Dimas Dionysios, Boutas Ioannis, Potiris Anastasios, Koufopoulos Nektarios, Balalis Dimitrios, Sitara Kyparissia, Danglis Fotios, Misitzis Ioannis
Breast Unit, Athens Medical Center, Psychiko Clinic, Athens 11525, Greece.
Third Department of Obstetrics and Gynecology, Attikon University Hospital, National and Kapodistrian University of Athens, Athens 12462, Greece.
Mol Clin Oncol. 2021 Mar;14(3):50. doi: 10.3892/mco.2021.2212. Epub 2021 Jan 20.
Dermatofibrosarcoma protuberans (DFSP) is a superficial mesenchymal neoplasm that originates from the dermal fibroblasts and tends to be locally aggressive. Although infrequent, it is one of the most common cutaneous sarcomas. It mainly affects young and middle-aged patients 20 to 50 years old. Any area of skin may be involved, but the most common sites of presentation are the trunk and extremities. DFSP of the breast is extremely rare. It classically presents as a nodular, exophytic, cutaneous mass, though initially it can manifest as a flat plague and can show persistent but slow growth for many years. Due to increased risk of local recurrence, the standard of care for localized disease is surgical excision with adequate margins. Wide local excision is the most common technique used, but as an alternative Mohs micrographic surgery has emerged as a procedure that offers lower local recurrence rates. Metastases are rare but have been previously reported. In such patients, treatment with imatinib or radiotherapy can be considered. The current case presents a 52-year-old lady with DFSP of the breast that was successfully managed by the Breast Unit of Athens Medical Center-Psychiko Clinic.
隆突性皮肤纤维肉瘤（DFSP）是一种起源于真皮成纤维细胞的浅表间叶性肿瘤，具有局部侵袭性。虽然发病率不高，但它是最常见的皮肤肉瘤之一。主要影响20至50岁的中青年患者。皮肤的任何部位都可能受累，但最常见的发病部位是躯干和四肢。乳腺DFSP极为罕见。典型表现为结节状、外生性皮肤肿物，不过起初可能表现为扁平斑块，且多年来可呈持续但缓慢生长。由于局部复发风险增加，局限性疾病的标准治疗方法是进行切缘足够的手术切除。广泛局部切除是最常用的技术，但作为一种替代方法，莫氏显微外科手术已成为一种局部复发率较低的手术。转移罕见，但此前已有报道。对于此类患者，可考虑使用伊马替尼治疗或放疗。本病例介绍了一名52岁女性，患有乳腺DFSP，由雅典医学中心-普西基科诊所乳腺科成功治疗。
