Dermatofibrosarcoma Protuberans - Cutting through the Complexity

Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade malignant soft tissue sarcoma, representing less than 0.1% of all malignant neoplasms and 1% of soft tissue sarcomas. It generally manifests in the third and fourth decades of life, with a greater prevalence in males. DFSP originates from dermal fibroblasts and is distinguished by gradual, infiltrative proliferation, local recurrence, and a minimal propensity for metastasis. This case report describes a 17-year-old female with a two-year history of a rapidly growing nodular mass on the anterior chest wall. Diagnostic assessments, comprising MRI, FNAC, and histology, validated the diagnosis. The patient had a broad local excision with negative margins, and immunohistochemistry indicated CD34 positivity. The postoperative follow-up indicated no recurrence. Timely identification, interdisciplinary care, and prolonged surveillance are crucial to enhance outcomes and reduce recurrence risk in DFSP cases.