Adrenocortical carcinoma natural history
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If left untreated, patients with adrenocortical carcinoma may progress to develop hyperglycemia, osteoporosis, delayed wound healing, hypertension, Cerebrovascular disease, and local or distant metastasis. Prognosis is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma stage I-III is approximately 30%. Complications may include metastasis, Conn's syndrome and Cushing'a syndrome.
The symptoms of adrenocortical carcinoma usually develop in the fifth to sixth decade of life and start with symptoms of Cushing's syndrome such as weight gain, acne, irritability, insomnia, symptoms of androgen excess such as virilization, deepening of the voice, and coarsening of the facial feature.
The following are the complications of Adrenocortical carcinoma:
- Cushing's syndrome-related complications
- Conn's syndrome
- Local and distant metastasis
- Paraneoplastic syndrome
- A set of signs and symptoms that is the consequence of cancer in the body, but unlike mass effect, is not due to the local presence of cancer cells.
- In contrast, these phenomena are mediated by humoral factors (such as hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.
- Tumor thrombus formation
- Adrenocortical carcinoma, generally, carries a poor prognosis.
- The five-year disease-free survival rate for a complete resection of a stage I-III is approximately 30%.
- Survival ranges from a few months to several years
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
- Cortisol production as an adverse prognostic factor
- Ki-67/MIB1 labeling index (antigen identified by monoclonal antibody Ki-67)
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- Gonzalez RJ, Tamm EP, Ng C, Phan AT, Vassilopoulou-Sellin R, Perrier ND; et al. (2007). "Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma.". Surgery. 142 (6): 867–75; discussion 867–75. PMID 18063070. doi:10.1016/j.surg.2007.09.006.
- Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM (2010). "Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade.". Langenbecks Arch Surg. 395 (7): 955–61. PMID 20694732. doi:10.1007/s00423-010-0698-y.