Aggressive giant-cell tumor in a young female with congenital adrenal virilism (adrenogenital syndrome).Report of an unusual association of a bone neoplasm with an endocrine disorder

The first described association of an aggressive giant-cell tumor of the tibia is reported in a young female with congenital adrenal virilism (adrenogenital syndrome) who was followed for 24 years. The adrenogenital syndrome is probably transmitted by a single autosomal recessive gene and is characterized by an enzyme defect with abnormal growth, virilism, and high levels of circulating androgenic steroids. The giant-cell tumor is an epiphyseal lesion which is relatively uncommon. The 2 conditions may be coincidental, but their association is noteworthy. Unlike Albright's syndrome in which precocious virilism is associated with a relatively benign process, fibrous dysplasia, the bony lesion associated in this case was a giant-cell tumor which appeared early in life and was more aggressive than usual. It is possible that high levels of adrenal androgenic steroids may have been responsible for stimulating such a lesion or hastened its appearance by accelerating growth and maturation in the epiphysis.