Primary Adrenocortical Carcinoma and Ganglioneuroblastoma Followed by Rhabdomyosarcoma in a Child: A Case Report

We report a case of a young child affected by triple cancerswho developed a pleomorphic rhabdomyosarcoma of the retroperitoneumfollowing prophylactic chemotherapy after surgery for doubleprimary adrenocortical carcinoma and ganglioneuroblastoma. It is suggested that genetic susceptibility and chemotherapymight be responsible for the development of rhabdomyosarcomaas the second malignant neoplasm.     

Four Cases of T-Cell Malignancy in Childhood

Four cases of T-cell malignancy in childhood are reported. Inthe two older boys (seven [Case 1] and eight [Case 2] yearsold) the disease began as leukemia without a clinically detectablemediastinal mass. However, thymic involvement was found at autopsyin Case 1. Tumor cells of both patients had a rosetteformingcapacity with sheep erythrocytes (ERFC), high terminal deoxynucleotidyltransferase (TdT) activity and a positive acid phosphatase (AcPase)reaction. These findings suggest that the tumor cells of Cases1 and 2 originated from thymic T lymphocytes. The two younger patients (two [Case 3] and three [Case 4] yearsold) had cervical lymphadenopathy with mediastinal mass at onset,followed by leukemic change. The youngest patient (Case 3) lackedERFC and the AcPase reaction, but had C3 Receptor (C3-R), humanT-lymphocyte antigen (HLTA) and TdT activity. These findingsindicate that the tumor cells of Case 3 are compatible withearly thymic T lymphocytes. Tumor cells of Case 4 had ERFC,C3-R, HTLA, and slightly increased TdT activity. These findingssuggest that the tumor cells of Case 4 originated from thymicT lymphocytes. Only one patient has maintained an initial complete remissionfor more than one year. The three others relapsed or had a leukemicconversion within 12 months of the initial remission and twodied within 13 months. This indicates that childhood T-cellmalignancies have a poor prognosis.     

Comprehensive treatment of advanced neuroblastoma involving autologous bone marrow transplant

Encouraging results are reported with high-dose chemotherapy and total body irradiation followed by autologous bone marrow transplantation in the treatment of advanced neuroblastoma. However, relapse remains a significant problem. We used high-dose chemotherapy, surgery, intraoperative radiation and an autologous bone marrow transplant treated in vitro to remove tumor cells followed by 13-cis-retinoic acid to treat 36 children with advanced neuroblastoma. This comprehensive treatment appears to improve the survival rate of patients with advanced neuroblastoma, including those with N-myc amplification and bony involvement. The disease-free survival rate was 66% (95% confidence interval, 49–84%) at 3 years. All patients who received 13-cis-retinoic acid developed cheilitis, but no bone marrow depression occurred in these patients. Five patients developed hemolytic uremic syndrome (HUS) post-transplant. This may have been related to the procedure used for total body irradiation. Patients who had their kidneys shielded during this procedure did not develop this syndrome. Patients who received local irradiation at the primary site showed no evidence of relapse in this region, indicating that such therapy may help to prevent a relapse. These data suggest a high rate of 3 year disease-free survival with this treatment strategy. The nonrandomized nature of the study and use of multiple modalities precludes analysis of the specific contribution of each.