Adrenocortical carcinoma causes

	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] Mohammed Abdelwahed M.D [4]

Overview

There are no established causes for adrenocortical carcinoma. The relatively increased incidence in childhood is mainly explained by germline TP53 mutations, which are the underlying genetic cause of ACC in more than 50% to 80% of children.

Causes

There are no established causes for adrenocortical carcinoma.
The relatively increased incidence in childhood is mainly explained by germline TP53 mutations, which are the underlying genetic cause of ACC in >50% to 80% of children.

Genetics

Hereditary syndromes associated with adrenocortical carcinoma are:

Lynch syndrome


Associated conditions	Gene mutations	Clinical picture
Lynch syndrome^[1]	MSH2, MSH6, MLH1, PMS2	Colorectal cancer Endometrial cancer Sebaceous neoplasms Ovarian cancer Pancreatic cancer Brain cancer
Neurofibromatosis type 1	NF1	Malignant peripheral nerve sheet tumor Pheochromocytoma Café au lait spots Neurofibroma Optic glioma Lisch nodule Skeletal abnormalities
MEN1^[2]	MENIN	Foregut neuroendocrine tumors Pituitary tumors Parathyroid hyperplasia Collagenoma Angiofibroma Adrenal adenoma/hyperplasia
Carney complex	PRKAR1A	Adrenal disease Sertoli cell tumors Thyroid adenoma Myxoma Somatotroph pituitary adenoma
BWS^[3]	IGF2, CDKN1C, H19	Wilm’s tumor Hepatoblastoma Macrosomia Adrenocortical cytomegaly Adrenal adenoma Adrenal cyst Hemihypertrophy Macroglossia Omphalocele

References

↑ Carethers JM, Stoffel EM (2015). "Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer". World J Gastroenterol. 21 (31): 9253–61. doi:10.3748/wjg.v21.i31.9253. PMC 4541378. PMID 26309352.
↑ B. Gatta-Cherifi, O. Chabre, A. Murat, P. Niccoli, C. Cardot-Bauters, V. Rohmer, J. Young, B. Delemer, H. Du Boullay, M. F. Verger, J. M. Kuhn, J. L. Sadoul, Ph Ruszniewski, A. Beckers, M. Monsaingeon, E. Baudin, P. Goudet & A. Tabarin (2012). "Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database". European journal of endocrinology. 166 (2): 269–279. doi:10.1530/EJE-11-0679. PMID 22084155. Unknown parameter |month= ignored (help)
↑ H. Segers, R. Kersseboom, M. Alders, R. Pieters, A. Wagner & M. M. van den Heuvel-Eibrink (2012). "Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients". European journal of cancer (Oxford, England : 1990). 48 (17): 3249–3256. doi:10.1016/j.ejca.2012.06.008. PMID 22796116. Unknown parameter |month= ignored (help)

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[pmid26309352-1] Carethers JM, Stoffel EM (2015). "Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer". World J Gastroenterol. 21 (31): 9253–61. doi:10.3748/wjg.v21.i31.9253. PMC 4541378. PMID 26309352.

[2] B. Gatta-Cherifi, O. Chabre, A. Murat, P. Niccoli, C. Cardot-Bauters, V. Rohmer, J. Young, B. Delemer, H. Du Boullay, M. F. Verger, J. M. Kuhn, J. L. Sadoul, Ph Ruszniewski, A. Beckers, M. Monsaingeon, E. Baudin, P. Goudet & A. Tabarin (2012). "Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database". European journal of endocrinology. 166 (2): 269–279. doi:10.1530/EJE-11-0679. PMID 22084155. Unknown parameter |month= ignored (help)

[3] H. Segers, R. Kersseboom, M. Alders, R. Pieters, A. Wagner & M. M. van den Heuvel-Eibrink (2012). "Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients". European journal of cancer (Oxford, England : 1990). 48 (17): 3249–3256. doi:10.1016/j.ejca.2012.06.008. PMID 22796116. Unknown parameter |month= ignored (help)

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