Dermatofibrosarcoma Protuberans in a Child: A Case Report

Background. Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade soft tissue neoplasm originating from the dermal layer of the skin. It usually occurs in adults; however, it can rarely be seen in infancy and childhood. Diagnosis of DFSP in children is quite difficult-given-rarity of this lesion, its variegated appearance, and its presentation sometimes at unusual sites. Case. We present the case of five-year-old boy who came with painless lesion on a forehead. Fine needle aspiration cytology (FNAC) suggested possibility of mesenchymal neoplasm. Patient was advised excision biopsy. Final diagnosis of DFSP was made based on histopathological findings. The patient was then advised reexcision surgery with wide margins. The patient was lost to followup and later turned up after two months with recurrence of a similar swelling at the same site. Conclusion. DFSP in children is rare and difficult to diagnose. Treatment of childhood DFSP is often delayed leading to incomplete excision. Hence, there is need to recognize and appropriately manage this uncommon childhood neoplasm. 1. Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, intermediate grade neoplasm originating from the dermal layer of the skin [1]. It is most often found on the trunk and proximal limbs and is rare on the head and neck. It commonly favors young to middle aged adults. However, it can also occur in infancy and childhood. Because of the rarity of the tumor and its variegated appearance, diagnosis of DFSP in children is quite difficult. We are presenting this case to document the uncommon clinical presentation of DFSP in pediatric age group. Key Message DFSP in children is a rare and difficult diagnosis particularly when presenting at uncommon site like head and neck. Treatment of childhood DFSP is often delayed because of misdiagnosis leading to local growth and incomplete excision. Hence, pediatricians should be aware of this uncommon entity and should ensure wide excision and reduce risk of recurrence. 2. Case Report We present a case of a five-year-old boy who presented with a slightly raised, painless lesion on base of the nose near the medial side of a the left eyebrow (Figure 1) since 6 months. On examination, there was nontender, firm nodule, measuring 0.5？cm in diameter. A fine needle aspiration cytology (FNAC) was performed with 23？G needle, and provisional diagnosis of mesenchymal neoplasm was rendered. The patient was advised excision biopsy for final diagnosis and behavior of neoplasm. Subsequently an excision biopsy was performed, and final