Dedifferentiated chondrosarcoma in Albright syndrome. A case report and review of the literature.

Abstract

Fibrous dysplasia is a developmental condition in which the skeleton fails to mature normally. Albright syndrome, which was first reported in 1937 by McCune and Bruch23 and by Albright1, is characterized by polyostotic fibrous dysplasia, endocrine disorders, and brown patches on the skin ( cafe-au-lait spots)3,17,25. It is seen more often in female than in male patients35. Malignant transformation in fibrous dysplasia was noted, in 1945, by Coley and Stewart5, and since then it has been reported in more than 100 patients12,19,33,43. The most common secondary malignant lesion in fibrous dysplasia is osteosarcoma, followed by fibrosarcoma and chondrosarcoma6,9,15,18,33,42. The prevalence of malignant transformation in fibrous dysplasia is only 0.4 per cent (six of 1517 patients)36. However, the prevalence of malignant transformation in Albright syndrome is 4 per cent (four of 100 patients36), which is higher than that in other forms of fibrous dysplasia36. To our knowledge, we are the first to report the case of a patient who had a dedifferentiated chondrosarcoma secondary to Albright syndrome. A fifty-two-year-old man who had had polyostotic fibrous dysplasia and many cafe-au-lait spots since he was an infant was seen at our hospital because of mild pain and increased swelling of the left knee. There was no family history of bone tumors, skin pigmentation, or precocious puberty. During childhood, he had sustained multiple fractures of the long bones of the lower limbs. When he was five years old, acne, growth of pubic hair, and an increase in the size of the penis and testes were observed. During adolescence and adulthood, more fractures of the lower limbs occurred. …