Survival and late effects of comprehensive treatment on children with neuroblastoma / 中华实用儿科临床杂志

Objective To investigate the organ function survival and late effects and secondary malignancy of childhood neuroblastoma (NB) with high intensity comprehensive treatment.Methods A total of 23 children with NB who received comprehensive treatment in Shanghai Children's Medical Center from December 1998 to October 2010 were enrolled.All tests were formulated according to the Children's Oncology Group (COG)'s guidelines with the approval of parents.The late side effects were graded by CTCAE v3.0,and hearing loss related to platinum was graded by Brock and Chang.Results The median follow-up was 96 (65-170) months.All the patients had at least one late side effect.The occurrence rate of 1-2 adverse effects was 100％,and 17.4％ (4/23 cases) of patients had grade 3-4 adverse effects.90.5％ of children (19/21 cases)developed hearing loss on both sides.Eleven children (52.4％,11/21 cases) developed dental abnormities,comprising microdontia,missing teeth,root stunting and enamel hypoplasia.9.5％ of children (2/12 cases) had scoliosis.42.8％ of children (9/12 cases) developed hypogonadism with the approval of parents or delayed growth.47.4％ of children (9/19 cases) had abnormal respiratory function.36.8％ of children (7/19 cases)had abnormal cardiac function.33.4％ of children (7/21 cases)developed renal damage.23.8 ％ of children (5/21 cases) had abnormal liver function.66.7％ of children (14/21 cases) had low level of cortisol,but adrenocorticotrophic hormone was normal.All children had normal function of thyroid.Three children developed secondary malignancy:leukemia,malignant fibroma and liver tumor.Conclusions The incidence of long-term adverse reactions of patients with neuroblastoma treated with high intensity comprehensive treatment is very high and the patients may have adverse effects,like hearing loss,dental abnormality,cardiopulmonary function abnormality,musculoskeletal problems and secondary malignancy.The upmost importance is to establish long-term follow-up program to detect the life quality of children and amend current therapeutic schemes improve life quality.

Report of 14 cases with pancreatoblastoma / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze clinical features, outcomes and correlative prognostic factors of the disease.</p><p><b>METHOD</b>Totally 14 newly diagnosed children with pancreatoblastoma at Shanghai Children's Medical Center, Children's Hospital of Chongqing Medical University, Sun Yat-sen University Cancer Center and Beijing Children's Hospital were enrolled into this study between April 2003 and July 2013. The clinical features, surgery, chemotherapy protocol and prognostic factors of patients were retrospectively analyzed.</p><p><b>RESULT</b>The median age at diagnosis was 6.5 years (0.6 to 11 years). Four cases had local tumor, 9 cases had local invasion, and in 1 case lung and liver metastasis was found at diagnosis. Increased serum α-fetoprotein were found in 8 cases (66-54,000 μg/L). In 8 patients the tumor was resected at first. The remaining 6 patients had biopsy and in four of them the tumor was completely resected after 4-6 cycles of chemotherapy. Except for 2 patients who gave up treatment, all the other patients fulfilled the comprehensive treatment including surgery and chemotherapy. One patient received radiofrequency ablation. The median follow-up period was 26 months(16-140 months). The estimated 2 year event free survival was (66.7 ± 13.6)%.On univariate analysis, non-resectable tumor(χ(2) = 6.663, P = 0.010), progression and metastases during treatment(χ(2) = 14.927, P = 0.000), poor response to treatment(χ(2) = 12.293, P = 0.000)were independent predictors for poor prognosis.</p><p><b>CONCLUSION</b>Complete resection and complete remission after treatment were excellent prognostic factors. Some initially unresectable disease seems to be resectable after adjuvant chemotherapy, which may be a key point of treatment strategy.</p>

Long-term follow-up of stage 1-2 neuroblastoma / 临床儿科杂志

Objectives To evaluate the clinical features, treatment scheme and long-term outcomes of stage 1、2 childhood neuroblastoma (NB). Methods The retrospective study included 49 newly diagnosed NB stage 1、2 patients from June 1998 to December 2010. Clinical data and long-term outcomes were analyzed. Results Twenty-four patients with stage 1 NB and twenty patients with stage 2 NB were found among all 237 patients with NB enrolled in this study. The median age at diagnosis was 25 months( 2 week to 9 year old),29 males and 20 females. Thirty-one patients (63.6%) without symptoms were discovered with tumor by physical or imaging examination. Thorax and abdomen were the most common sites of primary tumor (21 and 22 cases, accounting for 42.9% and 44.9% of all patients, respectively). Forty (81.6%) NB patients had favorable pathology classification. One patient was of MYCN amplification status. Urine vanilla mandelic acid was normal in 32 (91.4%) patients, and serum lactate dehydrogenase was less than five times of the normal value in all patients. Ten NB patients were treated ac-cording to the low-risk protocol who received surgery alone.Thirty-nine patients were treated according to intermediate-risk protocol who received both surgery and chemotherapy. All the patients achieved very good partial remission (100%).The medi-an follow-up period was 60 months(22 months to148months). Nine patients were lost after a follow up of 3 months in medi-an. The 2-、3-、5-year event free survival and overall survial of all 49 patients was 100%. Conclusions The prognosis for neu-roblastoma of stage 1、2 in this study was with 100%survival, which provides opportunity for further reduction of dosage and/or duration of episodes in chemotherapy.