title: Virilization of a female infant by a maternal adrenocortical carcinoma.
abstract: OBJECTIVE
  To describe a possible mechanism underlying the partial virilization of a 46, XX infant by a functional maternal adrenocortical carcinoma (ACC).
  
  
  METHODS
  We performed immunocytochemical staining of tumor sections for luteinizing hormone (LH)/human chorionic gonadotropin (hCG) receptors. In addition, related reports in the literature are discussed.
  
  
  RESULTS
  A previously healthy mother developed a large cortisol- and androgen-producing stage III adrenal tumor that did not interfere with conception or early morphogenesis. The tumor eluded detection until after delivery of a partially virilized 46, XX female infant with ambiguous genitalia. Immunohistochemical staining of tumor sections revealed overexpression of the LH/hCG receptor. Virilization of the genetically female fetus may have resulted from hCG-stimulated steroid secretion by the ACC.
  
  
  CONCLUSIONS
  Because hypercortisolism and hyperandrogenism are associated with menstrual disturbances and spontaneous abortion, pregnancy in patients with functional adrenal tumors is uncommon. Rarely, maternal steroid excess from a functional adrenal tumor has caused 46, XX disordered sex differentiation. This unusual case demonstrates the influence of hCG on the functionality of an ACC and demonstrates the rare phenomenon of virilization of a female infant by a functional maternal adrenal tumor.
authors.affiliation: Endocrine Surgical Unit, Department of General Surgery, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles California, USA.
authors.forename: Lilah F
authors.initials: LF
authors.lastname: Morris
authors.forename: Steven
authors.initials: S
authors.lastname: Park
authors.forename: Timothy
authors.initials: T
authors.lastname: Daskivich
authors.forename: Bernard M
authors.initials: BM
authors.lastname: Churchill
authors.forename: C V
authors.initials: CV
authors.lastname: Rao
authors.forename: Zhenmin
authors.initials: Z
authors.lastname: Lei
authors.forename: Dorothy S
authors.initials: DS
authors.lastname: Martinez
authors.forename: Michael W
authors.initials: MW
authors.lastname: Yeh
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000378:metabolism; D018268:Adrenocortical Carcinoma / Q000150:complications* / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D011247:Pregnancy; D011248:Pregnancy Complications; D011974:Receptors, LH / Q000378:metabolism; D014770:Virilism / Q000209:etiology* / Q000235:genetics; D055815:Young Adult
chemical_list: D011974:Receptors, LH