A case of estrogen-secreting adrenocortical carcinoma with subclinical Cushing's syndrome.
A 25-year-old man was found to have a large right adrenal mass detected by abdominal echography and computed tomography, and presented with a mild gynecomastia. Endocrine study showed increased serum concentrations and urinary excretion of estrogens and dehydroepiandorosterone sulfate (DHEA-S). The patient had no Cushingoid features but autonomous cortisol secretion, compatible with the diagnosis of subclinical Cushing's syndrome. Surgical removal of the adrenal tumor led to normalization of serum and urinary excretion of estrogens and DHEA-S. Histopathological examination revealed a high-grade adrenocortical carcinoma (ACC). The disorganized expression of all the steroidogenic enzymes in individual tumor cells was demonstrated by immunohistochemical analysis, and the abundant expression of both aromatase mRNA and insulin-like growth factor (IGF)-II mRNA was shown by RT-PCR. These data suggest the excessive secretion of estrogen as well as the ineffective steroidogenesis by the adrenal tumor. This is a very rare case of estrogen-secreting ACC associated with subclinical Cushing's syndrome.
Fukai
Nozomi
N
Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Japan.
Hirono
Yuki
Y
Yoshimoto
Takanobu
T
Doi
Masaru
M
Ohtsuka
Yukihiko
Y
Homma
Keiko
K
Shibata
Hirotaka
H
Sasano
Hironobu
H
Hirata
Yukio
Y
D000306:Adrenal Cortex Neoplasms / Q000473:pathology / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism* / Q000473:pathology; D000328:Adult; D003480:Cushing Syndrome / Q000150:complications*; D003687:Dehydroepiandrosterone / Q000097:blood / Q000652:urine; D004967:Estrogens / Q000097:blood / Q000378:metabolism* / Q000652:urine; D006729:Hormones, Ectopic / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis
D004967:Estrogens; D006729:Hormones, Ectopic; D003687:Dehydroepiandrosterone