Adrenocortical carcinoma natural history
|
Adrenocortical carcinoma Microchapters |
|
Differentiating Adrenocortical carcinoma from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Study |
|
Adrenocortical carcinoma natural history On the Web |
|
American Roentgen Ray Society Images of Adrenocortical carcinoma natural history |
|
Risk calculators and risk factors for Adrenocortical carcinoma natural history |
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
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.
Natural history
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 facial feature.
Without treatment, the patient will develop symptoms of which will eventually lead to cardiovascular complications such as hyperglycemia, Diabetes mellitus, metastasis to
Complications
The following are the complications of Adrenocortical carcinoma:
- Cushing's syndrome-related complications
- Conn's syndrome
- Local and distant metastasis
- Invasion of adjacent organs or venous extension into the renal vein and inferior vena cava may be present.[2]
- Inferior vena cava invasion has been reported in 9% to 19% of cases at presentation.[3]
- 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
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[2]
- Stage of the tumor[3]
- Mitotic activity[4]
- 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)
- Survival ranges from a few months to several years[5]
References
- ↑ 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.
- ↑ Libé R, Borget I, Ronchi CL, Zaggia B, Kroiss M, Kerkhofs T; et al. (2015). "Prognostic factors in stage III-IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study". Ann Oncol. 26 (10): 2119–25. doi:10.1093/annonc/mdv329. PMID 26392430.
- ↑ 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. doi:10.1016/j.surg.2007.09.006. PMID 18063070.
- ↑ 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. doi:10.1007/s00423-010-0698-y. PMID 20694732.
- ↑ Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR (2008). "Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma". Eur J Endocrinol. 158 (6): 911–9. doi:10.1530/EJE-07-0723. PMID 18505909.