Congenital Dermatofibrosarcoma Protuberans (DFSP) is an extremely rare low-grade soft tissue sarcoma arising from the dermis. Accounting for less than 0.1% of all malignancies and approximately 1% of soft tissue sarcomas, congenital DFSP presents a significant diagnostic challenge due to its atypical presentation in neonates and infants. Often mimicking benign skin lesions such as hemangiomas, vascular malformations or pigmented moles, it is frequently misdiagnosed, leading to delays in appropriate treatment. This narrative review highlights the importance of early recognition and accurate diagnosis of congenital DFSP to prevent aggressive local growth and reduce the risk of recurrence. We discuss the epidemiology, clinical presentation and diagnostic hurdles associated with this condition. Histopathological examination remains the gold standard for diagnosis, with characteristic findings of spindle-shaped cells in a storiform pattern and strong CD34 positivity on immunohistochemical staining. Imaging modalities such as MRI may be helpful in assessing the extent of the lesion. Surgical excision with clear margins, preferably using Mohs micrographic surgery, is the cornerstone of treatment, balancing oncological control with functional and aesthetic considerations in paediatric patients. A multidisciplinary approach involving dermatologists, paediatric surgeons, oncologists and pathologists is essential for optimal management. We also emphasise the need for clinician vigilance, early biopsy of suspicious lesions, patient education and long-term follow-up to improve outcomes and quality of life for affected children.

Superficial acral fibromyxoma (SAF) is a benign fibromyxoid tumor that presents as a slow-growing nodule on the subungual and periungual areas of the hands and feet. Superficial acral fibromyxoma needs to be better documented in the literature, and thus, increased research and education are required to aid clinicians in recognizing its appearance. To review the current literature on superficial acral fibromyxoma and present its epidemiology, pathogenesis, clinical presentation, diagnosis, treatment, and common differential diagnosis. A literature review was performed on Pubmed and Scopus for case reports, case series, clinical studies, and reviews that described epidemiology, clinical presentation, pathogenesis, histological examination, and treatment of superficial acral fibromyxoma. The gathered literature was analyzed to extract concise information on superficial acral fibromyxoma. Results: Superficial acral fibromyxoma is associated with Rb1 gene deletion and grows as a 0.5 to 5.0-centimeter gelatinous-to-firm nodular mass on periungual or subungual regions of middle-aged males. It is also noted to disrupt nail architecture and cause lytic or erosive lesions of bone. Histopathologically, superficial acral fibromyxoma comprises stellate and spindle cells surrounded by a myxo-collagenous matrix and is strongly positive for CD34, CD99, nestin, and vimentin. Complete surgical excision is the first-line treatment for superficial acral fibromyxoma; however, the tumor has a post-operative recurrence rate of 20-25%. Mohs surgery has been attempted to decrease the rate of recurrence. After excluding other cutaneous neoplasms, superficial acral fibromyxoma should be considered in patients with a periungual or subungual slow-growing mass and other soft tissue. Complete surgical excision can lead to recurrence; therefore, patient follow-up is necessary.