Dupuytren's disease in children-differential diagnosis

Abstract

Background/purpose: Dupuytren's disease in children is uncommon; only a few histologically confirmed diagnoses are found in literature. In its early, proliferative phase with many fibroblasts, it can resemble the early stadium of an epitheloid sarcoma, which also is a rare tumor. The purpose of this report is to show pediatric surgeons and pathologists the importance of differential diagnosis of nodules and fibrotic bands in children's hands, especially to exclude a malignancy.

Methods: Between 1998 and 2000, 3 children at ages of 2(1/2) years, 9 years, and 10 years, respectively, presented with the clinical signs of Dupuytren's disease of the hand. Each of them had fibrous bands with a flexion contracture of the fingers. All of them underwent fasciectomy.

Results: In patient 1, a 10 degrees extension lag of the index finger resulted after 3 operations. Histology findings showed the typical features of Dupuytren's disease. In patient 2, histology findings showed a recurring digital fibroma of childhood. After consultation with the pathologist and reevaluation of the slides, the histologic diagnosis was corrected to Dupuytren's disease. Full extension and thumb abduction has been achieved. In patient 3, histology of the first and second operation was misdiagnosed as Dupuytren's disease. In the third operation 2(1/2) years later, the histologic diagnosis of epitheloid sarcoma was made. Amputation of the forearm was necessary.

Conclusions: Exact anamnesis, location of the lesion, and suspicious diagnosis must be mentioned to the pathologist in the case of biopsy or excision of Dupuytrenlike lesions in children's hands. Awareness of the possibility of the epitheloid sarcoma may help prevent misdiagnosis.