A case of cebocephaly-holoprosencephaly with an aberrant adenohypophysis.
An autopsy case of cebocephaly-holoprosencephaly at 27 weeks' gestation is reported. Chromosome analysis revealed a 46, XX,-7, +der(7), t(7;13) (q32;q34) pat karyotype. Pathological examination disclosed a hypoplastic aberrant adenohypophysis, in which immunohistochemical localization of four anterior pituitary hormones (ACTH, GH, PRL, TSH-beta) was demonstrated. Malformation of the pituitary gland in holoprosencephaly can include ectopic adenohypophysis as well as pituitary dysgenesis, so a careful search for adenohypophysis should be made in future cases for a better understanding of endocrine dysgenesis associated with holoprosencephaly.
Ikeda
H
H
Division of Neurosurgery, Tohoku University, Sendai, Japan.
Niizuma
H
H
Suzuki
J
J
Takabayashi
T
T
Ozawa
N
N
D000015:Abnormalities, Multiple / Q000235:genetics / Q000473:pathology*; D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D002828:Choristoma / Q000473:pathology*; D002872:Chromosome Deletion; D002897:Chromosomes, Human, Pair 7; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D007231:Infant, Newborn; D010255:Paranasal Sinus Neoplasms / Q000473:pathology*; D010903:Pituitary Gland, Anterior; D010908:Pituitary Hormones, Anterior / Q000378:metabolism; D013101:Sphenoid Sinus / Q000473:pathology*
D010908:Pituitary Hormones, Anterior