Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
JCR Impact Factor (IF) – 2.1 (5-Year IF – 2.0)
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Index Copernicus  – 171.00; MNiSW – 70 pts

ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
Periodicity – monthly

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Advances in Clinical and Experimental Medicine

2015, vol. 24, nr 2, March-April, p. 185–193

doi: 10.17219/acem/30645

Publication type: editorial

Language: English

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Current and Emerging Therapies for Adrenocortical Carcinoma – Review

Justyna Przytulska1,B,C,D,E, Natalia Rogala1,B,C,D, Grażyna Bednarek-Tupikowska1,A,E,F

1 Department of Endocrinology, Diabetology and Isotope Therapy, Wroclaw Medical University, Poland

Abstract

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.

Key words

molecular genetics, adrenocortical carcinoma, mitotane, treatment options.

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