ReviewAdrenocortical carcinoma in a 17th-century girl

- We discuss a report published in 1697 describing the autopsy of a girl who died in 1688 with an adrenocortical carcinoma (ACC).
- Adrenals were controversial in the 17th century, and were not described, but the signs of glucocorticoid and androgen excess are unmistakable
- The tumor invaded the left kidney and had metastasized to the lungs and possibly elsewhere.
- Pediatric ACC is most common in young girls, and is most frequently caused by mutations in the TP53 tumor suppressor gene.

Adrenocortical carcinoma (ACC) is a rare, often fatal disease, that may be seen sporadically or with hereditary predisposition syndromes. Patients with ACC are usually girls under the age of seven who present signs of excess production of adrenal glucocorticoids and androgens, with the diagnosis being confirmed by imaging. Here we reproduce and examine what we believe to be the first autopsy case report of a child with ACC, reported by Dr. Henry Sampson in Philosophical Transactions, published by The Royal Society of London in 1697. The paper describes the autopsy of a girl with severe virilization and profound signs of Cushing syndrome who died at age six, strongly suggesting ACC. She apparently had extensive pulmonary metastases, and may have had liver involvement. The report indicates her disease arose from her left kidney and there is no indication of an adrenal origin, perhaps because the adrenal gland was not generally known as a separate organ at that time. This classic example of an early case report is particularly instructive in the context of medical knowledge and understanding in the 17th century compared to current knowledge.