Familial adrenocortical carcinoma in association with Lynch syndrome

Benjamin G Challis; Narayanan Kandasamy; Andrew S Powlson; Olympia Koulouri; Anand Kumar  Annamalai; Lisa Happerfield; Alison  Marker; Mark Arends; Serena Nik-Zainal; Mark Gurnell

doi:10.1210/jc.2016-1460#sthash.HPajwAWS.dpuf

Familial adrenocortical carcinoma in association with Lynch syndrome

Benjamin G Challis, Narayanan Kandasamy, Andrew S Powlson, Olympia Koulouri, Anand Kumar Annamalai, Lisa Happerfield, Alison Marker, Mark Arends, Serena Nik-Zainal, Mark Gurnell

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Abstract

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Although the majority of childhood ACC arise in the context of inherited cancer susceptibility syndromes, it remains less clear whether a hereditary tumour predisposition exists for the development of ACC in adults. Here, we report the first occurrence of familial ACC in a family with Lynch syndrome due to a pathogenic germline MSH2 mutation.

Original language	English
Journal	The Journal of Clinical Endocrinology & Metabolism (JCEM)
Early online date	4 May 2016
DOIs	https://doi.org/10.1210/jc.2016-1460#sthash.HPajwAWS.dpuf
Publication status	Published - Jun 2016

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Familial adrenocortical carcinoma in association with Lynch syndrome
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title = "Familial adrenocortical carcinoma in association with Lynch syndrome",

abstract = "Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Although the majority of childhood ACC arise in the context of inherited cancer susceptibility syndromes, it remains less clear whether a hereditary tumour predisposition exists for the development of ACC in adults. Here, we report the first occurrence of familial ACC in a family with Lynch syndrome due to a pathogenic germline MSH2 mutation.",

author = "Challis, \{Benjamin G\} and Narayanan Kandasamy and Powlson, \{Andrew S\} and Olympia Koulouri and Annamalai, \{Anand Kumar\} and Lisa Happerfield and Alison Marker and Mark Arends and Serena Nik-Zainal and Mark Gurnell",

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T1 - Familial adrenocortical carcinoma in association with Lynch syndrome

AU - Challis, Benjamin G

AU - Kandasamy, Narayanan

AU - Powlson, Andrew S

AU - Koulouri, Olympia

AU - Annamalai, Anand Kumar

AU - Happerfield, Lisa

AU - Marker, Alison

AU - Arends, Mark

AU - Nik-Zainal, Serena

AU - Gurnell, Mark

PY - 2016/6

Y1 - 2016/6

N2 - Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Although the majority of childhood ACC arise in the context of inherited cancer susceptibility syndromes, it remains less clear whether a hereditary tumour predisposition exists for the development of ACC in adults. Here, we report the first occurrence of familial ACC in a family with Lynch syndrome due to a pathogenic germline MSH2 mutation.

AB - Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Although the majority of childhood ACC arise in the context of inherited cancer susceptibility syndromes, it remains less clear whether a hereditary tumour predisposition exists for the development of ACC in adults. Here, we report the first occurrence of familial ACC in a family with Lynch syndrome due to a pathogenic germline MSH2 mutation.

U2 - 10.1210/jc.2016-1460#sthash.HPajwAWS.dpuf

DO - 10.1210/jc.2016-1460#sthash.HPajwAWS.dpuf

M3 - Article

SN - 0021-972X

JO - The Journal of Clinical Endocrinology & Metabolism (JCEM)

JF - The Journal of Clinical Endocrinology & Metabolism (JCEM)

ER -

Familial adrenocortical carcinoma in association with Lynch syndrome

Abstract

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