ABSTRACT
<p><b>OBJECTIVE</b>To investigate the efficacy of high dose chemotherapy combined with autologous peripheral blood stem cell transplantation (APBSCT) for the treatment of neural ectodermal solid tumor originated from neural crest in children.</p><p><b>METHODS</b>Twenty-three children at a medium age of 5.8 + or - 3.5 years with neural ectodermal solid tumor originated from neural crest were enrolled. Of the 23 children, 20 with stage IV neuroblastoma (9 were in complete remission, 7 were in partial remission and 4 were in progressive disease), 2 with stage IV primitive neuroectodermal tumor (PNET) in complete remission, and 1 with retinoblastoma in partial remission. Before APBSCT the children received 8.0 + or - 4.3 courses of chemotherapy. During chemotherapy the autologous peripheral blood stem cells were harvested and the tumor excision was performed. Then APBSCT was performed.</p><p><b>RESULTS</b>The reconstruction of the hematopoietic system was noted in 19 of 20 children with stage IV neuroblastoma 16.5 + or - 0.9 days after transplantation. A follow-up (median 15.8 months) was done in these children. The follow-up showed that the survival rate in children in complete remission before transplantation was 100%, 57% in those in partial remission, and none of children in progressive disease survived (P<0.05). The total survival rate was 67% in children with neuroblastoma. The child with retinoblastoma had complete remission in a 6-months follow-up. The tumors recurred in children with PNET 5 to 8 months after transplantation and all died within one year after transplantation.</p><p><b>CONCLUSIONS</b>High dose chemotherapy combined with APBSCT can result in a good outcome in children with neural ectodermal solid tumor originated from neural crest in complete remission before transplantation and can improve the outcome in patients in partial remission before transplantation. However, the children with PNET, even in complete remission before transplantation, do not respond to the therapy.</p>
Subject(s)
Female , Humans , Male , Antigens, CD34 , Antineoplastic Agents , Therapeutic Uses , Combined Modality Therapy , Follow-Up Studies , Neural Crest , Pathology , Neuroblastoma , Therapeutics , Neuroectodermal Tumors, Primitive , Therapeutics , Peripheral Blood Stem Cell Transplantation , Transplantation, AutologousABSTRACT
Objective To explore the value of seeking tumor cells through cerebrospinal fluid in the metastasis and prognosis of retinoblastoma.Methods Two hundred and fifty-six cases clinic diagnosed retinoblastoma were collected,154 cases were boys and 102 cases were girls.There were 85 children with bilateral disease and 171 children with unilateral disease.Lumbar puncture was examined before treatment,compare the result and prognosis.Children who were found tumor cells in their cerebrospinal fluid were treated with 6 to 9 cycles of systemic chemotherapy with CTV(carboplatin,teniposide and vincristine)protocol and 8 to 10 intrathecal injections with cytrarabine,methotrexate and dexametha-sone.Follow-up were between 8 to 23 months,the mean time was 14.6 months.Results There were 8 cases found tumor cells in their cerebrospinal fluid in all 256 children,one of them died of wide spread intracranial metastasis;the other patient was given high dose chemotherapy with autologous perip-heral stem cell transplantation(APBSCT) because of intracranial metastasis,follow-up to now the patient′s condition was stable.The rest 6 children were enucleated in follow-up period,there were 4 cases(67%) with the histopathology of the eye indicates spread of tumour cells beyond the lamina cribrosa,and 2 cases with invading the cut end of the optic nerve.Six children were no metabasis evidences at other tissues.Conclusions Intracranial metastasis is the common metastasis style in retinoblastoma,so lumbar puncture to seeking tumor cells was important to evaluate intracranial metastasis,early treatment and prognosis.