We report a case of asynchronous occurrence of bilateral
adrenocortical adenoma in a 13-yr-old girl with
Beckwith-Wiedemann syndrome. A right virilizing adrenal
adenoma was surgically removed at age 6, following clinical manifestation of
virilization such as
acne, voice change, clitoris
hypertrophy and overgrowth. Histopathological examination of the resected specimen revealed an
adrenocortical adenoma predominantly composed of eosinophilic
tumor cells expressing all the steroidogenic
enzymes. High serum levels of
DHEA-S (6,380 ng/ml) and
testosterone (547 ng/dl) were noted prior to the operation. Postoperative course was unremarkable. Menstruation started at age 11, with a regular interval. At the age of 13 yr old, a high serum level of
DHEA-S (8,250 ng/ml) was detected. In contrast to the episode of
virilization at age 6, however, the serum
testosterone level was not so high (122 ng/dl), and no clinical symptoms of
virilization were apparent. Abdominal ultrasonography demonstrated the presence of a left
adrenocortical adenoma. Pathological examination of the resected specimen revealed a circumscribed and well encapsulated
tumor with essentially the same histological features as the
tumor previously removed, except that the
tumor cells showed a more prominent morphological similarity to the fetal adrenal cortex and did not express 3β HSD. The absence of
virilization at the second episode was due to the relatively low serum level of
testosterone compared with that of
DHEA-S.