An unusual case of dermatofibrosarcoma protuberans misdiagnosed as vascular malformation for over 30 years: A case report

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a rare and slowly growing soft tissue tumor and it is frequently misdiagnosed and mismanaged like more common masses. Therefore diagnostic delays are common and may result in challenging reconstructions. Case presentation: We report the peculiar case of a 36-year-old patient with dermatofibrosarcoma protuberans of the right iliac fossa misdiagnosed as vascular malformation for over 30 years. Due to the delayed diagnosis resulting in a large tumor to be resected, surgical reconstruction was performed with a miniabdominoplasty approach with an excellent cosmetic and functional result. Discussion: The review of the literature showed that mismanagements and delayed diagnosis of this sarcoma are frequent. Large skin and soft-tissue defects are frequently encountered in the surgical treatment of this tumor, and adequate knowledge of the reconstructive options is mandatory to provide the best possible outcome. Conclusions: Superficial skin masses could be easily misdiagnosed. These diagnostic delays may lead to increased patient morbidity and more challenging reconstructive procedures. In this scenario, preoperative biopsies of suspicious lesions may be useful to avoid mismanagement of rare malignant neoplasms such as DFSP. In some challenging cases, the use of a surgical approach typical of cosmetic procedures may be useful to obtain satisfactory aesthetic and functional results.