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Like other solid malignancies of childhood, over half of ACCs present in the first 4 years of life in over 50% of cases. Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes. Despite the fact that the microscopic features are often quite atypical and identical in many respects to ACCs in adults, the clinical outcome is favorable in 70% or more of cases. Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g. A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.<\/jats:p>","DOI":"10.2350\/08-06-0489.1","type":"journal-article","created":{"date-parts":[[2009,3,30]],"date-time":"2009-03-30T08:35:42Z","timestamp":1238402142000},"page":"284-291","source":"Crossref","is-referenced-by-count":78,"title":["Adrenal Cortical Neoplasms in Children: Why So Many Carcinomas and Yet So Many Survivors?"],"prefix":"10.1177","volume":"12","author":[{"given":"Louis P.","family":"Dehner","sequence":"first","affiliation":[{"name":"Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Ashley","family":"Hill","sequence":"additional","affiliation":[{"name":"Lauren V. 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