【摘要】 目的　进一步提高对隆凸性皮肤纤维肉瘤 (DFSP)的认识 ,强调正确和规范化手术的重要性 ,并探讨综合治疗途径。方法　对 1985年 1月至 2 0 0 2年 9月收治的 16 3例DFSP病例 ,进行临床和病理资料的回顾性分析。结果　 16 3例患者中有 15 0例 (占 92 0 % )曾被误诊为良性肿瘤而行局部切除手术 ,经补充广泛切除、病理检查最终明确局部有肿瘤残留者 6 9例 ,占 4 6 0 % ,其中 4 9例 (占71 0 % )在体检或B超检查时未能发现残留灶。局部切除术后 ,肿瘤复发率可达 4 5 1% ,明显高于广泛切除术后复发率 (5 6 % )。在施行广泛切除手术的 14 2例患者中 ,有 99例皮肤切缘≥ 3cm ,术后局部复发 5例 (5 1% ) ,36例皮肤切缘在 1～ 2cm ,术后复发 3例 (8 3% )。 4 6例 (32 4 % )行游离植皮 ,11例行筋膜皮瓣转移 ,1例行缺损区涤纶修补。术后的并发症主要是移植皮瓣的坏死 (2 0例 )和切口感染 (6例 ) ,但术后 2个月内均能治愈。有 17例患者因为曾经多次复发或广泛切除术后病理报告明确切缘或基底仍有肿瘤细胞残留而补充放射治疗 ,放射量 32 75～ 70 0 0cGy。放疗后有 1例患者出现湿性蜕皮 ,局部复发 2例。在全部病例中 ,只有 2例 (1 2 % )死亡 ,其中 1例死于肺、肝转移 ;另有 2例曾出现区域淋巴结转移 ,经手术治疗后?更多还原

【Abstract】 Objective To emphasize the importance of correct and standardized surgical treatment on dematofibrosarcoma protuberans ( DFSP ), and discuss the suitable synthesized therapy on it.Methods 163 cases of DFSP, which were treated between January 1985 and September 2002,were submitted to a retrospective study.Results Among the 163 cases, 150 (92 0%) were treated with local excision as benign tumors before accepted to Cancer Hospital, Fudan University. 69 cases (46 0%) were approved by pathological examination to have tumor remnants after they were treated with wide excision, and 49 (71 0%) of them couldn’t be found to have any tumor remnants by physical examination or B ultrasonic examination before that operation. It was easy for the tumor to recur after excision, especially the local excision. The recurrent rate after it was 45.1%, which was much higher than the one after wide excision (5 6%). Among the 142 cases which wide excision were performed, 99 ones had excision margins ≥3cm and 5 of them (5 1%) developed local recurrence while 36 ones had excision margins 1~2 cm and 3 of them (8 3%) developed local failure. 46 cases (32 4%) were given skin graft, 11 cases were given flap, and 1 case had dacron mending in skin defection area. The main complications after these operations were necrosis of the skin flap (20 cases) and infection of the wound (6 cases). They could all be cured in 2 months. 17 cases were given complimentary radiotherapy with the dose range from 3275 cGy to 7000 cGy because of their recurrences for times or positive resection margins after wide excision. Only one case had wet molting after radiotherapy and 2 developed local recurrence. Among all the 163 cases, only 2 (1 2%) were dead, and 1 of them was died of metastasis of lung and liver. 2 cases got lymph node metastasis, then were given surgical treatment and still alive now. 13 cases (8 0%) were DFSP FS with their malignancies increased. 11 of them were the recurrent ones after local excision or wide excision.Conclusions In order to avoid misdiagnosis, it is necessary for the clinician to know much about DFSP. Once the tumor was diagnosed of DFSP after local excision, it is necessary to take wide excision. Because DFSP is a malignancy of a high recurrent rate after local excision, standardized wide excision is the key in reducing local failure. Adjuvant radiotherapy is an effective treatment for the patients with positive resection margin or the patients don’t suit for surgical treatment. The DFSP FS need to use more energetic treatment in curing it.更多还原