Surgical Excision of Pulmonary Metastases of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare malignancy of mesenchymal origin of medium-low grade with a tendency to local recurrences but not to distant metastases. We present the case of a 37-year-old male who underwent surgical resection of a 1.2 cm DFSP lesion on the left shoulder in May 2020. In the absence of a standardized follow-up protocol for DFSP, the attending physician opted for ultrasound monitoring every six months to detect any local recurrences. Due to chest pain and mild exertional dyspnea, a CT scan was performed three years post-excision revealed a 10 cm mass in the left lower lung lobe and two lesions in the right lung measuring 3.2 cm and 1.2 cm, respectively. Thoracotomy was performed in July 2023 to remove the large lesion in the left lower lobe, necessitating intrapericardial resection of the left lower pulmonary vein and extra-pleural dissection due to parietal pleural infiltration. Uniportal-video-assisted thoracoscopic surgery (U-VATS) was performed one month later to resect the lesions on the right side. Pathological examination showed high mitotic activity, cellularity, and nuclear pleomorphism, in the absence of other malignant mesenchymal neoplasms, suggesting fibrosarcomatous transformation of DFSP in the metastatic lesions (FS-DFSP). The patient remains disease-free under close radiological and clinical surveillance. Given the potential for aggressive transformation and the risk of recurrence and distant metastasis, our experience suggests including chest CT scans in the follow-up algorithms for DFSP.