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 Lynch syndrome, Beckwith-Wiedemann syndrome, Carney complex, Neurofibromatosis type 1, Multiple endocrine neoplasia type 1 (MEN1), and Carney complex.

Risk Factors

Risk factor associated with adrenocortical carcinoma are:

• Lynch syndrome
• Beckwith-Wiedemann syndrome
• Carney complex
• Neurofibromatosis type 1
• Multiple endocrine neoplasia 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	MENIN	Foregut neuroendocrine tumors Pituitary tumors Parathyroid hyperplasia Collagenoma Angiofibroma Adrenal adenoma/hyperplasia
Carney complex	PRKAR1A	Adrenal disease Sertoli cell tumors Thyroid adenoma Myxoma Somatotroph pituitary adenoma
BWS	IGF2, CDKN1C, H19	Wilm’s tumor Hepatoblastoma Macrosomia Adrenocortical cytomegaly Adrenal adenoma Adrenal cyst Hemihypertrophy Macroglossia Omphalocele