Han Anne, Chen Elbert H, Niedt George, Sherman William, Ratner Désirée
Department of Dermatology, Mount Sinai School of Medicine, New York, New York, USA.
Arch Dermatol. 2009 Jul;145(7):792-6. doi: 10.1001/archdermatol.2009.140.
Dermatofibrosarcoma protuberans (DFSP) is an unusual soft-tissue tumor with a propensity for subclinical extension and local recurrence. Surgical excision, even with tissue-sparing techniques, may cause significant deformity or disability because of the infiltrative nature of DFSP. In this study, we evaluate retrospective data obtained from 4 patients with locally advanced or recurrent DFSP who received neoadjuvant imatinib mesylate therapy before undergoing Mohs micrographic surgery.
Patients treated with neoadjuvant imatinib therapy had an average tumor size reduction of 36.9%. This clinical response was paralleled by histopathologic changes, including decreased cellularity in 100% of the total area as well as significant hyalinization. Imatinib therapy for DFSP before Mohs micrographic surgery was associated with 100% local control at a maximum follow-up of 4 years.
Neoadjuvant imatinib therapy is a well-tolerated, novel approach to DFSP that reduces tumor burden and facilitates resection. Larger prospective studies are needed to confirm and expand on these results.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的软组织肿瘤，具有亚临床扩展和局部复发的倾向。由于DFSP的浸润性，即使采用保留组织的技术进行手术切除，也可能导致明显的畸形或残疾。在本研究中，我们评估了4例局部晚期或复发性DFSP患者在接受莫氏显微外科手术前接受新辅助甲磺酸伊马替尼治疗的回顾性数据。
接受新辅助伊马替尼治疗的患者肿瘤平均缩小36.9%。这种临床反应与组织病理学变化平行，包括总面积的100%细胞密度降低以及明显的玻璃样变。在莫氏显微外科手术前对DFSP进行伊马替尼治疗，在最长4年的随访中局部控制率为100%。
新辅助伊马替尼治疗是一种耐受性良好的新型DFSP治疗方法，可减轻肿瘤负担并便于切除。需要更大规模的前瞻性研究来证实并扩展这些结果。
