Adrenal carcinoma: Difference between revisions

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== Classification ==
== Classification ==
* Adrenal cancer is subclassified according to its activity into:<ref name="urlAdrenocortical Carcinoma Treatment - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq#section/_6 |title=Adrenocortical Carcinoma Treatment - National Cancer Institute |format= |work= |accessdate=}}</ref>
*[[Adrenal Cancer|Adrenal cancer]] is subclassified according to its [[Activity (chemistry)|activity]] into:<ref name="urlAdrenocortical Carcinoma Treatment - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq#section/_6 |title=Adrenocortical Carcinoma Treatment - National Cancer Institute |format= |work= |accessdate=}}</ref>
** Functional
**[[Function (biology)|Functional]]
** Non-functional
** Non-[[Function (biology)|functional]]
*In 2017, WHO presented following updated classification of adrenal tumors in fourth edition of the World Health Organization classification of endocrine tumors:<ref name="pmid28477311">{{cite journal| author=Lam AK| title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. | journal=Endocr Pathol | year= 2017 | volume= 28 | issue= 3 | pages= 213-227 | pmid=28477311 | doi=10.1007/s12022-017-9484-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28477311  }} </ref><ref name="urlWHO Classification of Tumours of Endocrine Organs. Fourth Edition - WHO - OMS -">{{cite web |url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4010 |title=WHO Classification of Tumours of Endocrine Organs. Fourth Edition - WHO - OMS - |format= |work= |accessdate=}}</ref><ref name="urlUpdate on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours - Semantic Scholar">{{cite web |url=https://www.semanticscholar.org/paper/Update-on-Adrenal-Tumours-in-2017-World-Health-of-Lam/5e3f9aca58d8986bcaa909a3bd79690c70e05776 |title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours - Semantic Scholar |format= |work= |accessdate=}}</ref><ref name="pmid25743702">{{cite journal| author=Pinto EM, Chen X, Easton J, Finkelstein D, Liu Z, Pounds S et al.| title=Genomic landscape of paediatric adrenocortical tumours. | journal=Nat Commun | year= 2015 | volume= 6 | issue=  | pages= 6302 | pmid=25743702 | doi=10.1038/ncomms7302 | pmc=4352712 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25743702  }} </ref><ref name="pmid27505681">{{cite journal| author=Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA et al.| title=Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. | journal=Cancer Cell | year= 2016 | volume= 30 | issue= 2 | pages= 363 | pmid=27505681 | doi=10.1016/j.ccell.2016.07.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27505681  }} </ref><ref name="pmid27165744">{{cite journal| author=Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA et al.| title=Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. | journal=Cancer Cell | year= 2016 | volume= 29 | issue= 5 | pages= 723-736 | pmid=27165744 | doi=10.1016/j.ccell.2016.04.002 | pmc=4864952 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27165744  }} </ref><ref name="pmid24747642">{{cite journal| author=Assié G, Letouzé E, Fassnacht M, Jouinot A, Luscap W, Barreau O et al.| title=Integrated genomic characterization of adrenocortical carcinoma. | journal=Nat Genet | year= 2014 | volume= 46 | issue= 6 | pages= 607-12 | pmid=24747642 | doi=10.1038/ng.2953 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24747642  }} </ref>
*In 2017, [[WHO]] [[Presenting symptom|presented]] the following updated [[classification]] of [[Adrenal tumor|adrenal tumors]] in fourth edition of the [[World Health Organization]] [[classification]] of [[endocrine tumors]]:<ref name="pmid28477311">{{cite journal| author=Lam AK| title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. | journal=Endocr Pathol | year= 2017 | volume= 28 | issue= 3 | pages= 213-227 | pmid=28477311 | doi=10.1007/s12022-017-9484-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28477311  }} </ref><ref name="urlWHO Classification of Tumours of Endocrine Organs. Fourth Edition - WHO - OMS -">{{cite web |url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4010 |title=WHO Classification of Tumours of Endocrine Organs. Fourth Edition - WHO - OMS - |format= |work= |accessdate=}}</ref><ref name="urlUpdate on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours - Semantic Scholar">{{cite web |url=https://www.semanticscholar.org/paper/Update-on-Adrenal-Tumours-in-2017-World-Health-of-Lam/5e3f9aca58d8986bcaa909a3bd79690c70e05776 |title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours - Semantic Scholar |format= |work= |accessdate=}}</ref><ref name="pmid25743702">{{cite journal| author=Pinto EM, Chen X, Easton J, Finkelstein D, Liu Z, Pounds S et al.| title=Genomic landscape of paediatric adrenocortical tumours. | journal=Nat Commun | year= 2015 | volume= 6 | issue=  | pages= 6302 | pmid=25743702 | doi=10.1038/ncomms7302 | pmc=4352712 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25743702  }} </ref><ref name="pmid27505681">{{cite journal| author=Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA et al.| title=Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. | journal=Cancer Cell | year= 2016 | volume= 30 | issue= 2 | pages= 363 | pmid=27505681 | doi=10.1016/j.ccell.2016.07.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27505681  }} </ref><ref name="pmid27165744">{{cite journal| author=Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA et al.| title=Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. | journal=Cancer Cell | year= 2016 | volume= 29 | issue= 5 | pages= 723-736 | pmid=27165744 | doi=10.1016/j.ccell.2016.04.002 | pmc=4864952 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27165744  }} </ref><ref name="pmid24747642">{{cite journal| author=Assié G, Letouzé E, Fassnacht M, Jouinot A, Luscap W, Barreau O et al.| title=Integrated genomic characterization of adrenocortical carcinoma. | journal=Nat Genet | year= 2014 | volume= 46 | issue= 6 | pages= 607-12 | pmid=24747642 | doi=10.1038/ng.2953 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24747642  }} </ref>


{| class="wikitable"
{| class="wikitable"
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! colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Tumors of adrenal gland}}
! colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Tumors of adrenal gland}}
|-
|-
| rowspan="7"  style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Tumors of adrenal cortex
| rowspan="7"  style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Tumors]] of [[adrenal cortex]]
|
|
* Cortical carcinoma
*[[Cortical area|Cortical]] [[carcinoma]]
|-
|-
|
|
* Cortical adenoma
*[[Cortical area|Cortical]] [[adenoma]]
|-
|-
|
|
* Sex cord stromal tumours
*[[Sex cord-Stromal Tumor|Sex cord stromal tumors]]
|-
|-
|
|
* Adenomatoid tumour
*[[Adenomatoid tumor]]
|-
|-
|
|
* Mesenchymal and stromal tumours:
*[[Mesenchymal]] and [[stromal]] [[tumors]]:
** Myelolipoma
**[[Myelolipoma]]
** Schwannoma
**[[Schwannoma]]
|-
|-
|
|
* Haematological tumors
*[[Haematological malignancy|Haematological tumors]]
|-
|-
|
|
* Secondary tumors
*[[Secondary]] [[tumors]]
|-
|-
| rowspan="5"  style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Tumours of the adrenal medulla and extra-adrenal paraganglia  
| rowspan="5"  style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Tumors]] of the [[adrenal medulla]] and extra-[[adrenal]] [[paraganglia]]
|
|
* Pheochromocytoma
*[[Pheochromocytoma]]
|-
|-
|
|
* Paraganglioma:
*[[Paraganglioma]]:
** Head and neck paraganglioma
**[[Head and neck anatomy|Head and neck]] [[paraganglioma]]
** Sympathetic paraganglioma
**[[Sympathetic]] [[paraganglioma]]
|-
|-
|
|
* Neuroblastic tumors:
*[[Neuroblast|Neuroblastic]] [[tumors]]:
** Neuroblastoma
**[[Neuroblastoma]]
** Nodular ganglioneuroblastoma
**[[Nodular]] ganglioneuroblastoma
** Intermixed ganglioneuroblastoma
** Intermixed ganglioneuroblastoma
** Ganglioneuroma
**[[Ganglioneuroma]]
|-
|-
|
|
* Composite pheochromocytoma
*[[Composite particle|Composite]] [[pheochromocytoma]]
|-
|-
|
|
* Composite paraganglioma
*[[Composite particle|Composite]] [[paraganglioma]]
|}
|}



Revision as of 23:03, 5 August 2019

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Adrenal Carcinoma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an aggressive cancer originating in the cortex (steroid hormone-producing tissue) of the adrenal gland. Adenocortical carcinoma is remarkable for the many hormonal syndromes which can occur in patients with steroid hormone-producing ("functional") tumors, including Cushing's syndrome, Conn syndrome, virilization, and feminization.

Historical perspective

  • In 2017, WHO presented an update on recent classification of adrenal tumors (in fourth edition of the World Health Organization classification of endocrine tumors)[1]

Classification

Updated 2017 WHO classification of adrenal tumors
Tumors of adrenal gland
Tumors of adrenal cortex
Tumors of the adrenal medulla and extra-adrenal paraganglia

Pathophysiology

Epidemiology and demographics

Risk factors

Prognosis

  • ACC, generally, carries a poor prognosis and is unlike most tumours of the adrenal cortex, which are benign (adenomas) and only occasionally cause Cushing's syndrome
  • Five-year disease-free survival for a complete resection of a stage I-III ACC is approximately 30%[20][21][22][23]
  • Weiss criteria has a really good prognostic value for adrenocortical tumors[24]

Diagnosis

History and Symptoms

Symptoms in children

Symptoms in adults

Presentation of non-functional adrenal carcinoma

Physical Examination

Laboratory findings

Laboratory findings of different hormonal syndromes in adrenal carcinoma
Hormonal syndrome Laboratory findings
Cushing syndrome
Virilization
Conn syndrome
Feminization

Imaging studies

CT

PET scan

Molecular imaging

  • Iodometomidate (IMTO) as tracer for molecular imaging of cytochrome P450 family 11B (Cyp11B) enzymes[34]

Treatment

Medical Therapy

Surgery

  • The only curative treatment is complete surgical excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the renal vein or inferior vena cava
  • The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates

References

  1. 1.0 1.1 Lam AK (2017). "Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours". Endocr Pathol. 28 (3): 213–227. doi:10.1007/s12022-017-9484-5. PMID 28477311.
  2. "Adrenocortical Carcinoma Treatment - National Cancer Institute".
  3. "WHO Classification of Tumours of Endocrine Organs. Fourth Edition - WHO - OMS -".
  4. "Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours - Semantic Scholar".
  5. 5.0 5.1 Pinto EM, Chen X, Easton J, Finkelstein D, Liu Z, Pounds S; et al. (2015). "Genomic landscape of paediatric adrenocortical tumours". Nat Commun. 6: 6302. doi:10.1038/ncomms7302. PMC 4352712. PMID 25743702.
  6. Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA; et al. (2016). "Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma". Cancer Cell. 30 (2): 363. doi:10.1016/j.ccell.2016.07.013. PMID 27505681.
  7. Zheng S, Cherniack AD, Dewal N, Moffitt RA, Danilova L, Murray BA; et al. (2016). "Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma". Cancer Cell. 29 (5): 723–736. doi:10.1016/j.ccell.2016.04.002. PMC 4864952. PMID 27165744.
  8. Assié G, Letouzé E, Fassnacht M, Jouinot A, Luscap W, Barreau O; et al. (2014). "Integrated genomic characterization of adrenocortical carcinoma". Nat Genet. 46 (6): 607–12. doi:10.1038/ng.2953. PMID 24747642.
  9. Weiss LM (1984). "Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors". Am J Surg Pathol. 8 (3): 163–9. PMID 6703192.
  10. "Mechanism of abnormal production of adrenal androgens in patients with adrenocortical adenomas and carcinomas | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
  11. "Variable Expression of the Transcription Factors cAMP Response Element-Binding Protein and Inducible cAMP Early Repressor in the Normal Adrenal Cortex and in Adrenocortical Adenomas and Carcinomas | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
  12. Fassnacht M, Libé R, Kroiss M, Allolio B (2011). "Adrenocortical carcinoma: a clinician's update". Nat Rev Endocrinol. 7 (6): 323–35. doi:10.1038/nrendo.2010.235. PMID 21386792.
  13. Menon V, Krishnamurthy SV (2006). "Adrenocortical carcinomas: a 12-year clinicopathologic study of 15 cases". Indian J Pathol Microbiol. 49 (1): 7–11. PMID 16625963.
  14. Magro G, Esposito G, Cecchetto G, Dall'Igna P, Marcato R, Gambini C; et al. (2012). "Pediatric adrenocortical tumors: morphological diagnostic criteria and immunohistochemical expression of matrix metalloproteinase type 2 and human leucocyte-associated antigen (HLA) class II antigens. Results from the Italian Pediatric Rare Tumor (TREP) Study project". Hum Pathol. 43 (1): 31–9. doi:10.1016/j.humpath.2011.04.016. PMID 21820153.
  15. Tischler AS, Kimura N, Mcnicol AM (2006). "Pathology of pheochromocytoma and extra-adrenal paraganglioma". Ann N Y Acad Sci. 1073: 557–70. doi:10.1196/annals.1353.059. PMID 17102124.
  16. Ragazzon B, Libé R, Gaujoux S, Assié G, Fratticci A, Launay P; et al. (2010). "Transcriptome analysis reveals that p53 and {beta}-catenin alterations occur in a group of aggressive adrenocortical cancers". Cancer Res. 70 (21): 8276–81. doi:10.1158/0008-5472.CAN-10-2014. PMID 20959480.
  17. Ragazzon B, Libé R, Assié G, Tissier F, Barreau O, Houdayer C; et al. (2014). "Mass-array screening of frequent mutations in cancers reveals RB1 alterations in aggressive adrenocortical carcinomas". Eur J Endocrinol. 170 (3): 385–91. doi:10.1530/EJE-13-0778. PMID 24347427.
  18. "Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology".
  19. Hsing AW, Nam JM, Co Chien HT, McLaughlin JK, Fraumeni JF (1996). "Risk factors for adrenal cancer: an exploratory study". Int J Cancer. 65 (4): 432–6. doi:10.1002/(SICI)1097-0215(19960208)65:4<432::AID-IJC6>3.0.CO;2-Y. PMID 8621222.
  20. Weiss LM, Medeiros LJ, Vickery AL (1989). "Pathologic features of prognostic significance in adrenocortical carcinoma". Am J Surg Pathol. 13 (3): 202–6. PMID 2919718.
  21. Moreno S, Montoya G, Armstrong J, Leteurtre E, Aubert S, Vantyghem MC; et al. (2004). "Profile and outcome of pure androgen-secreting adrenal tumors in women: experience of 21 cases". Surgery. 136 (6): 1192–8. doi:10.1016/j.surg.2004.06.046. PMID 15657575.
  22. Wagner M, Walter PR, Ghnassia JP, Gasser B (1993). "[Adrenocortical tumors. I. Prognostic evaluation of a series of 17 cases using the Weiss criteria]". Ann Pathol. 13 (5): 306–11. PMID 8311856.
  23. Gandour MJ, Grizzle WE (1986). "A small adrenocortical carcinoma with aggressive behavior. An evaluation of criteria for malignancy". Arch Pathol Lab Med. 110 (11): 1076–9. PMID 3778125.
  24. Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A (2010). "Weiss criteria in large adrenocortical tumors: a validation study". Indian J Pathol Microbiol. 53 (2): 222–6. doi:10.4103/0377-4929.64325. PMID 20551521.
  25. "Hypoaldosteronism accompanied by normal or elevated mineralocorticosteroid pathway steroid: a marker of adrenal carcinoma. | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
  26. "ADRENAL CORTICAL CARCINOMA IN A MALE WITH EXCESS GONADOTROPIN IN THE URINE | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic".
  27. "UpToDate".
  28. Allolio B, Fassnacht M (2006). "Clinical review: Adrenocortical carcinoma: clinical update". J Clin Endocrinol Metab. 91 (6): 2027–37. doi:10.1210/jc.2005-2639. PMID 16551738.
  29. Sandrini R, Ribeiro RC, DeLacerda L (1997). "Childhood adrenocortical tumors". J Clin Endocrinol Metab. 82 (7): 2027–31. doi:10.1210/jcem.82.7.4057. PMID 9215267.
  30. Moreno S, Guillermo M, Decoulx M, Dewailly D, Bresson R, Proye Ch (2006). "Feminizing adreno-cortical carcinomas in male adults. A dire prognosis. Three cases in a series of 801 adrenalectomies and review of the literature". Ann Endocrinol (Paris). 67 (1): 32–8. PMID 16596055.
  31. Szolar DH, Korobkin M, Reittner P, Berghold A, Bauernhofer T, Trummer H; et al. (2005). "Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT". Radiology. 234 (2): 479–85. doi:10.1148/radiol.2342031876. PMID 15671003.
  32. Groussin L, Bonardel G, Silvéra S, Tissier F, Coste J, Abiven G; et al. (2009). "18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients". J Clin Endocrinol Metab. 94 (5): 1713–22. doi:10.1210/jc.2008-2302. PMID 19190108.
  33. Khan TS, Sundin A, Juhlin C, Långström B, Bergström M, Eriksson B (2003). "11C-metomidate PET imaging of adrenocortical cancer". Eur J Nucl Med Mol Imaging. 30 (3): 403–10. doi:10.1007/s00259-002-1025-9. PMID 12634969.
  34. Hahner S, Stuermer A, Kreissl M, Reiners C, Fassnacht M, Haenscheid H; et al. (2008). "[123 I]Iodometomidate for molecular imaging of adrenocortical cytochrome P450 family 11B enzymes". J Clin Endocrinol Metab. 93 (6): 2358–65. doi:10.1210/jc.2008-0050. PMID 18397978.


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