Feminizing adreno-cortical carcinomas in male adults. A dire prognosis. Three cases in a series of 801 adrenalectomies and review of the literature.
We describe the clinical presentation, biochemical features, diagnostic criteria, clinical course and differential diagnosis in three cases of feminizing adreno-cortical carcinoma (FACC) with a review of the literature.


METHODS
From 1970 throughout December 2003 among a series of 801 adrenalectomies, three had been performed for FACC.


RESULTS
Age at presentation was 74, 63 and 23 years. Estradiol hypersecretion was observed in 3/3 patients, 17 OH progesterone was elevated in 2/3 patients and both of them had a diminution of testosterone, delta 4 androstenedione was elevated in 1/3 patients. Imaging studies suggested malignancy in 3/3 patients by the presence of necrosis, heterogeneity, calcifications, size of the tumor and compression of adjacent organs. All patients were stage III at presentation and had a Weiss score >or=6. Size and weight of the tumors were 30, 20, 15cm and 3750, 480 and 275g respectively. All 3 patients received mitotane and cortisone post-operatively and at follow up (7, 3 and 2 years) all 3 died of the disease.


CONCLUSIONS
Feminizing adreno-cortical carcinomas in adults are exceedingly rare (1-2% of adreno-cortical carcinomas). Tumors are huge and even after surgery for cure their prognosis is worse than for other varieties of adreno-cortical carcinomas either secreting or non secreting. Early diagnosis and treatment may improve overall prognosis.
Moreno
S
S
Clinique Chirurgicale, Service de Chirurgie Générale et Endocrinienne, Université de Lille, Hôpital Claude Huriez, France. sebastianmg@yahoo.com
Guillermo
M
M
Decoulx
M
M
Dewailly
D
D
Bresson
R
R
Proye
Ch
Ch
D000306:Adrenal Cortex Neoplasms / Q000097:blood / Q000601:surgery*; D000315:Adrenalectomy; D000328:Adult; D000728:Androgens / Q000097:blood; D004967:Estrogens / Q000097:blood; D005262:Feminization / Q000209:etiology*; D005640:Follicle Stimulating Hormone / Q000097:blood; D006801:Humans; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D011379:Prognosis; D013997:Time Factors; D016896:Treatment Outcome
D000728:Androgens; D004967:Estrogens; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone