Ahmed Mohamed Badie, Al-Mohannadi Fatima Saoud, Ahmed Abdelrahman Badie, Althalathini Mahmoud, Alsherawi Abeer
Plastic Surgery Department, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.
College of Medicine, QU Health, Qatar University, Doha, Qatar.
Case Rep Oncol. 2024 Jan 23;17(1):128-134. doi: 10.1159/000536062. eCollection 2024 Jan-Dec.
Dermatofibrosarcoma protuberans (DFSP) originated as keloid sarcoma, gaining its current designation in 1925. DFSP exhibits slow growth, categorizing it as a low- to intermediate-grade malignant sarcoma. Initially presenting as a small, firm, irregular skin nodule, it undergoes sudden, rapid growth, forming a prominent mass. While locally aggressive, distant metastasis is rare. DFSP affects mainly the torso then proximal extremities.
In this case study, we described a 57-year-old male individual who presented with a chest midline swelling that was progressing in size. A punch biopsy showed inconclusive results. Thus, a wide local excision was carried out along with sending the initial biopsy slides to Mayo Clinic for second opinion. A diagnosis of DFSP was confirmed, which is an uncommon and locally aggressive tumor affecting soft tissues. The primary histological diagnosis relies on immunohistochemical stains, enabling the distinction between DFSP and other fibrous tumors.
Diagnosing DFSP is challenging due to its similarity to other skin lesions. A multidisciplinary approach is vital for accurate diagnosis and management.
隆突性皮肤纤维肉瘤（DFSP）最初被认定为瘢痕疙瘩肉瘤，于1925年获得了现在的命名。DFSP生长缓慢，属于低至中等级别的恶性肉瘤。它最初表现为一个小的、坚硬的、不规则的皮肤结节，随后会突然迅速生长，形成一个突出的肿块。虽然它具有局部侵袭性，但远处转移很少见。DFSP主要影响躯干，其次是近端肢体。
在本病例研究中，我们描述了一名57岁男性，其胸部中线出现肿胀，且肿胀大小不断增大。穿刺活检结果不明确。因此，进行了广泛的局部切除，并将最初的活检切片送往梅奥诊所寻求二次诊断意见。确诊为DFSP，这是一种罕见的、影响软组织的局部侵袭性肿瘤。主要的组织学诊断依赖免疫组化染色，以此来区分DFSP与其他纤维性肿瘤。
由于DFSP与其他皮肤病变相似，因此诊断具有挑战性。多学科方法对于准确诊断和管理至关重要。
