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Evaluation, Staging, and Surgical Management for Adrenocortical Carcinoma: An Update from the SSO Endocrine and Head and Neck Disease Site Working Group

  • Endocrine Tumors
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

This is the first of a two-part review on adrenocortical carcinoma (ACC), a rare and aggressive malignancy that often presents at an advanced stage. Most patients present with symptoms related to cortisol and/or androgen excess. Appropriate biochemical evaluation and imaging is important in assessing the extent of disease, operative planning, and oncologic surveillance for patients with ACC. For patients with locoregional disease, potential cure requires margin-negative resection, and accumulating evidence suggests that regional lymphadenectomy should be performed. Although laparoscopic adrenalectomy is reported by some to be adequate for localized ACC, open resection in the hands of an experienced adrenal surgeon is the gold standard for operative management of this disease. Cure is rare following disease relapse, however select patients with severe symptoms related to hormone excess or pain may benefit from resection of local or distant recurrence. For best oncologic outcomes, it is recommended that all patients with ACC be treated at centers with multidisciplinary expertise in management of this rare and aggressive malignancy.

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Authors and Affiliations

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

  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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Dickson, P.V., Kim, L., Yen, T.W.F. et al. Evaluation, Staging, and Surgical Management for Adrenocortical Carcinoma: An Update from the SSO Endocrine and Head and Neck Disease Site Working Group. Ann Surg Oncol 25, 3460–3468 (2018). https://doi.org/10.1245/s10434-018-6749-2

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