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October 1, 2024Endocrinology Diabetes and Metabolism Case ReportsOpen Access

Extra-adrenal adrenocortical cancer associated with multiple endocrine neoplasia type 1

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Why the study?

Adrenocortical carcinoma is a rare malignancy that has been linked with MEN-1, but ectopic extra-adrenal presentations are extremely rare.

Population

A 66-year-old lady with newly diagnosed diabetes and primary hyperparathyroidism

Design

Case report

Authors

DGDeirdre GreenKRKate RichardsBDBrendan Doyle

Discussion

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Overview

Ectopic ACC may present with new diabetes and hyperparathyroidism; extends MEN-1 spectrum but leaves open diagnostic implications.

Structured PICO

P
Population
66-year-old woman with newly diagnosed diabetes, primary hyperparathyroidism, and ACTH-independent Cushing's syndrome
I
Intervention
Right adrenalectomy, lung biopsy, and endoscopic ultrasound-guided biopsy
O
Outcome
Histological and genetic diagnosis

This is the first reported case of extra-adrenal adrenocortical carcinoma in a patient with MEN-1 syndrome, highlighting the need for screening and close follow-up of adrenal lesions in this population.

Cite This Study

Green et al. (2024) studied this question.

synapsesocial.com/papers/6a702299f44fa9f079ddb0b1https://doi.org/10.1530/edm-23-0068
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