Darier-ferrand Dermatofibrorsarcoma

SavageGlow · #1 ● 11-06-2024, 05:07 PM

We present the case of a 60-year-old Tunisian man with a medical history of recurrent abdominopelvic DFS for 35 years, for which he had undergone ten surgical excisions in another clinic. Not all the histologic reports were available, but for those that were found, the margins were free of tumor. He presented with a giant DFS of the abdominal wall and pelvis. The biopsy confirmed the diagnosis and the patient was referred to our outpatient clinic.

The patient had a poor general state; he was afebrile and hemodynamically stable. On physical examination, multiple tumors were observed extending from the abdominal wall to the pelvis and external genitals a distance of 1 cm from the anal margin (Figs. 1, 2, 3).

A computed tomography scan of the abdomen and pelvis revealed multiple subcutaneous and infiltrative tumor masses of the anterior abdominal wall and pelvis that invaded the right scrotum. No metastases were found. After discussion in a multidisciplinary meeting, we opted for wide local excision combined with emasculation and reconstruction with a local skin flap for the pelvis and thin cutaneous graft for the abdominal wall (Figs. 4, 5, 6).

The surgery lasted 240 minutes. No preoperative complications or blood loss occurred. The excised specimen measured approximately 33 cm × 17 cm × 7 cm in the greatest dimensions. The postoperative course was uneventful. The patient was discharged on day 7. The final histology report showed clear margins.

The case was discussed again in a multidisciplinary meeting and the decision was made for close follow-up every 3 months for the first 2 years. The patient progressed well during 7 months of follow-up. No urinary problems were reported. The only concern was an episode of depression, which was followed up by a psychiatrist.