Subject(s)
Chromosome Aberrations , Chromosomes, Human, Pair 12 , Chromosomes, Human, Pair 15 , DNA-Binding Proteins/genetics , Leukemia, Myeloid, Acute/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Receptor, trkC/genetics , Repressor Proteins/genetics , Child , Child, Preschool , Female , Gene Expression , Humans , Male , Oncogene Proteins, Fusion/genetics , Proto-Oncogene Proteins c-ets , RNA/analysis , Reverse Transcriptase Polymerase Chain Reaction , Sensitivity and Specificity , ETS Translocation Variant 6 ProteinABSTRACT
BACKGROUND: A relationship between young age and increased risk of recurrence of pediatric differentiated thyroid carcinoma has been suggested; however, no attempts have been made to assess the prognostic factors or efficacy of treatment in very young children with this malignancy. The objectives of this study were to evaluate the association of age with outcome in pediatric differentiated thyroid carcinoma and to compare the clinical, pathologic, prognostic, and treatment variables between younger and older children with this disease. PROCEDURE: A retrospective review of all patients presenting to the British Columbia's Children's Hospital or British Columbia Cancer Agency <17 years of age at diagnosis with differentiated thyroid carcinoma between January, 1955, and December, 1996, was completed. RESULTS: Thirty-eight patients were identified, 12 of whom were 10 years. An association between young age and extrathyroidal tumor invasion was identified (P = 0.016); however, the latter factor did not independently predict outcome. There was a trend for suppressive doses of thyroid hormone to improve outcome, particularly with increasing age at diagnosis, but this was not statistically significant. CONCLUSIONS: Age is the major determinant of recurrence in pediatric differentiated thyroid carcinoma. The results suggest different tumor biology in young children requiring novel approaches to therapy to decrease recurrence rates.
Subject(s)
Adenocarcinoma, Follicular/mortality , Adenocarcinoma, Follicular/secondary , Carcinoma, Papillary/mortality , Carcinoma, Papillary/secondary , Thyroid Neoplasms/mortality , Thyroid Neoplasms/pathology , Adolescent , Age Factors , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Lung Neoplasms/secondary , Lymphatic Metastasis , Male , Medical Records , Multivariate Analysis , Neoplasm Recurrence, Local/mortality , Neoplasm Recurrence, Local/pathology , Registries , Retrospective Studies , Survival Analysis , Time FactorsABSTRACT
A girl with Diamond-Blackfan anemia diagnosed in infancy started cyclosporine A (CSA) therapy at 9 years and 8 months of age after experiencing unacceptable side effects while receiving prednisone. Since then, she has been followed-up for more than 4 years. She exhibited a dramatic response to CSA, with weaning and then cessation of steroid therapy after 5 months. She has remained transfusion-independent. Attempts to discontinue CSA therapy have been unsuccessful. Relapse of the anemia has occurred in the context of viral infections with missed CSA doses. The major clinical problem during treatment has been recurrent oral aphthous ulceration, which responds to topical therapy. She is currently maintained on CSA 100 mg twice daily with a hemoglobin of 10.2 g/dL and a reticulocyte count of 1.6%. A trial of CSA therapy should be considered in patients with Diamond-Blackfan anemia in whom steroid therapy has failed before a transfusion program is instituted or alternative donor stem cell transplantation is entertained.
