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Adjuvant and Neoadjuvant Therapy, Treatment for Advanced Disease, and Genetic Considerations for Adrenocortical Carcinoma: An Update from the SSO Endocrine and Head and Neck Disease Site Working Group

  • Endocrine Tumors
  • Published:
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Abstract

This is the second of a two-part review on adrenocortical carcinoma (ACC) management. While margin-negative resection provides the only potential cure for ACC, recurrence rates remain high. Furthermore, many patients present with locally advanced, unresectable tumors and/or diffuse metastases. As a result, selecting patients for adjuvant therapy and understanding systemic therapy options for advanced ACC is important. Herein, we detail the current literature supporting the use of adjuvant mitotane therapy, consideration of adjuvant radiation therapy, and utility of cytotoxic chemotherapy in patients with advanced disease. Ongoing investigation into molecular targeted agents, immunotherapy, and inhibitors of steroidogenesis for the treatment of ACC are also highlighted. Lastly, the importance of genetic counseling in patients with ACC is addressed as up to 10% of patients will have an identifiable hereditary syndrome.

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Author information

Authors and Affiliations

  1. Division of Surgical Oncology, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, 38163, USA

    Paxton V. Dickson MD

  2. Division of Surgical Oncology and Endocrine Surgery, University of North Carolina, Chapel Hill, NC, USA

    Lawrence Kim MD

  3. Division of Surgical Oncology, Medical College of Wisconsin, Milwaukee, WI, USA

    Tina W. F. Yen MD, MS

  4. Department of Surgery, Division of Surgical Oncology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Anthony Yang MD

  5. University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Elizabeth G. Grubbs MD, MS

  6. Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD, USA

    Dhavel Patel MD

  7. Division of Surgical Oncology and Endocrine Surgery, Vanderbilt University, Nashville, TN, USA

    Carmen C. Solórzano MD

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  1. Paxton V. Dickson MD
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  2. Lawrence Kim MD
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  5. Elizabeth G. Grubbs MD, MS
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  7. Carmen C. Solórzano MD
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Correspondence to Paxton V. Dickson MD.

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Dickson, P.V., Kim, L., Yen, T.W.F. et al. Adjuvant and Neoadjuvant Therapy, Treatment for Advanced Disease, and Genetic Considerations for Adrenocortical Carcinoma: An Update from the SSO Endocrine and Head and Neck Disease Site Working Group. Ann Surg Oncol 25, 3453–3459 (2018). https://doi.org/10.1245/s10434-018-6750-9

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  • DOI: https://doi.org/10.1245/s10434-018-6750-9

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