An inhibin B and estrogen-secreting adrenocortical carcinoma leading to selective FSH suppression.
OBJECTIVE
Hormone-secreting adrenocortical tumors are frequently associated with endocrine syndromes. We describe a 30-year-old man who had abdominal pain, a nodule in the right breast and loss of libido. Abdominal magnetic resonance imaging revealed a very large tumor in the right adrenal gland.


METHODS
Hormonal profile disclosed increased levels of estradiol and slightly low testosterone levels. The basal and stimulated LH levels were normal, whereas basal and stimulated FSH levels were totally suppressed. Cortisol and adrenal androgen levels were normal. The unusual finding of selective FSH suppression suggested secretion of inhibin B by the adrenocortical tumor. A very high level of serum inhibin B (405 pg/ml) was demonstrated by ELISA assay. Right adrenalectomy and nephrectomy were performed and the tumor was classified as a malignant tumor (Weiss score: 7.0) and unilateral mastectomy disclosed a lipoma.


RESULTS
One week after surgery, a GnRH-stimulation test disclosed normal basal and stimulated FSH levels and low levels of inhibin B and estradiol. Immunohistochemical analysis with anti-B-inhibin antibody revealed intense staining in the adrenocortical tumor cells. One month after surgery, an abdominal magnetic resonance imaging revealed a local recurrence of the tumor and a second surgery was performed with partial resection of the tumor and the patient died 1 year after the first surgery.


CONCLUSIONS
We herein report the first inhibin B and estradiol-secreting adrenocortical carcinoma. The unusual selective inhibition of FSH secretion should be considered a valuable hormonal finding for the diagnosis of inhibin B-secreting adrenocortical tumors.
Fragoso
Maria Candida Barisson Villares
MC
Laboratório de Hormônios e Genética Molecular LIM/42, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. mariafragoso@uol.com.br
Kohek
Maria Beatriz Fonte
MB
Martin
Regina Matsunaga
RM
Latronico
Ana Claudia
AC
Lucon
Antonio Marmo
AM
Zerbini
Maria Claudia
MC
Longui
Carlos Alberto
CA
Mendonca
Berenice Bilharinho
BB
Domenice
Sorahia
S
D000306:Adrenal Cortex Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D000328:Adult; D002277:Carcinoma / Q000097:blood* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D015536:Down-Regulation; D004958:Estradiol / Q000097:blood* / Q000378:metabolism; D017809:Fatal Outcome; D005640:Follicle Stimulating Hormone / Q000097:blood*; D006801:Humans; D007150:Immunohistochemistry; D007265:Inhibins / Q000097:blood* / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male
C422364:inhibin B; D004958:Estradiol; D007265:Inhibins; D005640:Follicle Stimulating Hormone