Adrenocortical carcinoma risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Overview

The most potent risk factors in the development of adrenocortical cancer are TP53 mutation, Beckwith-Wiedemann syndrome and Carney complex.

Risk Factors

Risk factor associated with adrenocortical carcinoma are:

• Lynch syndrome
• Beckwith-Wiedemann syndrome
• Carney complex
• Neurofibromatosis type 1
• MEN1

Differential Diagnosis	Gene mutations	Clinical picture
Lynch syndrome	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 45	MENIN	Foregut neuroendocrine tumors Pituitary tumors Parathyroid hyperplasia Collagenoma Angiofibroma Adrenal adenoma/hyperplasia
Carney complex	PRKAR1A	Primary pigmented nodular Adrenal disease Large-cell calcifying Sertoli cell tumors Thyroid adenoma Myxoma Somatotroph pituitary adenoma
BWS 41	IGF2, CDKN1C, H19	Wilms’ tumor Hepatoblastoma Macrosomia Adrenocortical cytomegaly Adrenal adenoma Adrenal cyst Hemihypertrophy Macroglossia Omphalocele