St. Jude Children's Research Hospital
This study aims to collect demographic and medical information including detailed family history of cancer of children and adolescents with adrenocortical tumors in order to learn more about the clinical and epidemiological aspects, treatment modalities, and outcome of patients with this rare disease, worldwide. In addition, investigators at St. Jude Children's Research Hospital (SJCRH) plan to perform molecular studies of tumor cells aimed to clarify the role of the TP53 gene and other genetic pathways in these tumors. They aim to obtain relevant biological material from participants with adrenocortical tumor (ACT), their biological parents, and relatives for determination of the TP53 germline status, molecular studies of the TP53 gene, and other molecular pathways.
Adrenocortical tumors (ACT) are rare cancer types that form in the outer layer of the adrenal gland and are very uncommon in children and teenagers. There is variation in pediatric ACT incidence worldwide. In the United States, only about 25 new cases of ACT per million per year, making this a very rare tumor. However, in southern Brazil, the annual incidence of ACT is 15 times that seen in the United States accounting for 3.4-4.2 per million per year. Molecular studies have revealed that the majority of children with ACT, particularly those younger than 4 years of age, have constitutional TP…
STRATIFICATION ASSIGNMENT: * Stratum A: participant suspected or confirmed diagnosis of adrenocortical tumor (ACT) * Stratum R: relative of participant with ACT and TP53 mutation who has diagnosis of malignancy * Stratum P: biological parent of participant with ACT Inclusion Criteria - Stratum A (participant with ACT): * Age ≤ 21 years old at diagnosis * Suspected or confirmed diagnosis of adrenocortical tumor (adenoma, carcinoma or undefined histology). * Signed informed consent Inclusion Criteria - Stratum R (relative): * Any age * Diagnosis of malignant tumor * Signed informed consent…