Dermatofibrosarcoma Protuberans: The Current State of Multidisciplinary Management

Area of Science:

• Oncology
• Dermatology
• Pathology

Background:

• Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative soft tissue tumor with high local recurrence rates.
• DFSP typically presents as a slow-growing, painless skin lesion on the trunk or arms.
• Accurate diagnosis requires distinguishing DFSP from benign dermatofibroma via immunohistochemistry.

Purpose of the Study:

• To review the characteristics, diagnosis, and management of DFSP.
• To explore emerging theranostic targets for DFSP, specifically folate hydrolase-1 (FOLH1).

Main Methods:

• Review of DFSP literature regarding presentation, diagnosis, and treatment.
• Discussion of immunohistochemical markers for DFSP diagnosis (CD34, factor XIIIa, etc.).
• Exploration of FOLH1 expression in DFSP and its potential theranostic applications.

Main Results:

• DFSP requires a multidisciplinary approach, often involving surgical excision followed by adjuvant therapy due to high recurrence.
• Lifelong surveillance is necessary for DFSP recurrence, with most occurring within 3 years.
• FOLH1 expression in benign dermatofibromas and sarcomas suggests potential for FOLH1-targeted surveillance and therapy in DFSP.

Conclusions:

• DFSP management necessitates early diagnosis, appropriate surgical techniques, and consideration of adjuvant therapies.
• FOLH1 represents a promising theranostic target for DFSP, potentially enabling novel imaging and treatment strategies.