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Primary Culture of Rat Adrenocortical Cells and Assays of Steroidogenic Functions
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Adrenocortical Carcinoma with Hypercortisolism.

Soraya Puglisi1, Paola Perotti1, Anna Pia1

  • 1Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, Regione Gonzole 10, Orbassano 10043, Italy.

Endocrinology and Metabolism Clinics of North America
|May 15, 2018
PubMed
Summary

Adrenocortical carcinoma (ACC), a rare cancer, is often linked to hormone excess. Cortisol excess negatively impacts prognosis, making surgery and mitotane crucial treatments for managing this aggressive tumor.

Keywords:
Adrenocortical carcinomaCortisolCushing’s syndromeMitotane

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Area of Science:

  • Endocrinology
  • Oncology

Background:

  • Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy.
  • ACC frequently presents with hormone excess syndromes, notably Cushing's syndrome and androgen hypersecretion.
  • Elevated cortisol levels are increasingly recognized as a detrimental prognostic factor in ACC.

Purpose of the Study:

  • To review the management of adrenocortical carcinoma, focusing on hypercortisolism.
  • To highlight the role of surgery and medical therapies in treating ACC with excess cortisol.

Main Methods:

  • Literature review of current treatment strategies for adrenocortical carcinoma.
  • Analysis of the impact of cortisol excess on ACC prognosis and treatment efficacy.

Main Results:

  • Radical surgery is the primary curative treatment for ACC when feasible, especially in cases with hypercortisolism.
  • Mitotane is the established first-line medical therapy for ACC, used postoperatively and for advanced/metastatic disease.
  • Combination therapy with agents like metyrapone can accelerate cortisol control due to mitotane's slow action.

Conclusions:

  • Effective management of hypercortisolism is critical for improving outcomes in adrenocortical carcinoma.
  • A multimodal approach combining surgery, mitotane, and adjunctive therapies offers the best strategy for ACC.
  • Addressing cortisol excess is a key component in the treatment of this rare endocrine tumor.