Our knowledge about inherited susceptibility to adrenocortical carcinoma (ACC) almost
exclusively stems from experiences with familial cancer susceptibility syndromes, which are …

Adrenocortical carcinoma (ACC), particularly when occurring during childhood, has been a
traditional component of the tumor spectrum of Li-Fraumeni syndrome. Recent research has …

Adrenocortical malignancies can occur in the context of several tumor predisposition
syndromes. The Carney complex (CNC) is responsible for the majority of primary pigmented …

Adrenocortical carcinoma (ACC) affects children and adults. Roughly 50% of very early
onset ACCs occur in children with germline TP53 mutations. Several recent studies have …

The incidence of adrenocortical tumors (ACTs) is increased in several familial cancer
syndromes resulting from abnormalities in genes that encode transcription factors implicated …

Endocrine tumor syndromes, eg, multiple endocrine neoplasia types 1 and 2, were among
the first recognized hereditary predisposition syndromes to tumor development. Over time …

Advances in genomics over the past two decades have allowed for elucidation of the genetic
alterations leading to the development of adrenocortical tumors and/or hyperplasias. These …

Context: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor
prognosis. Although the majority of childhood ACC arises in the context of inherited cancer …

Purpose Adrenocortical carcinoma (ACC) is an endocrine malignancy with a poor
prognosis. The association of adult-onset ACC with inherited cancer predisposition …

Adrenal tumors are common, with an estimated incidence of 7.3% in autopsy cases, while
adrenocortical carcinomas (ACCs) are rare, with an estimated prevalence of 4–12 per …