Pediatric adrenocortical carcinoma. [PDF]
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy of the adrenal gland with an unfavorable prognosis. It is rare in the pediatric population, with an incidence of 0.2-0.3 patients per million in patients under 20 years old. It is primarily associated with Li-Fraumeni and Beckwith-Wiedemann tumor predisposition syndromes in children.
Ilanchezhian M +7 more
europepmc +4 more sources
Radiotherapy for pediatric adrenocortical carcinoma - Review of the literature. [PDF]
Pediatric adrenocortical carcinoma (pACC) is a rare disease with poor prognosis. Publications on radiotherapy (RT) are scarce. This review summarizes the current data on RT for pACC and possibly provides first evidence to justify its use in this setting.We searched the PubMed and Embase database for manuscripts regarding RT for pACC.We included 17 ...
Wiegering V +12 more
europepmc +7 more sources
Pediatric adrenocortical carcinoma: clinical features and application of neoadjuvant chemotherapy. [PDF]
Abstract Objective To summarize the clinical characteristics of children with adrenocortical carcinoma (ACC) and preliminarily explore the indications for and efficacy of neoadjuvant chemotherapy in certain patients. Methods The data of 49 children with adrenocortical tumors
Lin Y +17 more
europepmc +4 more sources
Inflammation-based score in pediatric adrenocortical carcinoma. [PDF]
Inflammation-based scores have been demonstrated to be independent prognostic factors in predicting outcomes in adult adrenocortical carcinoma (ACC). We aimed to investigate the prognostic role of these scores in pediatric adrenocortical carcinoma (pACC) patients.
Riedmeier M +28 more
europepmc +4 more sources
Network analysis reveals potential markers for pediatric adrenocortical carcinoma. [PDF]
Pediatric adrenocortical carcinoma (ACC) is a rare malignancy with a poor outcome. Molecular mechanisms of pediatric ACC oncogenesis and advancement are not well understood. Accurate and timely diagnosis of the disease requires identification of new markers for pediatric ACC.
Kulshrestha A, Suman S, Ranjan R.
europepmc +5 more sources
Pediatric adrenocortical carcinoma complicated by uric acid nephrolithiasis: a unique case report. [PDF]
Adrenocortical carcinoma (ACC) is an exceedingly rare malignancy in children, typically presenting with Cushing's syndrome due to excessive hormone secretion. The occurrence of uric acid nephrolithiasis in pediatric ACC patients is exceptionally uncommon.We report the case of a 5-year-old boy who exhibited symptoms of Cushing's syndrome over a period ...
Zhang M, Guo W, Li H, Xi X.
europepmc +4 more sources
Pediatric Adrenocortical Carcinoma: The Nuts and Bolts of Diagnosis and Treatment and Avenues for Future Discovery. [PDF]
Adrenocortical tumors (ACTs) are infrequent neoplasms in children and adolescents and are typically associated with clinical symptoms reflective of androgen overproduction. Pediatric ACTs typically occur in the context of a germline TP53 mutation, can be cured when diagnosed at an early stage, but are difficult to treat when advanced or associated with
O'Neill AF +7 more
europepmc +5 more sources
Treatment of Pediatric Adrenocortical Carcinoma With Surgery, Retroperitoneal Lymph Node Dissection, and Chemotherapy: The Children's Oncology Group ARAR0332 Protocol. [PDF]
PURPOSE Adrenocortical carcinoma (ACC) is a rare aggressive pediatric malignancy with distinct biology. Its treatment follows the principles developed for adults; pediatric-specific studies are scarce. PATIENTS AND METHODS Prospective single-arm risk-stratified interventional study.
Rodriguez-Galindo C +17 more
europepmc +3 more sources
Factors associated with survival in pediatric adrenocortical carcinoma: An analysis of the National Cancer Data Base (NCDB). [PDF]
Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival.The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011.
Gulack BC +7 more
europepmc +4 more sources
Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: a children's oncology group study. [PDF]
Purpose Adrenocortical carcinoma (ACC) is a rare pediatric malignancy. It occurs in excess among individuals with the Li-Fraumeni syndrome, which results primarily from germline mutations in the TP53 gene. Prior series exploring frequencies of germline TP53 mutation among children with ACC have been small, geographically limited, or subject to referral
Wasserman JD +6 more
europepmc +4 more sources

