Adrenocortical carcinoma natural history: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Adrenocortical carcinoma}} | {{Adrenocortical carcinoma}} | ||
{{CMG}} {{AE}} {{RT}} {{AAM}} | {{CMG}}; {{AE}} {{RT}} {{AAM}} {{MAD}} | ||
==Overview== | ==Overview== | ||
If left untreated, patients with adrenocortical carcinoma may | If left untreated, patients with adrenocortical carcinoma may develop [[diabetes mellitus]], [[osteoporosis]], delayed [[wound healing|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 [[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 | 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 features. | ||
Without treatment, the patient | Without treatment, the patient may develop complications such as [[diabetes mellitus]], [[osteoporosis]], [[hypertension]], [[hypercoagulable states]], [[metastasis]] to other organs, [[cardiovascular]] and [[Cerebrovascular disease|cerebrovascular diseases]]. | ||
==Complications== | ==Complications== | ||
| Line 24: | Line 22: | ||
* [[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]] | **[[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]] | ||
* [[Tumor]] [[thrombus]] formation. | |||
* [[Tumor]] [[thrombus]] formation | |||
The most important prognostic factors are: | ==Prognosis== | ||
* 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> | * 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> | ||
* 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> | * The five-year disease-free survival rate for a complete [[resection]] of a [[Cancer staging|stage]] I-III is approximately 30%.<ref name="allolio" /> | ||
* [[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> | * 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> | ||
'''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> | |||
* 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 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 | * Size/Diameter more than 6.5 cm | ||
* [[Cortisol]] production | * [[Cortisol]] production is an adverse [[Prognosis|prognostic factor]] | ||
* [[Ki-67 (Biology)|Ki-67/]][[MIB1]] labeling index ([[antigen]] identified by [[Monoclonal antibodies|monoclonal antibody]] [[Ki-67 (Biology)|Ki-67]]) | * [[Ki-67 (Biology)|Ki-67/]][[MIB1]] labeling index ([[antigen]] identified by [[Monoclonal antibodies|monoclonal antibody]] [[Ki-67 (Biology)|Ki-67]]). | ||
==References== | ==References== | ||
Latest revision as of 18:51, 30 October 2017
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Adrenocortical carcinoma Microchapters |
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Differentiating Adrenocortical carcinoma from other Diseases |
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Adrenocortical carcinoma natural history On the Web |
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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] Mohammed Abdelwahed M.D[4]
Overview
If left untreated, patients with adrenocortical carcinoma may develop diabetes mellitus, 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 the facial features.
Without treatment, the patient may develop complications such as diabetes mellitus, osteoporosis, hypertension, hypercoagulable states, metastasis to other organs, cardiovascular and cerebrovascular diseases.
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
- 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]
- Survival ranges from a few months to several years.[2]
The most important prognostic factors are:
- Age of the patient [3]
- Stage of the tumor [4]
- Mitotic activity [5]
- Venous invasion
- Weight more than 50 Kg
- Size/Diameter more than 6.5 cm
- Cortisol production is an adverse prognostic factor
- Ki-67/MIB1 labeling index (antigen identified by monoclonal antibody Ki-67).
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.
- ↑ 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.
- ↑ 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.