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Congenital dermatofibrosarcoma protuberans: 30 years of follow-up
Esther von Stebut1, Wolfgang Brsuninger
1Department of Dermatology, Johannes Gutenberg University, Mainz, Germany. vonstebut@mail.uni-mainz.de
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor that commonly appears in adult patients. Few cases of DFSP in childhood have been reported. We describe a case of congenital DFSP that had been incompletely excised in childhood, and again at 28 years of age. The 33-year-old woman presented with a recurrence. When comparing the histologic features of the previous specimens excised in 1970 and 30 years later, the similar typical storiform pattern of fibromatous cells was found. The small number of proliferation-marker-positive cells was not altered over the course of 30 years. In line with a few additional reports, we suggest that congenital dermatofibrosarcoma protuberans is a tumor with a low malignant potential.
Insights
Congenital dermatofibrosarcoma protuberans (DFSP) is a rare tumor. This case suggests congenital DFSP has a low malignant potential, even after multiple incomplete excisions over 30 years.
Area of Science:
- Oncology
- Dermatopathology
- Pediatric Oncology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer.
- DFSP typically affects adults, with rare childhood occurrences.
- Congenital DFSP is exceptionally uncommon.
Observation:
- A case of congenital DFSP initially excised in childhood and later at age 28 is presented.
- The patient, a 33-year-old woman, experienced a recurrence.
- Histological analysis revealed consistent storiform patterns and stable proliferation markers over 30 years.
Findings:
- Congenital DFSP exhibits a low malignant potential.
- Histological features remained consistent over three decades.
- Proliferation markers showed minimal change, indicating slow tumor growth.
Implications:
- Congenital DFSP may require long-term monitoring due to potential for recurrence.
- Understanding the low malignant potential informs treatment strategies.
- Further research into congenital DFSP is warranted.
