or external beam radiation for retinoblastoma (RB), is a serious late effect. We measured orbital volumes of RB survivors treated at Hadassah University Hospital, Jerusalem, between 1980-1998. PROCEDURE Forty-five orbits of 28 children with RB (17 bilateral, 11 unilateral) were examined. Thirty-six orbits were irradiated, 19 enucleated, and 10 both enucleated and irradiated. The orbital volumes were calculated from a three-dimensional orbital CT reconstruction. The orbits of RB survivors were compared to age-matched controls. RESULTS The mean age at diagnosis was 13 months, mean follow-up time was 56 months. The mean volume of RB orbits (14.4 cc) was statistically significantly smaller than control orbits (17.8 cc). There was no difference between the mean volume of orbits treated with enucleation, irradiation or both. The orbital volume of children treated before the age of 12 months was statistically significantly smaller than those treated later. There was no difference between mean volume of fellow orbits in unilateral RB and controls. The mean orbital asymmetry index in control children (2.6%) was statistically significantly smaller than in RB survivors (14%). CONCLUSIONS There was a significant orbital growth retardation after enucleation and/or irradiation for RB. There was no difference between mean orbital volumes after enucleation, radiation or both. Orbital growth retardation was most prominent in children treated in the first year of life. Although small in number, our study suggests that deferring enucleation and/or irradiation until after the age of 12 months may reduce long-term complications.
{"title":"Orbital growth retardation in retinoblastoma survivors: work in progress.","authors":"N. Peylan‐Ramu, Bin-Nun Alona, Miri Skleir‐Levy, Arie Bibas,, B. Koplewitz, I. Anteby, J. Pe’er","doi":"10.1002/MPO.1231","DOIUrl":"https://doi.org/10.1002/MPO.1231","url":null,"abstract":"BACKGROUND\u0000Orbital growth retardation, after enucleation and/or external beam radiation for retinoblastoma (RB), is a serious late effect. We measured orbital volumes of RB survivors treated at Hadassah University Hospital, Jerusalem, between 1980-1998.\u0000\u0000\u0000PROCEDURE\u0000Forty-five orbits of 28 children with RB (17 bilateral, 11 unilateral) were examined. Thirty-six orbits were irradiated, 19 enucleated, and 10 both enucleated and irradiated. The orbital volumes were calculated from a three-dimensional orbital CT reconstruction. The orbits of RB survivors were compared to age-matched controls.\u0000\u0000\u0000RESULTS\u0000The mean age at diagnosis was 13 months, mean follow-up time was 56 months. The mean volume of RB orbits (14.4 cc) was statistically significantly smaller than control orbits (17.8 cc). There was no difference between the mean volume of orbits treated with enucleation, irradiation or both. The orbital volume of children treated before the age of 12 months was statistically significantly smaller than those treated later. There was no difference between mean volume of fellow orbits in unilateral RB and controls. The mean orbital asymmetry index in control children (2.6%) was statistically significantly smaller than in RB survivors (14%).\u0000\u0000\u0000CONCLUSIONS\u0000There was a significant orbital growth retardation after enucleation and/or irradiation for RB. There was no difference between mean orbital volumes after enucleation, radiation or both. Orbital growth retardation was most prominent in children treated in the first year of life. Although small in number, our study suggests that deferring enucleation and/or irradiation until after the age of 12 months may reduce long-term complications.","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"58 1","pages":"465-70"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74731736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Sredni, B. Camargo, L. F. Lopes, R. Teixeira, Andrew K. Simpson
BACKGROUND Mutations of the tumor suppressor gene p53 are commonly found in several kinds of human cancer. In some types of neoplasms, accumulation of p53 protein has been reported to correlate with more aggressive clinical behavior. The role of p53 expression in Wilms tumors (WT) is not clear yet, but most studies have confirmed its correlation with anaplasia and advanced stage disease. PROCEDURE Ninety-seven WT were evaluated for p53 expression by immunohistochemistry in formalin-fixed paraffin-embedded tissue and correlated with outcome. Tumors were classified as p53-Negative (p53-N) when no positivity was observed or only few cells showed weak positivity (0/1+) and p53-Positive (p53-P) when there was a diffuse and strong nuclear positivity (2+/3+). RESULTS p53-P was detected in 13 out of 97 tumors and was associated with disease relapse (39 vs.17%; P = 0.06) but not with anaplasia. Among p53-N patients only 5% had metastatic disease compared with 31% of the p53-P sample. (P = 0.038). Overall survival was 94% for patients with p53-N vs. 85% for patients with p53-P at 1 year (P = 0.34). CONCLUSIONS p53 expression in Wilms tumor detected by immunohistochemistry seems to be associated with advanced disease and relapse.
{"title":"Immunohistochemical detection of p53 protein expression as a prognostic indicator in Wilms tumor.","authors":"S. Sredni, B. Camargo, L. F. Lopes, R. Teixeira, Andrew K. Simpson","doi":"10.1002/MPO.1229","DOIUrl":"https://doi.org/10.1002/MPO.1229","url":null,"abstract":"BACKGROUND\u0000Mutations of the tumor suppressor gene p53 are commonly found in several kinds of human cancer. In some types of neoplasms, accumulation of p53 protein has been reported to correlate with more aggressive clinical behavior. The role of p53 expression in Wilms tumors (WT) is not clear yet, but most studies have confirmed its correlation with anaplasia and advanced stage disease.\u0000\u0000\u0000PROCEDURE\u0000Ninety-seven WT were evaluated for p53 expression by immunohistochemistry in formalin-fixed paraffin-embedded tissue and correlated with outcome. Tumors were classified as p53-Negative (p53-N) when no positivity was observed or only few cells showed weak positivity (0/1+) and p53-Positive (p53-P) when there was a diffuse and strong nuclear positivity (2+/3+).\u0000\u0000\u0000RESULTS\u0000p53-P was detected in 13 out of 97 tumors and was associated with disease relapse (39 vs.17%; P = 0.06) but not with anaplasia. Among p53-N patients only 5% had metastatic disease compared with 31% of the p53-P sample. (P = 0.038). Overall survival was 94% for patients with p53-N vs. 85% for patients with p53-P at 1 year (P = 0.34).\u0000\u0000\u0000CONCLUSIONS\u0000p53 expression in Wilms tumor detected by immunohistochemistry seems to be associated with advanced disease and relapse.","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"37 5 1","pages":"455-8"},"PeriodicalIF":0.0,"publicationDate":"2001-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87847733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
G. Chan, Y. Leung, M. M. Shing, C. Luk, S. Ling, A. Lee
{"title":"Does a \"false negative\" MIBG scan predict a better outcome in neuroblastoma patients?","authors":"G. Chan, Y. Leung, M. M. Shing, C. Luk, S. Ling, A. Lee","doi":"10.1002/MPO.1190","DOIUrl":"https://doi.org/10.1002/MPO.1190","url":null,"abstract":"","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"2003 1","pages":"155"},"PeriodicalIF":0.0,"publicationDate":"2001-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88914970","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Repeated achilles tendinitis after high dose methotrexate.","authors":"E. Toverud, S. Landaas, M. Hellebostad","doi":"10.1002/MPO.1191","DOIUrl":"https://doi.org/10.1002/MPO.1191","url":null,"abstract":"","PeriodicalId":18531,"journal":{"name":"Medical and pediatric oncology","volume":"8 1","pages":"156"},"PeriodicalIF":0.0,"publicationDate":"2001-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79980696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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