Dermatofibrosarcoma protuberans and the Bednar tumor: Treatment in the pediatric population

• Published in: Journal of pediatric surgery Vol. 33; no. 12; pp. 1811 - 1814
• Main Authors: Marcus, Jeffrey R, Few, Julius W, Senger, Christof, Reynolds, Marleta
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01.12.1998; Elsevier
• Subjects: Biological and medical sciences; Dermatofibrosarcoma - congenital; Dermatofibrosarcoma - pathology; Dermatofibrosarcoma - surgery; Female; Humans; Infant; Medical sciences; Miscellaneous; Skin Neoplasms - congenital; Skin Neoplasms - pathology; Skin Neoplasms - surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; treatment; Bednar tumor; Dermatofibrosarcoma protuberans; Human; Skin disease; Relapse; Sarcoma; Bibliographic survey; Surgical resection; Malignant tumor; Case study; Pathology; Histopathology; Treatment; Adult; Female; Diagnosis; Child
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/S0022-3468(98)90292-5

Dermatofibrosarcoma protuberans (DFSP) is a low-intermediate-grade cutaneous sarcoma that has a marked propensity for local recurrence after excision. The Bednar variant of this tumor is even less common and is distinguished histologically by the dispersal of melanin containing cells in an otherwise typical DFSP. Both are considered to be tumors of the third and forth decades of life, but both DFSP and the Bednar variant have been described in children. Until this report of a congenital Bednar tumor, only the DFSP has also been described in the neonate. The histopathology and surgical management of DFSP and Bednar tumors are out-lined with emphasis on reported experience in the pediatric population. The surgical management of these lesions in children is based on numerous series in adults and the limited pediatric experience. The recommended treatment is wide excision with 3-cm margins of visibly uninvolved tissue and inclusion of superficial fascia.