Current and emerging therapies for adrenocortical carcinoma--review.
Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Patients may present with hormone excess or a local mass effect. The most common imaging techniques (CT and MRI) use both size and appearance to distinguish between benign and malignant tumors. Open surgery by an expert surgeon with R0 target is the treatment of choice. Mitotane (alone or in combination with cytotoxic drugs) may be administered after surgery or in patients not amenable to surgery. The role of radiotherapy as an adjuvant treatment is uncertain whereas targeted radionuclide therapy seems to be a promising option. New adjuvant treatment options, even after complete tumor removal, are desired because postoperative disease-free survival at 5 yrs is only around 30%. The establishment of detailed guidelines with the purpose of optimizing therapy with only mitotane but also in combination with other antineoplasmatic drugs is still a task to be done. Future advances in the management of ACC will probably be connected with better understanding of the molecular pathogenesis.
Department of Endocrinology, Diabetology and Isotope Therapy, Wroclaw Medical University, Poland.
Justyna
J
Przytulska
Department of Endocrinology, Diabetology and Isotope Therapy, Wroclaw Medical University, Poland.
Natalia
N
Rogala
Department of Endocrinology, Diabetology and Isotope Therapy, Wroclaw Medical University, Poland.
Grażyna
G
Bednarek-Tupikowska
D000306:Adrenal Cortex Neoplasms / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018268:Adrenocortical Carcinoma / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D000818:Animals; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D015972:Gene Expression Regulation, Neoplastic; D020022:Genetic Predisposition to Disease; D006801:Humans; D009367:Neoplasm Staging; D010641:Phenotype; D011237:Predictive Value of Tests; D015398:Signal Transduction; D016896:Treatment Outcome
D014408:Biomarkers, Tumor