Dermatofibrosarcoma protuberans in Child
DFSP typically presents as a slow-growing, red-brown, indurated plaque with irregular nodularity in an adult. The most common site is the trunk, followed by the proximal extremities, head, and neck. In children, DFSP can appear atrophic, hypopigmented, or blue-red in color.
DFSP is an uncommon neoplasm with low metastatic potential, carrying a 2%-5% risk of distant metastasis. However, it can be locally aggressive and has a high rate of recurrence after surgical excision. Poor prognostic factors include older age, male sex, and larger tumor size. Approximately 9%-20% of tumors can undergo fibrosarcomatous degeneration.
Related topic: giant cell fibroblastoma
C44.99 – Other specified malignant neoplasm of skin, unspecified
276799004 – Dermatofibrosarcoma protuberans
Differential Diagnosis & Pitfalls