title: Adrenocortical carcinoma in a 17th-century girl.
abstract: 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.
authors.affiliation: Department of Medicine, University of Michigan (TE, RJA), United States.
authors.forename: Tobias
authors.initials: T
authors.lastname: Else
authors.affiliation: Department of Medicine, University of Michigan (TE, RJA), United States.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Auchus
authors.affiliation: Department of Pediatrics and The Center for Reproductive Sciences, University of California, San Francisco, CA 94143-0556 (WLM), United States. Electronic address: wlmlab@ucsf.edu.
authors.forename: Walter L
authors.initials: WL
authors.lastname: Miller
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000266:history; D000311:Adrenal Glands / Q000378:metabolism; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000266:history; D000728:Androgens / Q000378:metabolism; D001344:Autopsy; D003480:Cushing Syndrome / Q000175:diagnosis; D005260:Female; D005938:Glucocorticoids / Q000378:metabolism; D049670:History, 17th Century; D006801:Humans; D007668:Kidney / Q000378:metabolism; D009154:Mutation; D009362:Neoplasm Metastasis; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
chemical_list: D000728:Androgens; D005938:Glucocorticoids; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53
keywords: Adrenal; Autopsy; Cancer; Cushing syndrome; Pediatrics