Association of image-defined risk factors,tumor resectability,and prognosis in children with localized neuroblastoma

Background Although localized neuroblastoma has a good prognosis,some cases have undergone treatment failure or recur-rence.Apart from biologic features such as MYCN status,we wondered whether some characteristics of growing tumors are prognostic,such as a well-encapsulated mass without infiltration of vital organs.We analyzed the diagnostic utility of image-defined risk factors(IDRFs)to predict successful treatment and prognosis.The overall goal was to achieve maximum cure rates for patients with localized neuroblastoma through a better understanding of clinical characteristics.Methods We retrospectively reviewed the images of patients with localized neuroblastoma who were enrolled between June 1998 and December 2012 at a single institution in Shanghai,China.Unequivocal categorization regarding IDRFs was available in 67 patients.IDRF was assessed at diagnosis and after four cycles of neoadjuvant chemotherapy,on average.The median follow-up period was 84 months(range: 48-132 months)after diagnosis.Results MRI and CT indicated a total of 177 IDRFs in these 67 patients.Logistic regression analysis revealed a highly significant negative correlation between the numbers of IDRFs and the possibility of complete removal of neuroblastoma.Intraspinal extension of the tumor,compression of the trachea,and encasement of the main artery in localized neuroblastoma were predictors for incomplete tumor resection.According to univariate analysis,≥4 IDRFs and intraspinal extension of the tumor were significant indicators of poor prognosis.Conclusions The number of IDRFs was useful in predicting surgical outcome and event-free survival.The number of IDRFs should be considered in protocol planning,instead of IDRF presence or absence.

Homoharringtonine is a safe and effective substitute for anthracyclines in children younger than 2 years old with acute myeloid leukemia

Homoharringtonine (HHT),a plant alkaloid from Cephalotaxus harringtonia,exhibits a unique anticancer mechanism and has been widely used in China to treat patients with acute myeloid leukemia (AML) since the 1970s.Trial SCMC-AML-2009 presented herein was a randomized clinical study designed based on our previous findings that pediatric AML patients younger than two years old may benefit from HHT-containing chemotherapy regimens.Patients randomized to arm A were treated with a standard chemotherapy regimen comprising mainly of anthracyclines and cytarabine (Ara-C),whereas patients in arm B were treated with HHTcontaining regimens in which anthracyclines in all but the initial induction therapy were replaced by HHT.From February 2009 to November 2015,59 patients less than 2 years old with de novo AML (other than acute promyelocytic leukemia) were recruited.A total of 42 patients achieved a morphologic complete remission (CR) after the first course,with similar rates in both arms (70.6% vs.72.0%).At the end of the follow-up period,40 patients remained in CR and 5 patients underwent hematopoietic stem cell transplantation in CR,which could not be considered as events but censors.The 5-year event-free survival (EFS) was 60.2%± 9.6% for arm A and 88.0%± 6.5% for arm B (P=0.024).Patients in arm B experienced shorter durations of leukopenia,neutropenia,and thrombocytopenia and had a lower risk of infection during consolidation chemotherapy with high-dosage Ara-C.Consequently,the homoharringtonine-based regimen achieved excellent EFS and alleviated hematologic toxicity for children aged younger than 2 years with de novo AML compared with the anthracycline-based regimen.

Early T-cell precursor leukemia:a subtype of high risk childhood acute lymphoblastic leukemia

Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia(T-ALL)and B-cell acute lymphoblastic leukemia(B-ALL).In children,T-ALL usually has a worse prognosis than B-ALL,although childhood T-ALL prognoses have improved remarkably.The varying outcomes among T-ALL cases suggest that an unrecognized biological heterogeneity may contribute to chemo-resistance.Deep exploration of T-lymphocyte development in recent years has found a subgroup of patients with a phenotype that resembles early T-cell precursor,which confers a much poorer prognosis than any other form of T-ALL.This novel subtype of T-ALL was called early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Flow cytometry data from T-ALL patients enrolled in Shanghai Children’s Medical Center between July 2002 and October 2010 were assessed according to Dr.Campana’s protocol.Among total 89 T-ALL cases,74 cases had enough immunophenotype data available to differentiate between ETP(CD1a^(-),CD8^(-),CD5^(dim),at least one marker of stem cell or myeloid lineage)and non-ETP.From these 74 subjects,12 ETP-ALL cases(16.2%)were identified.The event-free survival(EFS)rate at 66.8 months was 11.1%±10.1% for ETP-ALL and 57.6%±5.6% for non-ETP-ALL(P=0.003).The overall survival rates were 13.3%±11.0% for ETP-ALL and 64.7%±6.3% for non-ETP-ALL(P=0.002).Our findings demonstrate that early T-cell precursor leukemia is a very high-risk subtype of acute lymphoblastic leukemia with poor prognosis.

Renal tumor in developing countries:142 cases from a single institution at Shanghai,China

Background:The clinical management of children with renal tumors including Wilms'tumor,clear cell sarcoma,rhabdoid tumor and other renal tumors in our center was designed according to the National Wilms'Tumor Study Group protocols.Methods:A total of 142 consecutive patients who had been diagnosed as having renal tumors at Shanghai Children's Medical Center were reviewed retrospectively in the period of December 1998 and September 2012.Diagnosis and treatment were decided by a multi-disciplinary team including oncologists,surgeons,pathologists and sub-specialized radiologists.Results:The median age of the patients at the time of diagnosis was 27 months.The tumor stages of the patients were as follows:stage I 24.6%,stage II 23.2%,stage III 32.3%,stage IV 14.1%,and stage V 5.6%.Favorable histology was diagnosed in 80.3%,anaplasia in 4.2%,clear cell sarcoma in 9.8%,rhabdoid tumor in 4.9%,and other renal tumors in 0.7%of the patients.The event-free and overall 5-year survival rates were 80%and 83%,respectively.Tumor relapse and progress was seen in 25 patients(17.6%).The median relapse time was 6 months(range:2-37 months).Seven relapsing patients were re-treated and four of them got second complete remission(three in stage II,one in stage I).Conclusion:A multi-disciplinary team work model is feasible in developing countries,and the renal tumors protocols basically from developed countries are safe in developing countries.