ArticleEndocrine connections2019
Adrenocortical carcinoma in patients with MEN1: a kindred report and review of the literature.
Article in Endocrine connections, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 24 citations in OpenAlex.
- Concomitant parathyroid hormone-secreting oncocytic adrenocortical carcinoma and parathyroid adenoma.JCEM case reports · 2026Article
- [A combination of endogenous hypercortisolism and primary hyperparathyroidism: clinical and genetic characteristics].Problemy endokrinologii · 2026Observational
- In-depth Genetic and Molecular Characterization of Unilateral Coexisting Adrenal Cortical Adenoma and Carcinoma in the Context of MEN1 Syndrome.Endocrine pathology · 2026Article
- Multiple Endocrine Neoplasia Type 1 With Adrenal Cortical Adrenocortical Carcinoma: A 25-Year Follow-Up and Family Report.JCEM case reports · 2025Article
- Bilateral and recurrent adrenocortical carcinoma in MEN1: a case report and review of the literature.Endocrine oncology (Bristol, England) · 2025Article
- Sulfasalazine as a cause of false-positive elevation of urinary normetanephrine in patients with adrenal mass and suspected phaeochromocytoma: a diagnostic challenge.BMJ case reports · 2023Article
- Multiple endocrine neoplasia type 1 with Zollinger-Ellison syndrome: clinicopathological analysis of a Japanese family with focus on menin immunohistochemistry.Frontiers in endocrinology · 2023Article
- Bridging the Scientific Gaps to Identify Effective Treatments in Adrenocortical Cancer.Cancers · 2022Review
- Adrenocortical carcinoma: Pediatric aspects (Review).Experimental and therapeutic medicine · 2022Review
- Identification of a ferroptosis-related long noncoding RNA signature with a prognostic value in adrenocortical carcinoma.Frontiers in genetics · 2022Article
- Molecular genotyping of adrenocortical carcinoma: a systematic analysis of published literature 2019-2021.Current opinion in oncology · 2022Review
- Pediatric adrenocortical carcinoma.Frontiers in endocrinology · 2022Review
- The Challenging Pharmacokinetics of Mitotane: An Old Drug in Need of New Packaging.European journal of drug metabolism and pharmacokinetics · 2021Review
- Clinical aspects of multiple endocrine neoplasia type 1.Nature reviews. Endocrinology · 2021Review
- Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging.Biomedicines · 2021Review
- Multiple endocrine neoplasia 1: a broad overview.Therapeutic advances in chronic disease · 2021Review
- Genomic and Clinical Correlates of Adrenocortical Carcinoma in an Adult Patient with Li-Fraumeni Syndrome: A Case Report.Current oncology (Toronto, Ont.) · 2020Article
- Medical Approaches in Adrenocortical Carcinoma.Biomedicines · 2020Review
- Phenotypes Associated With MEN1 Syndrome: A Focus on Genotype-Phenotype Correlations.Frontiers in endocrinology · 2020Review
Corrections and comments
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Authors and funding
12 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Objective Up to 40% of multiple endocrine neoplasia type 1 (MEN1) patients may have adrenal cortical tumors. However, adrenocortical carcinoma (ACC) is rare. The clinical manifestations, prevalence, inheritance and prognosis of ACC associated with MEN1 remain unclear. Here we report the clinical manifestations and prevalence of ACC in patients with MEN1. Design and methods A retrospective analysis of ACC associated with MEN1 patients at a single tertiary care center from December 2001 to June 2017. Genetic analysis of MEN1 and other ACC associated genes, loss of heterozygosity (LOH) of MEN1 locus, immunohistochemistry staining of menin, P53 and β-catenin in ACC tissue were performed. Results Two related patients had ACC associated with MEN1. The father had ENSAT stage IV tumor with excessive production of cortisol; the daughter had nonfunctional ENSAT stage I tumor. Both patients carried novel germline heterozygous mutation (c.400_401insC) of MEN1. The wild-type MEN1 allele was lost in the resected ACC tissue from the daughter with no menin staining. The ACC tissue had nuclear β-catenin staining, with heterozygous CTNNB1 mutation of 357del24 and P53 staining in only 20% cells. Conclusions ACC associated with MEN1 is rare and may occur in familial aggregates.
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