Congenital Dermatofibrosarcoma Protuberans Treated with Wide Excision / 대한피부과학회지

Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor with intermediate malignancy that usually appears in adults. The congenital type is very rare, and only three cases have been reported so far in the Korean literature. A 2-month-old girl presented with a firm reddish-gray nodule on the middle of her back that had been present since birth. Histopathologically, the lesion was composed of fascicles of densely packed uniform spindle cells arranged in a storiform (mat-like) pattern. The cells had hyperchromatic nuclei and rare mitotic figures. On immunohisto-chemistry, the tumor cells were positive for CD34. Based on these findings, we made the diagnosis of congenital dermatofibrosarcoma protuberans. She underwent wide excision, and there was no evidence of recurrence during 24 months of follow-up. Our report is of interest because of the rarity of congenital dermatofibrosarcoma protuberans and its earliest detection reported in Korea.

A Case of Congenital Dermatofibrosarcoma Protuberans / 대한피부과학회지

Dermatofibrosarcoma protuberans (DFSP) is a tumor of intermediate malignancy that has long been considered to almost exclusively affect adults. Congenital DFSP is extremely rare and the diagnosis is often difficult because of the deceptive appearance of the lesions. A 12-month-old infant developed an asymptomatic red indurated nodule on the lower aspect of his back. The brownish macule had appeared at birth and the lesion grew rapidly 6 months later. Histologic examination showed spindle shaped cells with a storiform pattern and the cells were positive for CD34 stain. A diagnosis of congenital DFSP was made and the mass was removed by wide local excision. There was no evidence of recurrence at follow-up 8 months later.

A Case of Congenital Dermatofibrosarcoma Protuberans with a Histologic Feature of Bednar Tumor / 대한피부과학회지

Dermatofibrosarcoma protuberans(DFSP) is a cutaneous mesenchymal neoplasm originating in the dermis, which is diagnosed most in adulthood, with a range in age from birth to the ninth decade. Congenital DFSP is extremely rare and the diagnosis is difficult because of its resemblance to vascular birth mark of skin atrophy. We describe a case of congenital DFSP in a 19-year-old female on the left forearm, which was present as a vascular-appearing macule from birth with recent nodular change. Interestingly, the histopatholgy revealed Bednar tumor, a pigmented DFSP and it has not been reported in congenital case yet. The patient was treated with a wide and deep excision with surgical margin of 4cm and STSG, and no recurrence was noted for one year.