Adrenocortical carcinoma natural history: Difference between revisions
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{{CMG}} {{AE}} {{RT}} {{AAM}} | {{CMG}} {{AE}} {{RT}} {{AAM}} | ||
==Overview== | ==Overview== | ||
Prognosis is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma [[Cancer staging|stage]] I-III is approximately 30%. | If left untreated, patients with adrenocortical carcinoma may progress to develop h[[Hyperglycemia|yperglycemia]], o[[Osteoporosis|steoporosis,]] delayed [[wound healing|wound healing,]] h[[Hypertension|ypertension,]] [[Cerebrovascular disease]], and local or distant [[metastasis]]. [[Prognosis]] is generally poor, and the 5-year survival rate of patients with adrenocortical carcinoma [[Cancer staging|stage]] I-III is approximately 30%. Complications may include [[metastasis]], [[Conn's syndrome]] and [[Cushing's syndrome|Cushing'a syndrome]]. | ||
== Natural history == | == 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. | 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 h[[Hyperglycemia|yperglycemia]], [[Diabetes mellitus]], metastasis to | Without treatment, the patient will develop symptoms of which will eventually lead to cardiovascular complications such as h[[Hyperglycemia|yperglycemia]], [[Diabetes mellitus]], metastasis to | ||
==Complications== | ==Complications== | ||
The following are the complications of [[Adrenocortical carcinoma]]: | The following are the complications of [[Adrenocortical carcinoma]]: | ||
* [[Cushing's syndrome]]- | * [[Cushing's syndrome]]-related complications | ||
**[[Hyperglycemia]] | **[[Hyperglycemia]] | ||
**[[Diabetes mellitus]] | **[[Diabetes mellitus]] | ||
| Line 23: | Line 24: | ||
* [[Conn's syndrome]] | * [[Conn's syndrome]] | ||
* Local and distant [[metastasis]] | * Local and distant [[metastasis]] | ||
**Invasion of adjacent organs or venous extension into the renal vein and [[inferior vena cava]] may be present.<sup>[[Adrenocortical carcinoma pathophysiology#cite note-pmid7142516-2|[2]]]</sup> | **[[Invasion]] of adjacent organs or [[venous]] [[extension]] into the renal vein and [[inferior vena cava]] may be present.<sup>[[Adrenocortical carcinoma pathophysiology#cite note-pmid7142516-2|[2]]]</sup> | ||
**[[Inferior vena cava]] invasion has been reported in 9% to 19% of cases at presentation.<sup>[[Adrenocortical carcinoma pathophysiology#cite note-pmid21606258-3|[3]]]</sup> | **[[Inferior vena cava]] invasion has been reported in 9% to 19% of cases at presentation.<sup>[[Adrenocortical carcinoma pathophysiology#cite note-pmid21606258-3|[3]]]</sup> | ||
* [[Paraneoplastic syndrome]] | * [[Paraneoplastic syndrome]] | ||
**A set of signs and symptoms that is the consequence | **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 | **In contrast, these phenomena are mediated by humoral factors (such as [[Hormone|hormones]] or [[Cytokine|cytokines]]) excreted by [[Tumor cell|tumor cells]] or by an [[Immune system|immune response]] against the [[tumor]]. | ||
* [[Tumor]] [[thrombus]] formation | * [[Tumor]] [[thrombus]] formation | ||
==Prognosis== | ==Prognosis== | ||
Adrenocortical carcinoma, generally, carries a poor prognosis.<ref name="allolio">{{cite journal | author = Allolio B, Fassnacht M | title = Clinical review: Adrenocortical carcinoma: clinical update. | journal = J Clin Endocrinol Metab | volume = 91 | issue = 6 | pages = 2027-37 | year = 2006 | id = PMID 16551738}} [http://jcem.endojournals.org/cgi/content/full/91/6/2027 Free Full Text].</ref> | Adrenocortical carcinoma, generally, carries a poor [[prognosis]].<ref name="allolio">{{cite journal | author = Allolio B, Fassnacht M | title = Clinical review: Adrenocortical carcinoma: clinical update. | journal = J Clin Endocrinol Metab | volume = 91 | issue = 6 | pages = 2027-37 | year = 2006 | id = PMID 16551738}} [http://jcem.endojournals.org/cgi/content/full/91/6/2027 Free Full Text].</ref> | ||
The five-year disease-free survival rate for a complete resection of a [[Cancer staging|stage]] I-III is approximately 30%.<ref name="allolio" /> | The five-year disease-free survival rate for a complete resection of a [[Cancer staging|stage]] I-III is approximately 30%.<ref name="allolio" /> | ||
| Line 37: | Line 38: | ||
The most important prognostic factors are: | The most important prognostic factors are: | ||
* Age of the patient<ref name="pmid26392430">{{cite journal| author=Libé R, Borget I, Ronchi CL, Zaggia B, Kroiss M, Kerkhofs T et al.| title=Prognostic factors in stage III-IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study. | journal=Ann Oncol | year= 2015 | volume= 26 | issue= 10 | pages= 2119-25 | pmid=26392430 | doi=10.1093/annonc/mdv329 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26392430 }}</ref> | * Age of the patient<ref name="pmid26392430">{{cite journal| author=Libé R, Borget I, Ronchi CL, Zaggia B, Kroiss M, Kerkhofs T et al.| title=Prognostic factors in stage III-IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study. | journal=Ann Oncol | year= 2015 | volume= 26 | issue= 10 | pages= 2119-25 | pmid=26392430 | doi=10.1093/annonc/mdv329 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26392430 }}</ref> | ||
* Stage of the tumor<ref name="pmid18063070">{{cite journal| author=Gonzalez RJ, Tamm EP, Ng C, Phan AT, Vassilopoulou-Sellin R, Perrier ND et al.| title=Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma. | journal=Surgery | year= 2007 | volume= 142 | issue= 6 | pages= 867-75; discussion 867-75 | pmid=18063070 | doi=10.1016/j.surg.2007.09.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063070 }}</ref> | * Stage of the [[tumor]]<ref name="pmid18063070">{{cite journal| author=Gonzalez RJ, Tamm EP, Ng C, Phan AT, Vassilopoulou-Sellin R, Perrier ND et al.| title=Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma. | journal=Surgery | year= 2007 | volume= 142 | issue= 6 | pages= 867-75; discussion 867-75 | pmid=18063070 | doi=10.1016/j.surg.2007.09.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063070 }}</ref> | ||
* Mitotic activity<ref name="pmid20694732">{{cite journal| author=Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM| title=Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade. | journal=Langenbecks Arch Surg | year= 2010 | volume= 395 | issue= 7 | pages= 955-61 | pmid=20694732 | doi=10.1007/s00423-010-0698-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20694732 }}</ref> | * [[Mitotic index|Mitotic]] activity<ref name="pmid20694732">{{cite journal| author=Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM| title=Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade. | journal=Langenbecks Arch Surg | year= 2010 | volume= 395 | issue= 7 | pages= 955-61 | pmid=20694732 | doi=10.1007/s00423-010-0698-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20694732 }}</ref> | ||
* Venous invasion | * Venous [[invasion]] | ||
* Weight more than 50 Kg | * Weight more than 50 Kg | ||
* Diameter more than 6.5 cm | * Diameter more than 6.5 cm | ||
* Cortisol production as an adverse prognostic factor | * [[Cortisol]] production as an adverse [[Prognosis|prognostic factor]] | ||
* Ki-67/MIB1 labeling index (antigen identified by monoclonal antibody Ki-67) | * [[Ki-67 (Biology)|Ki-67/]][[MIB1]] labeling index ([[antigen]] identified by [[Monoclonal antibodies|monoclonal antibody]] [[Ki-67 (Biology)|Ki-67]]) | ||
* Survival ranges from a few months to several years<ref name="pmid18505909">{{cite journal| author=Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR| title=Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. | journal=Eur J Endocrinol | year= 2008 | volume= 158 | issue= 6 | pages= 911-9 | pmid=18505909 | doi=10.1530/EJE-07-0723 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18505909 }}</ref> | * Survival ranges from a few months to several years<ref name="pmid18505909">{{cite journal| author=Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR| title=Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. | journal=Eur J Endocrinol | year= 2008 | volume= 158 | issue= 6 | pages= 911-9 | pmid=18505909 | doi=10.1530/EJE-07-0723 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18505909 }}</ref> | ||
Revision as of 15:34, 25 September 2017
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Adrenocortical carcinoma Microchapters |
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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
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.