In recent days, we have encountered several cases of augmentation dermatofibrosarcoma, and sadly, all of them were treated as keloids several times in other hospitals and came to the clinic because they were ineffective.
There are some similarities between aneurysmal dermatofibrosarcoma and keloid, but the etiology, diagnosis, and treatment are very different. Some of the knowledge of dermatofibrosarcoma is described below: Dermatofibrosarcoma of the bulge (DSFP) is common in middle-aged men and is most likely to occur on the trunk. The lesion is a raised dark red mass, hard, lobulated and adherent to the skin. It grows slowly, is usually asymptomatic, and is prone to recurrence if excision is incomplete, but rarely metastasizes. The disease is a limited, low-grade malignant fibrosarcoma of fibroblastic or histiocytic origin that originates in the dermis and may extend to the subcutaneous tissue. The origin of the tumor is currently unknown.
Clinical manifestations: Patients can develop at any age, most commonly in middle age, and rarely in children, with a male predominance. The tumor can occur in any part of the body, but mostly in the trunk and extremities, especially in the anterior chest, more ventral than dorsal, more proximal than distal, less in the head, face, neck, and palmoplantar. 10%-20% of patients complain of a history of trauma before the onset of the disease. The disease progresses slowly and the tumor appears as a raised hard mass, starting as a hard plaque, skin color or dark red, with a slightly concave skin surface resembling atrophy, while the skin around the tumor is light blue-red, and later appears as a light red, dark red or purple-blue single nodule or adjacent multiple nodules of different sizes, with a bulging appearance, size from 0.5 to 2 cm, and can suddenly accelerate growth and surface rupture. A small number of tumors have a punctate pigmentation and are called pigmented dermatofibrosarcoma or Bednar’s tumor. Pain is evident as the tumor increases in size. The disease is locally aggressive and occasionally widely disseminated, but metastases are rare. The disease can last up to 50 years. In addition to the raised surface, the tumor may grow aggressively and invade the subcutaneous tissue. Local recurrence can occur if excision is not complete. Although metastases to the lungs, abdomen, brain, bone, or nearby lymph nodes are also seen, they are uncommon and only occur in advanced stages, often as a result of multiple local recurrences.
Diagnosis: The disease can be presumed to be present when there is clinically elevated, rigid fibrous damage with slow growth and surface skin atrophy, and the diagnosis is confirmed by finding dense fibroblasts arranged in a wheel-like structure in the pathology. Histological features and clinical data are the main basis, and immunohistochemical examination helps in differential diagnosis. In immunohistochemical staining, DFSP tumor cells show a strong and diffuse positive reaction for wave proteins; CD34 generally shows a strong and diffuse positive reaction, with a positive rate of 72% to 92%. Lysozyme is focally positive; smooth muscle actin (SMA) is expressed in DFSP at a rate of 50% to 95%, but its expression is often unstable and often focal.
Treatment: Because local invasive growth can occur, it should be excised more extensively. The resection margin should include a relatively wide area of normal skin, and the surgical margin should be 3.0 cm-4.0 cm from the outside of the tumor area, and deep fasciotomy can reduce the recurrence rate. The recurrence rate can be 11%-50% in patients with extensive resection. Mohs microsurgical resection is also feasible, with a recurrence rate of 2%. If the tumor is large, implantation is required and radiation therapy is not effective. In case of recurrent cases, more extensive resection is required. Metastasis can occur after multiple postoperative recurrences, but is rare.