The History of Adrenocortical Carcinoma Treatment – A Medical Perspective
Knowledge of the genetic and molecular alterations in adrenocortical carcinoma (ACC) has advanced in the past two decades, as a result of newer laboratory methodology to study mechanisms of oncogenesis and tumor pathophysiology. In contrast, limited progress has been made in our ability to treat and prolong survival in patients with advanced disease. Over the past five decades, a number of reports have summarized the experience of individual medical institutions with ACC; and collectively, based on several thousand cases, there is relative consensus on the epidemiology of ACC, its clinical presentation, criteria for pathological diagnosis, and tumor response to standard cytotoxic chemotherapy. Unfortunately, survival of patients with advanced disease remains poor (Fig. 1.1), and targeted therapies based on new knowledge of the biology of these tumors are only in clinical trial phase. This introduction will attempt to highlight the early experience with mitotane that forms the basis of our current approach to the management of patients with ACC.
KeywordsCongenital Adrenal Hyperplasia Adrenocortical Carcinoma Clinical Trial Phase Relative Consensus Adrenocortical Hyperplasia
- 2.Soffer LJ et al (1961) The human adrenal gland. Lea & Febiger, Philadelphia, PAGoogle Scholar
- 3.Cecil HF (1933) Hypertension, obesity, virilism and pseudohermaphroditism as caused by suprarenal tumors. J.A.M.A 100:463Google Scholar
- 4.Eisenstein AB (1967) The adrenal cortex. Little, Brown and Company, Boston, MAGoogle Scholar
- 6.Thompson NW (1983) Adrenocortical carcinoma. In: Thompson NW, Vinik AI (eds) Endocrine surgery update. Grune and Stratton, New York, pp 119–128Google Scholar