title: Mitotane treatment for adrenocortical carcinoma: an overview.
abstract: Adrenocortical carcinoma (ACC) is a rare aggressive endocrine neoplasm characterized by a 5-year survival of less than 50%. Due to the widespread use of imaging techniques in clinics, ACC is increasingly recognized as an incidentally discovered tumor. Mostly characterized by poor prognosis, ACC is often diagnosed at an advanced stage of disease. Early diagnosis is uncommon; when diagnosed, ACCs are usually large and have invaded adjacent organs, even if metastatic spread to distant sites can be absent. Complete surgical resection is the only potentially curative treatment for patients with localized disease; however, due to a recurrence rate of 50-70% after apparent radical surgery, there is a strong rationale for a concomitant systemic treatment. Adrenolytic therapy with mitotane (o,p›-DDD), administered alone or in combination with others antineoplastic agents, is the primary treatment for patients with advanced ACC and is increasingly used also in an adjuvant setting, even if controversy exists on this issue due to the limitations of the available literature. Despite being in use for many years, the rarity of ACC precluded the organization of randomized trials; thus, many areas of uncertainty and controversy remain regarding the role of this old drug in the clinical management of patients with ACC. The purpose of this paper is to review the current evidence on mitotane treatment in patients with advanced disease and in ACC patients after complete surgical resection as adjuvant treatment.
authors.affiliation: Department of Clinical and Biological Sciences, S. Luigi Hospital, University of Turin, Italy. silvia.defrancia@unito.it
authors.forename: S
authors.initials: S
authors.lastname: De Francia
authors.forename: A
authors.initials: A
authors.lastname: Ardito
authors.forename: F
authors.initials: F
authors.lastname: Daffara
authors.forename: B
authors.initials: B
authors.lastname: Zaggia
authors.forename: A
authors.initials: A
authors.lastname: Germano
authors.forename: A
authors.initials: A
authors.lastname: Berruti
authors.forename: F
authors.initials: F
authors.lastname: Di Carlo
mesh_terms: D018435:ATP Binding Cassette Transporter, Subfamily B; D020168:ATP Binding Cassette Transporter, Subfamily B, Member 1 / Q000037:antagonists & inhibitors; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000601:surgery; D000309:Adrenal Insufficiency / Q000139:chemically induced; D000315:Adrenalectomy; D018931:Antineoplastic Agents, Hormonal / Q000009:adverse effects / Q000737:chemistry / Q000493:pharmacokinetics / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001711:Biotransformation; D002277:Carcinoma / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D002986:Clinical Trials as Topic; D057210:Delayed Diagnosis; D019008:Drug Resistance, Neoplasm / Q000187:drug effects; D005260:Female; D005767:Gastrointestinal Diseases / Q000139:chemically induced; D006801:Humans; D033162:Incidental Findings; D008297:Male; D008939:Mitotane / Q000008:administration & dosage / Q000009:adverse effects / Q000737:chemistry / Q000493:pharmacokinetics / Q000627:therapeutic use*; D015394:Molecular Structure; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors; D011355:Prodrugs / Q000493:pharmacokinetics / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: C513055:ABCB1 protein, human; D018435:ATP Binding Cassette Transporter, Subfamily B; D020168:ATP Binding Cassette Transporter, Subfamily B, Member 1; D018931:Antineoplastic Agents, Hormonal; D009363:Neoplasm Proteins; D011355:Prodrugs; D008939:Mitotane