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Adrenal Cortical Low Grade Carcinoma

MedGen UID:
385205
•Concept ID:
C2349148
•
Neoplastic Process
Synonyms: Adrenal Cortex Low Grade Carcinoma; Adrenal Cortex Low-Grade Carcinoma; Adrenal Cortical Low-Grade Carcinoma; Well Differentiated Adrenal Cortex Carcinoma

Definition

A carcinoma that arises from the adrenal cortex and is characterized by the presence of twenty or less mitoses per ten square millimeters. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenal Cortical Low Grade Carcinoma

Professional guidelines

PubMed

ENDOCRINE TUMOURS: Our experience with the management of patients with non-metastatic adrenocortical carcinoma.
Terzolo M, Fassnacht M
Eur J Endocrinol 2022 Sep 1;187(3):R27-R40. Epub 2022 Jul 19 doi: 10.1530/EJE-22-0260. PMID: 35695575
European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma.
Gaujoux S, Mihai R; joint working group of ESES and ENSAT
Br J Surg 2017 Mar;104(4):358-376. doi: 10.1002/bjs.10414. PMID: 28199015
Impact of monitoring plasma 1,1-dichlorodiphenildichloroethane (o,p'DDD) levels on the treatment of patients with adrenocortical carcinoma.
Baudin E, Pellegriti G, Bonnay M, Penfornis A, Laplanche A, Vassal G, Schlumberger M
Cancer 2001 Sep 15;92(6):1385-92. doi: 10.1002/1097-0142(20010915)92:6<1385::aid-cncr1461>3.0.co;2-2. PMID: 11745214

Recent clinical studies

Etiology

Characteristics and outcomes of immunotherapy-related liver injury in patients with hepatocellular carcinoma versus other advanced solid tumours.
Celsa C, Cabibbo G, Fulgenzi CAM, Scheiner B, D'Alessio A, Manfredi GF, Nishida N, Ang C, Marron TU, Saeed A, Wietharn B, Pinter M, Cheon J, Huang YH, Lee PC, Phen S, Gampa A, Pillai A, Vivaldi C, Salani F, Masi G, Roehlen N, Thimme R, Vogel A, Schönlein M, von Felden J, Schulze K, Wege H, Galle PR, Kudo M, Rimassa L, Singal AG, El Tomb P, Ulahannan S, Parisi A, Chon HJ, Hsu WF, Stefanini B, Verzoni E, Giusti R, Veccia A, Catino A, Aprile G, Guglielmini PF, Di Napoli M, Ermacora P, Antonuzzo L, Rossi E, Verderame F, Zustovich F, Ficorella C, Di Pietro FR, Battelli N, Negrini G, Grossi F, Bordonaro R, Pipitone S, Banzi M, Ricciardi S, Laera L, Russo A, De Giorgi U, Cavanna L, Sorarù M, Montesarchio V, Bordi P, Brunetti L, Pinto C, Bersanelli M, Cammà C, Cortellini A, Pinato DJ
J Hepatol 2024 Mar;80(3):431-442. Epub 2023 Nov 15 doi: 10.1016/j.jhep.2023.10.040. PMID: 37972660
Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study.
Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A, Arlt W, Haak HR, Loli P, Decoudier B, Lasolle H, Quinkler M, Haissaguerre M, Chabre O, Caron P, Stigliano A, Giordano R, Zatelli MC, Bancos I, Fragoso MCBV, Canu L, Luconi M, Puglisi S, Basile V, Reimondo G, Kroiss M, Megerle F, Hahner S, Kimpel O, Dusek T, Nölting S, Bourdeau I, Chortis V, Ettaieb MH, Cosentini D, Grisanti S, Baudin E, Berchialla P, Bovis F, Sormani MP, Bruzzi P, Beuschlein F, Bertherat J, Berruti A
Lancet Diabetes Endocrinol 2023 Oct;11(10):720-730. Epub 2023 Aug 21 doi: 10.1016/S2213-8587(23)00193-6. PMID: 37619579Free PMC Article
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
What Did We Learn from the Molecular Biology of Adrenal Cortical Neoplasia? From Histopathology to Translational Genomics.
Juhlin CC, Bertherat J, Giordano TJ, Hammer GD, Sasano H, Mete O
Endocr Pathol 2021 Mar;32(1):102-133. Epub 2021 Feb 3 doi: 10.1007/s12022-021-09667-0. PMID: 33534120
Data set for reporting of carcinoma of the adrenal cortex: explanations and recommendations of the guidelines from the International Collaboration on Cancer Reporting.
Giordano TJ, Berney D, de Krijger RR, Erickson L, Fassnacht M, Mete O, Papathomas T, Papotti M, Sasano H, Thompson LDR, Volante M, Gill AJ
Hum Pathol 2021 Apr;110:50-61. Epub 2020 Oct 12 doi: 10.1016/j.humpath.2020.10.001. PMID: 33058949

Diagnosis

Comorbid Vulvar Lichen Sclerosus and High-Grade Squamous Intraepithelial Lesions: A Management Conundrum.
Janke MJ, DeBlanc J, Kobernik EK, Skala SL, Hiles GL, Kelly R, Welch KC
J Low Genit Tract Dis 2022 Oct 1;26(4):319-322. Epub 2022 Aug 15 doi: 10.1097/LGT.0000000000000690. PMID: 35972920
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
What Did We Learn from the Molecular Biology of Adrenal Cortical Neoplasia? From Histopathology to Translational Genomics.
Juhlin CC, Bertherat J, Giordano TJ, Hammer GD, Sasano H, Mete O
Endocr Pathol 2021 Mar;32(1):102-133. Epub 2021 Feb 3 doi: 10.1007/s12022-021-09667-0. PMID: 33534120
Diagnostic and Prognostic Biomarkers of Adrenal Cortical Carcinoma.
Mete O, Gucer H, Kefeli M, Asa SL
Am J Surg Pathol 2018 Feb;42(2):201-213. doi: 10.1097/PAS.0000000000000943. PMID: 28877067
New developments in the pathologic diagnosis of adrenal cortical neoplasms. A review.
Medeiros LJ, Weiss LM
Am J Clin Pathol 1992 Jan;97(1):73-83. doi: 10.1093/ajcp/97.1.73. PMID: 1728867

Therapy

Characteristics and outcomes of immunotherapy-related liver injury in patients with hepatocellular carcinoma versus other advanced solid tumours.
Celsa C, Cabibbo G, Fulgenzi CAM, Scheiner B, D'Alessio A, Manfredi GF, Nishida N, Ang C, Marron TU, Saeed A, Wietharn B, Pinter M, Cheon J, Huang YH, Lee PC, Phen S, Gampa A, Pillai A, Vivaldi C, Salani F, Masi G, Roehlen N, Thimme R, Vogel A, Schönlein M, von Felden J, Schulze K, Wege H, Galle PR, Kudo M, Rimassa L, Singal AG, El Tomb P, Ulahannan S, Parisi A, Chon HJ, Hsu WF, Stefanini B, Verzoni E, Giusti R, Veccia A, Catino A, Aprile G, Guglielmini PF, Di Napoli M, Ermacora P, Antonuzzo L, Rossi E, Verderame F, Zustovich F, Ficorella C, Di Pietro FR, Battelli N, Negrini G, Grossi F, Bordonaro R, Pipitone S, Banzi M, Ricciardi S, Laera L, Russo A, De Giorgi U, Cavanna L, Sorarù M, Montesarchio V, Bordi P, Brunetti L, Pinto C, Bersanelli M, Cammà C, Cortellini A, Pinato DJ
J Hepatol 2024 Mar;80(3):431-442. Epub 2023 Nov 15 doi: 10.1016/j.jhep.2023.10.040. PMID: 37972660
Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study.
Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A, Arlt W, Haak HR, Loli P, Decoudier B, Lasolle H, Quinkler M, Haissaguerre M, Chabre O, Caron P, Stigliano A, Giordano R, Zatelli MC, Bancos I, Fragoso MCBV, Canu L, Luconi M, Puglisi S, Basile V, Reimondo G, Kroiss M, Megerle F, Hahner S, Kimpel O, Dusek T, Nölting S, Bourdeau I, Chortis V, Ettaieb MH, Cosentini D, Grisanti S, Baudin E, Berchialla P, Bovis F, Sormani MP, Bruzzi P, Beuschlein F, Bertherat J, Berruti A
Lancet Diabetes Endocrinol 2023 Oct;11(10):720-730. Epub 2023 Aug 21 doi: 10.1016/S2213-8587(23)00193-6. PMID: 37619579Free PMC Article
Favourable prognosis in metastatic adrenal carcinoma: An unexpected outcome.
Hassan I, Sheikh A
J Pak Med Assoc 2022 Oct;72(10):2101-2104. doi: 10.47391/JPMA.3494. PMID: 36661007
Peritoneal metastases from adrenal cortical carcinoma treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Sugarbaker PH
Tumori 2016 Dec 1;102(6):588-592. Epub 2016 Sep 27 doi: 10.5301/tj.5000567. PMID: 27716880
Well-differentiated thymic carcinoma. An organotypical low-grade carcinoma with relationship to cortical thymoma.
Kirchner T, Schalke B, Buchwald J, Ritter M, Marx A, Müller-Hermelink HK
Am J Surg Pathol 1992 Dec;16(12):1153-69. PMID: 1463094

Prognosis

Characteristics and outcomes of immunotherapy-related liver injury in patients with hepatocellular carcinoma versus other advanced solid tumours.
Celsa C, Cabibbo G, Fulgenzi CAM, Scheiner B, D'Alessio A, Manfredi GF, Nishida N, Ang C, Marron TU, Saeed A, Wietharn B, Pinter M, Cheon J, Huang YH, Lee PC, Phen S, Gampa A, Pillai A, Vivaldi C, Salani F, Masi G, Roehlen N, Thimme R, Vogel A, Schönlein M, von Felden J, Schulze K, Wege H, Galle PR, Kudo M, Rimassa L, Singal AG, El Tomb P, Ulahannan S, Parisi A, Chon HJ, Hsu WF, Stefanini B, Verzoni E, Giusti R, Veccia A, Catino A, Aprile G, Guglielmini PF, Di Napoli M, Ermacora P, Antonuzzo L, Rossi E, Verderame F, Zustovich F, Ficorella C, Di Pietro FR, Battelli N, Negrini G, Grossi F, Bordonaro R, Pipitone S, Banzi M, Ricciardi S, Laera L, Russo A, De Giorgi U, Cavanna L, Sorarù M, Montesarchio V, Bordi P, Brunetti L, Pinto C, Bersanelli M, Cammà C, Cortellini A, Pinato DJ
J Hepatol 2024 Mar;80(3):431-442. Epub 2023 Nov 15 doi: 10.1016/j.jhep.2023.10.040. PMID: 37972660
Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study.
Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A, Arlt W, Haak HR, Loli P, Decoudier B, Lasolle H, Quinkler M, Haissaguerre M, Chabre O, Caron P, Stigliano A, Giordano R, Zatelli MC, Bancos I, Fragoso MCBV, Canu L, Luconi M, Puglisi S, Basile V, Reimondo G, Kroiss M, Megerle F, Hahner S, Kimpel O, Dusek T, Nölting S, Bourdeau I, Chortis V, Ettaieb MH, Cosentini D, Grisanti S, Baudin E, Berchialla P, Bovis F, Sormani MP, Bruzzi P, Beuschlein F, Bertherat J, Berruti A
Lancet Diabetes Endocrinol 2023 Oct;11(10):720-730. Epub 2023 Aug 21 doi: 10.1016/S2213-8587(23)00193-6. PMID: 37619579Free PMC Article
Favourable prognosis in metastatic adrenal carcinoma: An unexpected outcome.
Hassan I, Sheikh A
J Pak Med Assoc 2022 Oct;72(10):2101-2104. doi: 10.47391/JPMA.3494. PMID: 36661007
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Diagnostic and Prognostic Biomarkers of Adrenal Cortical Carcinoma.
Mete O, Gucer H, Kefeli M, Asa SL
Am J Surg Pathol 2018 Feb;42(2):201-213. doi: 10.1097/PAS.0000000000000943. PMID: 28877067

Clinical prediction guides

Characteristics and outcomes of immunotherapy-related liver injury in patients with hepatocellular carcinoma versus other advanced solid tumours.
Celsa C, Cabibbo G, Fulgenzi CAM, Scheiner B, D'Alessio A, Manfredi GF, Nishida N, Ang C, Marron TU, Saeed A, Wietharn B, Pinter M, Cheon J, Huang YH, Lee PC, Phen S, Gampa A, Pillai A, Vivaldi C, Salani F, Masi G, Roehlen N, Thimme R, Vogel A, Schönlein M, von Felden J, Schulze K, Wege H, Galle PR, Kudo M, Rimassa L, Singal AG, El Tomb P, Ulahannan S, Parisi A, Chon HJ, Hsu WF, Stefanini B, Verzoni E, Giusti R, Veccia A, Catino A, Aprile G, Guglielmini PF, Di Napoli M, Ermacora P, Antonuzzo L, Rossi E, Verderame F, Zustovich F, Ficorella C, Di Pietro FR, Battelli N, Negrini G, Grossi F, Bordonaro R, Pipitone S, Banzi M, Ricciardi S, Laera L, Russo A, De Giorgi U, Cavanna L, Sorarù M, Montesarchio V, Bordi P, Brunetti L, Pinto C, Bersanelli M, Cammà C, Cortellini A, Pinato DJ
J Hepatol 2024 Mar;80(3):431-442. Epub 2023 Nov 15 doi: 10.1016/j.jhep.2023.10.040. PMID: 37972660
Adjuvant mitotane versus surveillance in low-grade, localised adrenocortical carcinoma (ADIUVO): an international, multicentre, open-label, randomised, phase 3 trial and observational study.
Terzolo M, Fassnacht M, Perotti P, Libé R, Kastelan D, Lacroix A, Arlt W, Haak HR, Loli P, Decoudier B, Lasolle H, Quinkler M, Haissaguerre M, Chabre O, Caron P, Stigliano A, Giordano R, Zatelli MC, Bancos I, Fragoso MCBV, Canu L, Luconi M, Puglisi S, Basile V, Reimondo G, Kroiss M, Megerle F, Hahner S, Kimpel O, Dusek T, Nölting S, Bourdeau I, Chortis V, Ettaieb MH, Cosentini D, Grisanti S, Baudin E, Berchialla P, Bovis F, Sormani MP, Bruzzi P, Beuschlein F, Bertherat J, Berruti A
Lancet Diabetes Endocrinol 2023 Oct;11(10):720-730. Epub 2023 Aug 21 doi: 10.1016/S2213-8587(23)00193-6. PMID: 37619579Free PMC Article
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Erosive Vulvar Lichen Planus and Risk of Vulvar Neoplasia.
Lyra J, Melo C, Figueiredo R, Polonia-Valente R, Falcão V, Beires J, Vieira-Baptista P
J Low Genit Tract Dis 2021 Jan 1;25(1):71-75. doi: 10.1097/LGT.0000000000000581. PMID: 33214502
Diagnostic and Prognostic Biomarkers of Adrenal Cortical Carcinoma.
Mete O, Gucer H, Kefeli M, Asa SL
Am J Surg Pathol 2018 Feb;42(2):201-213. doi: 10.1097/PAS.0000000000000943. PMID: 28877067

Recent systematic reviews

Assessment of prognostic factors in pediatric adrenocortical tumors: a systematic review and evaluation of a modified S-GRAS score.
Riedmeier M, Decarolis B, Haubitz I, Reibetanz J, Wiegering A, Härtel C, Schlegel PG, Fassnacht M, Wiegering V
Eur J Endocrinol 2022 Dec 1;187(6):751-763. Epub 2022 Oct 26 doi: 10.1530/EJE-22-0173. PMID: 36193775

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