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Updated: May 27, 2026

A Novel Method: Super-selective Adrenal Venous Sampling

A Novel Method: Super-selective Adrenal Venous Sampling

Published on: September 15, 2017

Carney complex with adrenal cortical carcinoma.

Emilie Morin¹, Ozgur Mete, Jonathan D Wasserman

¹Department of Medicine, University Health Network, and Hospital for Sick Children, Toronto, Ontario, Canada.

The Journal of Clinical Endocrinology and Metabolism

|November 25, 2011

Summary

This summary is machine-generated.

Carney complex patients can develop adrenal cortical carcinoma, a rare malignancy. This study details a case, including genetic findings and response to radiation and kinase inhibitors, impacting future surveillance and management.

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Last Updated: May 27, 2026

A Novel Method: Super-selective Adrenal Venous Sampling

A Novel Method: Super-selective Adrenal Venous Sampling

Published on: September 15, 2017

Area of Science:

Endocrinology
Oncology
Genetics

Background:

Carney complex is a rare genetic syndrome associated with multiple neoplasms.
Adrenal cortical carcinoma is a rare malignancy with a poor prognosis, not previously linked to Carney complex.

Observation:

A 22-year-old female with Carney complex developed an 8.5-cm adrenal mass (T3NXM0 carcinoma).
Pathology revealed high MIB-1 labeling (20%) and angioinvasion.
Nontumorous adrenal cortex showed features of primary pigmented nodular adrenocortical disease.

Findings:

Genetic analysis identified a novel PRKAR1 frame shift mutation and a heterozygous p53 polymorphic substitution.
The patient's liver recurrence showed a partial response to stereotactic radiotherapy and sorafenib multikinase inhibition.

Implications:

This is the first characterization of malignancy in Carney complex patients.
Findings suggest the need for enhanced surveillance and tailored management strategies for individuals with this genetic syndrome.