Theriot Ty, Haas Christopher
Department of Dermatology, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA.
Case Rep Dermatol Med. 2025 Mar 14;2025:6640596. doi: 10.1155/crdm/6640596. eCollection 2025.
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue tumor affecting the dermis and subcutaneous tissues, with potential involvement of muscle and fascia. This case report describes a 68-year-old Caucasian male with a history of recurrent DFSP on the left cheek, initially excised 36 years ago, with multiple recurrences despite wide local excisions (WLEs), eventually requiring left orbital enucleation, presenting to the clinic with a 10-year history of a slow-growing lesion on the left temporal scalp. Examination revealed a 2 cm flesh-colored, firm nodule, which biopsy confirmed as DFSP. Despite two subsequent WLEs, positive margins persisted. The patient refused further surgical intervention and was referred for imatinib and radiation therapy, which he also declined. MRI revealed additional nodules near the left zygomatic arch and sternocleidomastoid. DFSP is diagnosed via biopsy, often confirmed with CD34 immunohistochemistry. Optimal treatment is Mohs micrographic surgery (MMS), but WLE is also used. The recurrence rate is high, especially in head and neck locations. This case underscores the necessity for multidisciplinary management and highlights the critical role of thorough physical and histopathologic examinations. Close clinical follow-up is essential due to the high recurrence risk within the first three years post-treatment. This report emphasizes the importance of early detection and comprehensive care strategies to manage DFSP effectively.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的、生长缓慢的软组织肿瘤,累及真皮和皮下组织,可能累及肌肉和筋膜。本病例报告描述了一名68岁的白种男性,有左侧脸颊复发性DFSP病史,最初于36年前切除,尽管进行了广泛局部切除(WLE)仍多次复发,最终需要进行左侧眼眶摘除术,现因左侧颞部头皮有一个生长缓慢的病灶长达10年而就诊于诊所。检查发现一个2厘米大小的肉色、质地坚硬的结节,活检证实为DFSP。尽管随后又进行了两次WLE,但切缘仍为阳性。患者拒绝进一步手术干预,并被转诊接受伊马替尼和放射治疗,他也拒绝了。MRI显示左侧颧弓和胸锁乳突肌附近还有其他结节。DFSP通过活检诊断,常通过CD34免疫组化确诊。最佳治疗方法是莫氏显微外科手术(MMS),但也使用WLE。复发率很高,尤其是在头颈部位置。本病例强调了多学科管理的必要性,并突出了全面体格检查和组织病理学检查的关键作用。由于治疗后前三年复发风险高,密切的临床随访至关重要。本报告强调了早期发现和综合护理策略对有效管理DFSP的重要性。