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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
Author(s) -
Burman Michael S.,
Gardner Richard C.,
Lauter Carl B.
Publication year - 1970
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197005)25:5<1174::aid-cncr2820250523>3.0.co;2-h
Subject(s) - adrenogenital syndrome , medicine , lesion , epiphysis , endocrine system , precocious puberty , dysplasia , endocrinology , pathology , anatomy , hormone
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.