隆突性皮肤纤维肉瘤诊断与治疗

目的：探讨隆突性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法：对28例经病理证实不同部位隆突性皮肤纤维肉瘤患者的诊治情况进行回顾性分析。躯干部16例（57％）,四肢部8例（28．5％）,头颈部4例（14．2）。5例行局部切除术,23例扩大切除术＋植皮或皮瓣转移修复术。16例在术后行放射治疗,平均照射剂量60Gy（50～70Gy）。结果：切除28例标本,CD34阳性26例,阴性2例。术后随访1～5年（平均2．3年）,失访2例。复发9例,平均复发时间2．6年（8个月～5年）。28例均未见有远处转移,无死亡。结论：隆突性皮肤纤维肉瘤是一种低度恶性肿瘤,行扩大切除术＋辅助放疗是治疗隆突性皮肤纤维肉瘤的主要治疗方法,手术范围大小及术后辅助放疗与否是影响隆突性皮肤纤维肉瘤的独立预后因素,可有效提高患者生存率及生存质量。     

隆突性皮肤纤维肉瘤外科治疗和复发因素

目的：探讨隆突性皮肤纤维肉瘤的外科治疗及复发相关因素。方法：将78例患者按入院时情况分为无复发组36例和复发组42例均行肿瘤广泛切除术治疗,标本按顺时针方向做好标志,如病理切缘阳性则再次补充切除直到切缘阴性,无法达到病理切缘阴性者予以放疗,剂量为45~60Gy。结果：35例切口一期缝合,发生切口积液4例,43例行植皮术或皮瓣修复术,发生植皮或皮瓣坏死11例。术后随访时间6月~10年,出现局部复发16例,合并肺转移死亡1例。复发组、病理切缘阳性、隆突性皮肤纤维肉瘤伴纤维肉瘤样改变的病人更容易出现复发P〈0.01~0.05。结论：隆突性皮肤纤维肉瘤首次接受规范的肿瘤广泛切除术是降低局部复发的关键,放疗不能常规作为手术的补充手段。     

隆突性皮肤纤维肉瘤扩大切除及皮瓣修复术的护理配合

目的:探讨隆突性皮肤纤维肉瘤扩大切除及皮瓣修复术的护理方法。方法:对53例隆突性皮肤纤维肉瘤患者进行扩大切除及皮瓣修复术的护理。护理措施包括做好术前准备,术后加强对体位和低负压引流的管理,做好移植皮瓣血管危象和切口感染等并发症的护理,以及提供心理支持和出院指导。结果:52例患者皮瓣完全成活,1例因感染皮瓣坏死,经再次植皮,皮瓣愈合良好。术后4~12月门诊随访,52例未见肿瘤复发和转移,功能和外观保持良好,1例于术后半年复发但无转移。结论:良好的护理措施是隆突性皮肤纤维肉瘤行扩大切除及皮瓣修复术成功的重要保障。     

皮肤软组织肿瘤临床治疗

为修复复发性隆凸性皮肤纤维肉瘤，自１９８８年以来，共治疗复发性隆凸性皮肤纤维肉瘤５例。认为本病治疗的预后与手术创面的处理技巧有关。适当选择、设计植皮或皮瓣移植以及重视切除范围，选择适当的方法修复创面，有助于本病的治愈。     

软组织肉瘤切除后的修复重建

目的探讨修复重建在技术手术治疗软组织肉瘤中的作用和地位。方法１９９０年１１月～１９９６年１１月,治疗软组织肉瘤１０７例,其中３２例进行了一期修复重建手术。３２例中,恶性纤维组织细胞瘤１２例,滑膜肉瘤６例,脂肪肉瘤３例,神经纤维肉瘤３例,横纹肌肉瘤３例,皮隆突纤维肉瘤２例,纤维肉瘤２例,上皮样肉瘤１例;４例为原发,２８例为复发;外科分期为ⅠＢ３例,ⅡＡ３例和ⅡＢ２６例。根治性切除１３例,广泛切除１７例,局部切除２例。手术加化疗１３例,手术加放疗７例。血管重建３例,动力功能重建１６例,皮肤缺损修复２２例。结果术后随访到３０例,随访时间４个月～６年６个月,肉瘤局部控制率为８０％,血管重建保肢率为１００％,动力功能重建优良率为８７．５％,缺损组织修复成活率为９６％。结论修复重建是软组织肉瘤切除后外科治疗的延续,是减低肿瘤的复发率,提高患者生活质量的重要措施之一。     

皮肤软组织肿瘤临床治疗

为修复复发性隆凸性皮肤纤维肉瘤，自１９８８年以来，共治疗复发性隆凸 皮肤纤维肉瘤５例。认为本病治疗的预后与手术创面的处理技巧有关。适当选择，设计植皮或皮瓣移植以及重视切除范围，选择适当的方法修复创面，有助于本病的治愈。     

整形外科手术联合局部放疗在隆突性皮肤纤维肉瘤治疗中的应用

目的:在提高对隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)的诊断、治疗意识的同时,利用整形外科手段改善治疗后的术区外观。方法:对来院确诊的2例隆突性皮肤纤维肉瘤患者,行扩大手术切除,切除范围包括距肿瘤边缘3cm的正常皮肤和皮下组织,并对局部进行放疗。结果:术后经6个月~1年随访,未见复发迹象。结论:扩大手术切除为隆突性皮肤纤维肉瘤首选治疗办法,主张切除范围包括距肿瘤边缘3cm的正常皮肤和皮下组织;放疗作为该肿瘤的一种有效辅助治疗方法,能显著降低局部复发率,尤其对于术后切缘阳性、不适合手术治疗以及无法实行广泛切除的病例更应辅助放疗。     

腹壁隆突性皮肤纤维肉瘤诊治策略及复发相关因素分析

目的 探讨腹壁隆突性纤维肉瘤的诊断与治疗方法,分析复发相关因素。方法 对51例腹壁隆突性皮肤纤维肉瘤患者的临床资料进行回顾性分析,根据是否放疗、切缘距离及肿瘤大小进行分组,比较组间术后复发率的差异。结果 患者肿物切除术后创面缺损面积4 cm×6cm至20cm×26cm,均I期予植皮、局部皮瓣或游离皮瓣修复,术后随访1-30.4年,总体复发率为23.1%,1例复发7次的患者发生肺转移。非放疗组与放疗组的复发率差异无统计学意义;切缘≤2cm组的复发率明显高于切缘〉2cm组（37.5%vs 19.4%,χ2=12.49,P〈0.01）;肿瘤≤5cm组患者复发率为16.7%,肿瘤〉5cm组患者的复发率为25.9%,两组间差异有显著统计学意义（χ2=3.91,P〈0.05）。结论 腹壁隆突性纤维肉瘤单纯局部切除复发率高,手术需行广泛切除,结合术后放疗并不能降低局部复发率。复发可能与肿瘤的大小、切缘距离等因素有关;多次复发可引起远处转移。     

老年人外耳皮肤恶性肿瘤术后创面缺损的修复

目的：分析老年人外耳皮肤恶性肿瘤的临床类型和探讨手术切除后创面缺损修复的方法和效果,为临床提供理想的修复方案。方法：根据外耳皮肤恶性肿瘤的临床类型和解剖特点,采用手术方法彻底切除病变组织,术中病理显示无残癌组织后,对外耳创面缺损进行直接缝合和皮肤移植以及耳局部皮瓣修复。结果：外耳皮肤恶性肿瘤共11例,基底细胞癌2例（18.18%）,鳞状细胞癌7例（63.64%）,棘皮角化瘤2例（18.18%）。修复方法中皮肤移植2例,星形切除直接缝合2例,耳局部皮瓣修复7例。9例随访3年未见复发。结论：鳞状细胞癌为老年人外耳常见的皮肤恶性肿瘤临床类型,局部皮瓣是常用的修复方法,对于皮肤和软骨组织缺损较大的残耳应用耳局部皮瓣修复是治疗老年人耳部皮肤恶性肿瘤较为理想的方法。     

大腿隆突性皮肤纤维肉瘤25例的治疗和预后分析

目的:探讨大腿隆突性皮肤纤维肉瘤(DFSP)的治疗及预后。方法:回顾性分析笔者科室2001年1月-2008年12月治疗的25例大腿隆突性皮肤纤维肉瘤患者资料。所有病例均先行手术,术中行快速冰冻病理切片检查确定切除范围,术后辅以放射治疗。结果:25例患者术后切口均愈合良好。术后随访5年,痊愈21例,复发4例。结论:隆突性皮肤纤维肉瘤的治疗关键在于早发现、早治疗。手术扩大切除是主要治疗方法,放疗为辅助治疗方法。     

Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report

Bednar tumor (pigmented dermatofibrosarcoma protuberans) is a variant of dermatofibrosarcoma protuberans (DFSP) that constitutes 5% of all DFSP and has a very low rate of distant metastases. We encountered a rare case of Bednar tumor with multiple different distant metastases. A 51-year-old man, who had had a history of mass resection in his left shoulder 4 years previously, was referred to our institution, complaining of a recurrence of the shoulder mass. The histological diagnosis of primary tumor was Bednar tumor, and he underwent resectional surgery for the recurrent lesion. A second local relapse, lung metastasis, retroperitoneal metastasis, and metastasis to the sigmoid colon have occurred. The recurrence lesion and all metastatic lesions were resected surgically. The histological features of all specimens showed fibrosarcomatous change. Seven months after the last surgical resection, the tumor recurred and the patient died of multiple abdominal metastases 10 years after the first surgical treatment.     

Distant metastasis of dermatofibrosarcoma protuberans of the scalp--case report

A 49-year-old man presented with a rare dermatofibrosarcoma protuberans (DFSP) of the scalp associated with local recurrence and distant metastasis to the lung and abdomen. An elastic-hard small mass on the right occipital scalp was initially treated by simple resection in another clinic. Ten years later, recurrent tumor was associated with infiltration to the calvarium, and resection was performed again also in another clinic. Approximately 1.5 years later, the patient was transferred to our clinic because of recurrence with intracranial involvement. Repeated relapses and metastasis to the lung were recognized despite surgery, chemotherapy, and local radiation. Eventually, the patient died of distant metastasis to the abdomen 17 years after the initial diagnosis. Scalp DFSP is very uncommon but is an aggressive scalp tumor, so initial wide local resection and local radiation therapy after surgery are important to prevent local recurrence and distant metastasis.     

头皮复发性隆突性皮肤纤维肉瘤的外科治疗

目的 探讨头皮复发性隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans,DFSP）的外科治疗方法和效果.方法 对近3年收治的7例头皮复发性DFSP患者行回顾性分析.肿瘤术中先行活检做冰冻切片病理检查,确认为阳性病例后行距肿瘤边缘≥3 cm的扩大切除术,基底深部的颅骨组织如亦被侵及,一并做颅骨外板清除或全层颅骨切除.切除标本再次行术中冰冻切片病理检查,证实切缘和基底肿瘤阴性后,继发创面行皮瓣/筋膜组织瓣转移修复.肿瘤标本于术后行HE常规染色和免疫组织化学等病理检查.结果 7例肿瘤标本,术中冰冻切片病理检查和术后病理检查均证实为DFSP复发.7例均行≥3 cm的扩大切除,同时做颅骨切除,其中5例行颅骨外板清除,另2例做颅骨全层切除.术中冰冻切片病理和术后病理报告均证实切缘干净,免疫组织化学病理诊断显示肿瘤组织Ki-67均为阳性,CD34部分阳性.术后随访15～41个月,均未见肿瘤局部复发和远位转移.结论 扩大切除及彻底清除被肿瘤侵及的颅骨是头皮复发性DFSP有效的治疗方法,能减少术后复发;皮瓣转移技术的应用有助于肿瘤完全切除后的创面妥善修复.     

Dermatofibrosarcoma protuberans with regional lymph node metastasis: a case report

The case of a 21-year-old man with dermatofibrosarcoma protuberans of the scalp that spread to deep cervical lymph nodes is presented. Available literature is reviewed, and the infrequency of metastasis is discussed. Since cases of metastases almost always involve recurrent lesions, the importance of wide and deep resection in the initial operation cannot be overemphasized.     

Dermatofibrosarcoma protuberans of the toe

Dermatofibrosarcoma protuberans is an unusual soft-tissue tumor with a propensity for local recurrence and occasional metastatic spread. Given its indolent course, it may be mistaken for atypical scarring or a keloid growth. Most lesions are trunkal or on the proximal extremities with digital lesions being extremely rare. There is only one previous reference of the tumor arising on a toe. Early diagnosis and prompt wide local resection of this entity are required to prevent a local recurrence, which would necessitate a wider, more debilitating resection. The management of a patient with a dermatofibrosarcoma protuberans of the second toe is presented along with a review of the literature.     

A case of childhood dermatofibrosarcoma protuberans of the face

We report an extremely rare case of dermatofibrosarcoma protuberans located on the face of an 8-year-old boy. Although dermatofibrosarcoma protuberans typically has intermediate malignancy, the tumor in the present case was highly invasive and required a multistaged operation, including wide resection and full-thickness skin grafting. These operations were successfully performed, and the patient is alive without recurrence for 7 years.     

DERMATOFIBROSARCOMA PROTUBERANS IN A RENALTRANSPLANT PATIENT

Dermnatofibrosarcoma protuberans is an unusual soft tissue tumour with a propensity for local recurrence and rarely metastatic spread. Given its indolent course it may be mistaken for atypical scarring or a keloid growth. Early diagnosis and prompt wide local resection of this entity are required in order to prevent a local recurrence. We report a patient who developed locally invasive dermatofibrosarcoma protuberans 4 years after a successful renal transplantation. Although other forms of sarcoma have been reported as a complication of organ transplantation and immunosuppression, this pathology has, so far, not been reported in a transplant recipient.     

Multiple recurrent dermatofibrosarcoma protuberans of the hand

We present a rare case of a dermatofibrosarcoma protuberans arising on the dorsum of the hand in a 51-year-old woman, who had experienced four recurrences of the tumour following local excisions. We performed a radical surgical excision of the lesion and covered the defect with a distal ulnar artery skin island flap. Adjuvant radiation therapy followed the surgical treatment. Forty months postoperatively, the patient has a functional hand without signs of recurrence and no evidence of disease. Wide surgical excision with margins of 2.5-3 cm is the optimal treatment for dermatofibrosarcoma protuberans. For selected patients, like those presenting large tumours involving the hand, adequate removal is not easily obtainable, or may result in major functional deficits. An alternative strategy may be the combination of less extensive surgery and radiotherapy, in order to prevent mutilation, decrease the local recurrence rates, and minimize the risk of metastases.     

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目的 进一步提高对隆凸性皮肤纤维肉瘤（DFSP）的认识，探讨正确和规范手术治疗的重要性。方法对119例隆凸性皮肤纤维肉瘤，进行临床和病理资料的回顾性分析。结果 119例中有111例（占93.3% )在入院前被误诊为良性肿瘤而行局部切除术，经补充广泛切除，病理检查最终明确局部有肿瘤残留者57例，占51.4%，其中40例（占70.2%)在查体或B超检查时未能发现残留灶；12例（占10.1%)出现纤维肉瘤样改变（DFSP-FS），其中10例见于局部切除或广泛切除后复发的病例（复发次数1-13次不等）。结论 为避免误诊，临床医师有必要进一步提高对DFSP的认识；在局部切除术后，一经病理检查确诊为DFSP，必须施行补充广泛切除以避免肿瘤残留；为减少复发，宜重视首次手术的正确治疗和广泛切除手术的规范治疗。