Adrenocortical carcinoma natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Ahmad Al Maradni, M.D. [3]

Overview

Prognosis is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma stage I-III is approximately 30%. Complication may include metastasis, Conn syndrom and Cushing syndrom

Complications

The following are the complications of Adrenocortical carcinoma:

Prognosis

Adrenocortical carcinoma, generally, carries a poor prognosis.[1] The five-year disease-free survival rate for a complete resection of a stage I-III is approximately 30%.[1]

The most important prognostic factors are:

  • Age of the patient
  • Stage of the tumor
  • Mitotic activity
  • Venous invasion
  • Weight more than 50 Kg
  • Diameter more than 6.5 cm
  • Ki-67/MIB1 labeling index (antigen identified by monoclonal antibody Ki-67)
  • Survival ranges from a few months to several years. (242)
  • In the Michigan Endocrine Oncology Repository, roughly 5% of all patients diagnosed with ACC will have a disease course of _10 years (T.E., unpublished results). Although this may be caused by a referral bias, there is an emerging notion of an ACC population with exceptionally long survival. Despite these variations in survival, prognostic factors have not been definitively researched. Naturally, age at diagnosis is correlated with decreased overall survival (243). However, whether this is true for tumor-free survival remains unclear. Tumor characteristics of malignancy and velocity of tumor growth are usually related to a decreased survival. Tumor extent (eg, stage), specifically the presence of distant metastasis and number of organs involved in metastatic disease, confers a worse prognosis (243, 244). High tumor grade (_20 mitoses per HPF) is also an unfavorable prognostic indicator (245). Although older studies did not show any differences in prognoses for patients harboring different hormone secretion subtypes of ACC, some recent studies identified cortisol production as an adverse prognostic factor (12, 85, 244).

References

  1. 1.0 1.1 Allolio B, Fassnacht M (2006). "Clinical review: Adrenocortical carcinoma: clinical update". J Clin Endocrinol Metab. 91 (6): 2027–37. PMID 16551738. Free Full Text.

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