{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:48Z","timestamp":1784557068944,"version":"3.55.0"},"reference-count":14,"publisher":"Bioscientifica","issue":"4","license":[{"start":{"date-parts":[[2024,10,1]],"date-time":"2024-10-01T00:00:00Z","timestamp":1727740800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,10,1]]},"abstract":"\n Summary<\/jats:title>\n Adrenocortical carcinoma (ACC) is a rare malignant tumour arising from the adrenal cortex, with an estimated annual incidence of one to two patients per million. Ectopic ACCs are extremely rare. The majority of ACCs are sporadic; however, ACC has been linked with genetic disease processes, including multiple endocrine neoplasia type-1 (MEN-1). We present the case of a 66-year-old lady referred with newly diagnosed diabetes on a background of primary hyperparathyroidism. Examination revealed Cushingoid features, and hormonal evaluation confirmed ACTH-independent Cushing\u2019s syndrome. Morning cortisol after a 1 mg overnight dexamethasone suppression test was 548 nmol\/L with an undetectable ACTH <3.0 pg\/mL. Dehydroepiandrosterone sulphate was 5.3 \u03bcmol\/L and androstenedione 3.49 nmol\/L, both of which were normal. Testosterone was suppressed at <0.4 nmol\/L. Imaging revealed a 6 \u00d7 6 \u00d7 4.5 cm right-sided presumed adrenal lesion, a pancreatic lesion (2.5 \u00d7 1.6 cm), and bilateral pulmonary nodules (0.9 \u00d7 0.8 cm, 0.7 \u00d7 0.6 cm, 0.3 cm). Right adrenalectomy was performed, and histology was consistent with an extra-adrenal ACC (Weiss score 5\/9) within the peri-adrenal adipose tissue. The resected adrenal gland was normal. Lung biopsy confirmed metastatic ACC tissue, and endoscopic ultrasound-guided biopsy of the pancreatic lesion revealed a pancreatic neuroendocrine tumour, which was confirmed biochemically to be an insulinoma. Genetic assessment confirmed MEN-1. This case highlights the importance of screening for MEN-1 in at-risk patients and the need for close clinical follow-up. To our knowledge, this is the first case report of extra-adrenal ACC in MEN-1 syndrome.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Adrenal lesions in MEN-1 syndrome have significant malignant potential. Newly diagnosed lesions should be followed closely with short-interval imaging, and a lower threshold for surgical removal is suggested.<\/jats:p>\n <\/jats:list-item>\n \n Primary hyperparathyroidism is often the earliest laboratory or clinical manifestation of MEN-1 syndrome. A detailed medical and family history is vital in order to appropriately identify patients at risk of MEN-1.<\/jats:p>\n <\/jats:list-item>\n \n To our knowledge, this is the first case report of extra-adrenal adrenocortical carcinoma in MEN-1 syndrome.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-23-0068","type":"journal-article","created":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T08:19:01Z","timestamp":1732609141000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["Extra-adrenal adrenocortical cancer associated with multiple endocrine neoplasia type 1"],"prefix":"10.1530","volume":"2024","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6470-5067","authenticated-orcid":true,"given":"Deirdre","family":"Green","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Beaumont Hospital, Dublin, Ireland"},{"name":"Academic Department of Endocrinology, Royal College of Surgeons in Ireland, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"Richards","sequence":"additional","affiliation":[{"name":"Department of Histopathology, Beaumont Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brendan","family":"Doyle","sequence":"additional","affiliation":[{"name":"Department of Histopathology, Beaumont Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Beaumont Hospital, Dublin, Ireland"},{"name":"Academic Department of Endocrinology, Royal College of Surgeons in Ireland, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnold","family":"Hill","sequence":"additional","affiliation":[{"name":"Department of Surgery, Royal College of Surgeons in Ireland and Beaumont Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael W","family":"O\u2019Reilly","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Beaumont Hospital, Dublin, Ireland"},{"name":"Academic Department of Endocrinology, Royal College of Surgeons in Ireland, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Sherlock","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Beaumont Hospital, Dublin, Ireland"},{"name":"Academic Department of Endocrinology, Royal College of Surgeons in Ireland, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Therapeutic Advances in Chronic Disease","first-page":"20406223211033103","article-title":"Adrenocortical carcinoma: current state of the art, ongoing controversies, and future directions in diagnosis and treatment","volume":"12","author":"Shariq","year":"2021","unstructured":"Shariq OA & McKenzie TJ. Adrenocortical carcinoma: current state of the art, ongoing controversies, and future directions in diagnosis and treatment. Therapeutic Advances in Chronic Disease 2021 12 20406223211033103. (https:\/\/doi.org\/10.1177\/20406223211033103)"},{"key":"bib2","series-title":"Biomedicines","first-page":"175","article-title":"Adrenocortical carcinoma: updates of clinical and pathological features after renewed World Health Organisation classification and pathology staging","volume":"9","author":"Lam","year":"2021","unstructured":"Lam AKY. Adrenocortical carcinoma: updates of clinical and pathological features after renewed World Health Organisation classification and pathology staging. Biomedicines 2021 9 175. (https:\/\/doi.org\/10.3390\/biomedicines9020175)"},{"key":"bib3","series-title":"Endocrine Pathology","first-page":"375","article-title":"Adrenal rests in the uro-geni","volume":"32","author":"Falco","year":"2021","unstructured":"Falco ECDaniele LMetovic JBollito EDe Rosa GVolante M & Papotti M. Adrenal rests in the uro-genital tract of an adult population. Endocrine Pathology 2021 32 375\u2013384. (https:\/\/doi.org\/10.1007\/s12022-021-09685-y)"},{"key":"bib4","series-title":"Journal of Clinical Oncology","first-page":"1250","article-title":"Beyond Li Fraumeni Syndrome: clinical characteristics of families with p53 germline mutations","volume":"27","author":"Gonzalez","year":"2009","unstructured":"Gonzalez KDNoltner KABuzin CHGu DWen-Fong CYNguyen VQHan JHLowstuter KLongmate JSommer SS, et al. Beyond Li Fraumeni Syndrome: clinical characteristics of families with p53 germline mutations. Journal of Clinical Oncology 2009 27 1250\u20131256. (https:\/\/doi.org\/10.1200\/JCO.2008.16.6959)"},{"key":"bib5","series-title":"Journal of Clinical Oncology","first-page":"602","article-title":"Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: a Children\u2019s Oncology Group study","volume":"33","author":"Wasserman","year":"2015","unstructured":"Wasserman JDNovokmet AEichler-Jonsson CRibeiro RCRodriguez-Galindo CZambetti GP & Malkin D. Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: a Children\u2019s Oncology Group study. Journal of Clinical Oncology 2015 33 602\u2013609. (https:\/\/doi.org\/10.1200\/JCO.2013.52.6863)"},{"key":"bib6","series-title":"Journal of Clinical Oncology","first-page":"3012","article-title":"Adrenocortical carcinoma is a lynch syndrome-associated cancer","volume":"31","author":"Raymond","year":"2013","unstructured":"Raymond VMEverett JNFurtado LVGustafson SLJungbluth CRGruber SBHammer GDStoffel EMGreenson JKGiordano TJ, et al. Adrenocortical carcinoma is a lynch syndrome-associated cancer. Journal of Clinical Oncology 2013 31 3012\u20133018. (https:\/\/doi.org\/10.1200\/JCO.2012.48.0988)"},{"key":"bib7","series-title":"Diseases of the Colon and Rectum","first-page":"1739","article-title":"Adrenal masses are associated with familial adenomatous polyposis","volume":"43","author":"Smith","year":"2000","unstructured":"Smith TGClark SKKatz DEReznek RH & Phillips RK. Adrenal masses are associated with familial adenomatous polyposis. Diseases of the Colon and Rectum 2000 43 1739\u20131742. (https:\/\/doi.org\/10.1007\/BF02236860)"},{"key":"bib8","series-title":"Surgery","first-page":"1077","article-title":"Adrenal lesion in multiple endocrine neoplasia type 1","volume":"118","author":"Skogseid","year":"1995","unstructured":"Skogseid BRastad JGobl ALarsson CBacklin KJuhlin CAkerstr\u00f6m G & Oberg K. Adrenal lesion in multiple endocrine neoplasia type 1. Surgery 1995 118 1077\u20131082. (https:\/\/doi.org\/10.1016\/s0039-6060(05)80117-5)"},{"key":"bib9","series-title":"World Journal of Surgery","first-page":"891","article-title":"Adrenal involvement in multiple endocrine neoplasia Type 1","volume":"26","author":"Langer","year":"2002","unstructured":"Langer PCupisti KBartsch DKNies CGoretzki PERothmund M & R\u00f6her HD. Adrenal involvement in multiple endocrine neoplasia Type 1. World Journal of Surgery 2002 26 891\u2013896. (https:\/\/doi.org\/10.1007\/s00268-002-6492-4)"},{"key":"bib10","series-title":"Frontiers in Endocrinology","first-page":"339","article-title":"Multiple endocrine neoplasia Type 1 (MEN1): an update and the significance of early genetic and clinical diagnosis","volume":"10","author":"Kamilaris","year":"2019","unstructured":"Kamilaris CDC & Stratakis CA. Multiple endocrine neoplasia Type 1 (MEN1): an update and the significance of early genetic and clinical diagnosis. Frontiers in Endocrinology 2019 10 339. (https:\/\/doi.org\/10.3389\/fendo.2019.00339)"},{"key":"bib11","series-title":"Acta Medica Indonesiana","first-page":"257","article-title":"An interesting case of hepatic adrenocortical carcinoma","volume":"50","author":"Permana","year":"2018","unstructured":"Permana HDarmawan GRitonga EKusumawati MMiftahurachman M & Soetedjo NN. An interesting case of hepatic adrenocortical carcinoma. Acta Medica Indonesiana 2018 50 257\u2013259."},{"key":"bib12","series-title":"World Journal of Clinical Cases","first-page":"2075","article-title":"Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: a case report","volume":"7","author":"Zhou","year":"2019","unstructured":"Zhou D-KLiu Z-HGao B-Q & Wang W-L. Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: a case report. World Journal of Clinical Cases 2019 7 2075\u20132080. (https:\/\/doi.org\/10.12998\/wjcc.v7.i15.2075)"},{"key":"bib13","series-title":"Familial Cancer","first-page":"381","article-title":"Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome","volume":"17","author":"Wright","year":"2018","unstructured":"Wright JPMontgomery KWTierney JGilbert JSol\u00f3rzano CC & Idrees K. Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome. Familial Cancer 2018 17 381\u2013385. (https:\/\/doi.org\/10.1007\/s10689-017-0042-6)"},{"key":"bib14","series-title":"Virchows Archives","article-title":"Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants.","author":"de Krijger","year":"2012","unstructured":"de Krijger RR Papathomas TG. Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants. Virchows Archives 2012 4609\u201318. (https:\/\/doi.org\/10.1007\/s00428-011-1166-y)"}],"container-title":["Endocrinology, Diabetes & Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2024\/4\/EDM23-0068.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2024\/4\/EDM23-0068.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2024\/4\/EDM23-0068.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:48:15Z","timestamp":1784555295000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/41581"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,1]]},"references-count":14,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.1530\/edm-23-0068","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,1]]}}}