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[隆突性皮肤纤维肉瘤的临床诊断与治疗体会]

[Realization of clinical diagnoses and treatment for dermatofibrosarcoma protuberans].

作者信息

Cai Hong, Shi Ying-Qiang, Wang Ya-Nong, Zhu Hui-Yan, Mo Shan-Jing

机构信息

Department of Surgery, Cancer Hospital, Fudan University, Shanghai 200032, China.

出版信息

Zhonghua Wai Ke Za Zhi. 2004 Jun 7;42(11):678-82.

PMID:15329259
Abstract

OBJECTIVE

To emphasize the importance of correct and standardized surgical treatment on dermatofibrosarcoma 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 >/= 3 cm and 5 of them (5.1%) developed local recurrence while 36 ones had excision margins 1 approximately 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.

摘要

目的

强调正确规范的手术治疗对隆突性皮肤纤维肉瘤(DFSP)的重要性,并探讨其合适的综合治疗方法。

方法

对1985年1月至2002年9月间收治的163例DFSP患者进行回顾性研究。

结果

163例中,150例(92.0%)在复旦大学附属肿瘤医院就诊前按良性肿瘤行局部切除。69例(46.0%)在接受广泛切除术后经病理检查证实有肿瘤残留,其中49例(71.0%)在手术前体格检查及B超检查未发现肿瘤残留。肿瘤切除后尤其是局部切除后极易复发,局部切除后的复发率为45.1%,远高于广泛切除后的复发率(5.6%)。在142例行广泛切除的病例中,切缘≥3 cm的99例,其中5例(5.1%)发生局部复发;切缘1~2 cm的36例,其中3例(8.3%)出现局部复发。46例(32.4%)在皮肤缺损区行植皮,11例行皮瓣移植,1例行涤纶修补。这些手术后的主要并发症为皮瓣坏死(20例)和伤口感染(6例),均在2个月内治愈。17例因复发多次或广泛切除后切缘阳性行辅助性放疗,剂量为3275 cGy至7000 cGy。放疗后仅1例出现湿性脱皮,2例发生局部复发。163例中仅2例(1.2%)死亡,其中1例死于肺肝转移。2例发生淋巴结转移,行手术治疗后至今存活。13例(8.0%)为纤维肉瘤型DFSP,恶性程度增加,其中11例为局部切除或广泛切除术后复发。

结论

临床医生应充分了解DFSP以避免误诊。一旦局部切除后诊断为DFSP,应行广泛切除。由于DFSP局部切除后复发率高,规范的广泛切除是降低局部复发的关键。辅助性放疗对切缘阳性或不宜手术的患者是有效的治疗方法。纤维肉瘤型DFSP需采取更积极的治疗措施以治愈。

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