Brahmachari Swagata, Pandey Anubha, Singh Mahendra Pratap, Agarwal Vandana
Department of General Surgery, All India Institute of Medical Sciences, Bhopal, IND.
Department of Pathology, Atal Bihari Vajpayee Government Medical College, Vidisha, IND.
Cureus. 2021 Aug 9;13(8):e17038. doi: 10.7759/cureus.17038. eCollection 2021 Aug.
Giant dermatofibrosarcoma protuberans (DFSP) is a very rare dermal sarcoma whose diagnosis and management are important because of the high local recurrence but low metastatic potential. Complete surgical excision of giant DFSP in a single stage is difficult but has a high cure rate. A 47-year-old man presented with a gradually increasing large (18 x 15 x 7 cm) DFSP in the epigastrium. A 3 cm circumferential wide local excision (WLE) with microscopic tumor-free margin confirmed by frozen section was performed. Immediate single staged tension-free primary closure of resultant defect was done on the principle of abdominal wall reconstruction (AWR) in ventral hernia repair. This technique of anterior component separation and bridge meshplasty is functional, avoids multiple surgeries, is cost-effective, and can be done in a resource-limited setting in developing countries. A multidisciplinary and integrated surgical approach to treat giant DFSP over epigastrium, by three-dimensional WLE and immediate AWR with anterior component separation technique (CST) and bridging meshplasty, can be of immense help in managing such rare cases in developing countries.
巨大型隆突性皮肤纤维肉瘤（DFSP）是一种非常罕见的皮肤肉瘤，由于其局部复发率高但转移潜能低，因此其诊断和治疗非常重要。一期完整手术切除巨大型DFSP很困难，但治愈率高。一名47岁男性患者，上腹部出现一个逐渐增大的巨大（18×15×7cm）DFSP。进行了3cm切缘的环周广泛局部切除（WLE），并通过冰冻切片确认切缘无肿瘤细胞。根据腹疝修补中腹壁重建（AWR）的原则，对 resulting defect 进行了一期即时无张力一期缝合。这种前侧成分分离和桥接补片成形术技术功能良好，避免了多次手术，具有成本效益，并且可以在发展中国家资源有限的环境中进行。通过三维WLE以及采用前侧成分分离技术（CST）和桥接补片成形术的即时AWR，对治疗上腹部巨大型DFSP采用多学科综合手术方法，对于在发展中国家处理此类罕见病例可能会有极大帮助。
