Masculinizing and feminizing syndromes caused by functioning tumors.
Steroidogenic tumors are derived from cells of male and female reproductive tracts, adrenal glands, central nervous system, and, to a lesser degree, from the liver and pituitary gland. The symptoms caused by these tumors are related to their secretory products. Because enzymatic pathways are shared by both adrenal- and gonadal-derived tissues, and the conversion of some of these steroids occurs in the adipose tissue, positive identification of many lesions cannot be based on peripheral blood hormone levels alone, but require complex protocols to improve diagnostic accuracy. Furthermore, these tumors often are smaller than the size limit of conventional imaging modalities and thus demand more precise imaging techniques. Although diagnosis and localization may be challenging, the rewards of a positive prognosis, with complete reversal of symptoms, are more likely to occur with early detection and treatment. This article is a review of the clinical syndromes associated with pediatric steroidogenic tumors; suggested strategies to facilitate their diagnosis, localization, and treatment are provided.
Masiakos
P T
PT
Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USA.
Flynn
C E
CE
Donahoe
P K
PK
D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000175:diagnosis*; D000311:Adrenal Glands / Q000473:pathology; D013734:Androgen-Insensitivity Syndrome / Q000097:blood / Q000175:diagnosis*; D001932:Brain Neoplasms / Q000097:blood / Q000175:diagnosis*; D002648:Child; D005260:Female; D005833:Genital Neoplasms, Female / Q000097:blood / Q000175:diagnosis*; D005834:Genital Neoplasms, Male / Q000097:blood / Q000175:diagnosis*; D012739:Gonadal Steroid Hormones / Q000097:blood*; D006801:Humans; D008297:Male; D010053:Ovary / Q000473:pathology; D009384:Paraneoplastic Endocrine Syndromes / Q000097:blood / Q000175:diagnosis*; D011629:Puberty, Precocious / Q000097:blood / Q000175:diagnosis; D013737:Testis / Q000473:pathology; D014770:Virilism / Q000097:blood / Q000175:diagnosis*
D012739:Gonadal Steroid Hormones