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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。     

隆凸性皮肤纤维肉瘤的诊断和治疗

目的 探讨隆凸性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法 分析第二军医大学长海医院收治的DFSP病人11例,均由手术后病理确诊。其中DFSP纤维肉瘤型（DFSP-FS）4例。肿瘤位于躯干部9例,位于四肢2例。2例行局部常规切除,其余9例均行扩大切除术。3例在术后行放射治疗,平均剂量55Gy（50—65Gy）。结果 切除11例标本,CD34阳性10例,阴性1例。术后随访1—10年（平均4．3年）,失访1例。复发6例,平均复发时间2、6年（10个月至5年）。11例均未见有远处转移,无死亡。结论 DFSP在临床上易与其他软组织肿瘤相混淆。诊断主要依据病理。治疗DFSP最主要的方法是扩大的手术切除,切缘阳性或肿块太靠近切缘的辅以放疗。单独使用放疗可以作为偶见的无法切除肿块的治疗方式。     

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目的 总结转化型隆凸性皮肤纤维肉瘤(DFSP)的临床病理特征,探讨规范化的手术方法和综合治疗途径.方法 对复旦大学上海医学院附属肿瘤医院1985年1月至2006年12月收治的34例转化型DFSP病例,进行临床和病理资料的回顾性分析.结果 34例转化型DESP中,伴有纤维内瘤样转化者(DFSP-FS)32例,伴有恶性纤维组织细胞瘤样转化者(DFSP-MFH)2例,占同期收治手术治疗的260例DFSP的13.1%.经中位随访49个月后,发现其广泛切除手术后局部复发率较传统型DFSP的复发率明显增高(P＜0.0001),且复发灶较 大(P=0.009),复发时间较短(P=0.012),淋巴结和血道等远处转移的发生率高(P＜0.0001),5年和10年存活率明显比传统型DFSP差(P＜0.0001).结论 转化型DFSP的恶性程度较高,预后较差,临床医师必须引起重视并采用更积极的治疗方案.及时和规范化的广泛切除手术非常重要.术后辅助放疗等综合治疗的应用也常需考虑.     

胃癌切除术后切缘残留癌31例报告

胃癌切除术后切缘残留癌３１例报告徐建，林江胃癌术后切缘残留癌，不仅５年生存率大大降低，且常需再次手术切除残留病变。我院１９８６年５月至１９９３年１２月共施行胃癌切除手术５３４例，术后病理证明切缘残留癌３１例，占５．８％，其中１２例在近期内再次手术，报...     

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。     

病灶局部广泛切除术在阴茎癌原发肿瘤治疗中的初步结果

目的 探讨阴茎癌病灶局部广泛切除术在阴茎癌原发肿瘤治疗中的价值.方法 阴茎癌患者33例.患者平均年龄46.8(24～70)岁.其中肿瘤位于包皮的10例,位于阴茎头的21例,位于阴茎根部的2例;原发肿瘤分期T1及以下的30例,其他分期3例;包皮广泛切除及补充切除8例、阴茎肿瘤局部广泛切除术18例,包皮环切术+阴茎肿瘤局部广泛切除术6例,保留阴茎的阴茎头部分切除术1例.结果 33例手术均顺利完成,术后随访3～55个月,平均22个月.术后局部复发率15.1%(5/33),平均复发时间6(2～14)个月.性功能恢复满意率93.9%(31/33),排尿功能恢复满意率100%.结论 根据适应证选择阴茎癌病灶局部广泛切除术治疗阴茎癌安全有效,术后局部复发率低.患者术后性功能和排尿功能恢复良好,生活质量满意.局部复发的机制尚需进一步研究.     

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。     

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.     

乳腺叶状囊肉瘤24例临床分析

目的探讨乳腺叶状囊肉瘤的临床特点、外科治疗方法及影响预后的因素。方法对我院收治的24例乳腺叶状囊肉瘤患者根据患者年龄、原发肿瘤大小、有无腋淋巴结转移等因素选择局部包块切除术或单乳切除术进行治疗,并对术后治疗结果进行随访。结果本组24例中行局部切除11例,单侧乳房切除术9例,乳房切除并腋窝淋巴结清除4例,其中5例(20％)曾诊断为腺纤维瘤或巨纤维瘤因术后肿瘤复发而经历2-3次局部切除术。术后病理学检查证实:良性11例,交界性9例,恶性4例。随访1-10年(中位5．2年),5年生存率97％,局部复发率31．3％。结论乳腺叶状囊肉瘤发病率低,多数病例病理分化较好,病程进展缓慢,手术切除是其首选的治疗措施,局部切除后反复复发者应行乳房切除;叶状囊肉瘤预后与肿瘤的病理类型、肿瘤大小和手术切除是否彻底有关。     

隆突性皮肤纤维肉瘤的广泛切除术

目的 探讨隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)经广泛性切除术后的长期临床随访结果,并分析该病经多次手术切除复发后的生物学行为变化.方法 回顾性分析1978年2月至2002年12月收治的109例DFSP患者的临床资料,患者无瘤生存率采用x2检验.结果 所有患者采用广泛性切除术,99例(90.8%)完全切除,7例(6.4%)切缘阳性,另有3例(2.8%)切缘距肿瘤组织＜1 mm.46例原发组中,40例术后直接缝合,6例需游离植皮或转移皮瓣覆盖创面.术后5年无瘤生存率95.7%,10年随访未见远处转移.复发组有43例术后直接缝合,20例需游离植皮或转移皮瓣,5年无瘤生存率为81%,10年随访有4例出现肺转移.两组间5年无瘤生存率比较,P=0.025;10年远处转移率比较,P=0.032,差异均有统计学意义.结论 对于DFSP患者行距肿瘤边缘2.5～3.5 cm的广泛性切除术,可获得较好的局部控制.多次复发的患者,切除术后局部复发和转移率上升.     

Dermatofibrosarcoma protuberans in the breast: Case report

Introduction and importanceDermatofibrosarcoma protuberans (DFSP) represents about 1% of soft tissue sarcomas with an estimated incidence of 0.8–5.0 cases per million per year. The involvement of DFSP in breast is very rare and very few cases have been reported in the literature. DFSP was recurred in situ, not spread to distant site. The complete surgical excision with wide, pathologically negative margins of 3 cms is the optimal treatment for primary or recurrent tumor.Presentation of caseA 46-year-old woman presented with palpable lump in the in the right breast. On ultrasonography, a lesion appeared as hypoechoic, circumscribed mass of approximately 37 mm × 30 mm in diameter in the upper central part of the right breast. The mass of right breast was demonstrated DFPS by pathologic examination. Chest computerized tomography (CT) scan and 2-[18F]-fluoro-2-deoxy-d-glucose (18F-FDG) positron-emission tomography (PET) showed only primary lesion in subcutaneous layer and no enlarged lymph node. The patient underwent excision of the tumor widely. There was no evidence of DFSP local recurrence after five years of follow-up of the patient.DiscussionDFSP is a rare tumor arising from dermis and subcutaneous mesenchymal tissue. Whereas, characteristic imaging feature of DFSP in the breast are not well-defined. The primary treatment for DFSP is considered to be surgical excision.ConclusionDFSP in breast is extremely uncommon and can mimic a primary breast tumor. Surgical excision with adequate resection margins is recommended to ensure local control of the disease.     

Dermatofibrosarcoma protuberans: wide and deep block excision including underlying muscle

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin, which has a propensity for local recurrence. Surgery is the only option of treatment and resection with wide surgical margins including the underlying fascia has become the standard treatment. Two cases of DFSP located on the trunk and forearm are presented who were managed with 3 cm tumor free lateral margins and in depth resection including underlying muscles. The patients were found to have no tumors after 3 and 4 years follow-up. This aggressive approach including excision of underlying muscles is advocated for DFSP located on the trunk or extremities to prevent local recurrence.     

Sarcomas arising in dermatofibrosarcoma protuberans: a reappraisal of biologic behavior in eighteen cases treated by wide local excision with extended clinical follow up

There is a prevailing view that sarcomas arising in dermatofibrosarcoma protuberans (DFSP) have a higher risk of metastasis than ordinary DFSP, but these data are based on cases with variable and often suboptimal treatment. There has not been a large study of sarcomas arising in DFSP in which all cases were treated by wide local excision, thereby arguably altering outcome. Clinicopathologic features of 18 cases of sarcomas arising in DFSP treated by wide local excision and having follow up of at least 5 years were analyzed. An estimate of the proportion of sarcoma and DFSP was made. The number of mitotic figures and degree of CD34 immunoreactivity were assessed in each case. The cohort included 13 females and 5 males (age, 23-87 yrs; median, 47 yrs). The tumors involved the trunk (7), scalp (4), extremities (4), and inguinal region (3), and ranged from 1.5 to 7 cm (median, 4 cm). Sarcoma occurred de novo in 15 cases and in a recurrence in three. Sarcomas resembled fibrosarcoma (17) or malignant fibrous histiocytoma (1) and occupied between 20% and 80% of the tumor (median, 60%). Mitotic activity ranged from 2 to 16 per 10 high-power field (HPF; median 7 per 10 HPF) in the sarcomatous component and 0 to 3 per 10 HPF (median, 1 per 10 HPF) in the DFSP component. All tumors expressed CD34 in the DFSP component but only nine (50%) in the sarcomatous component. All patients were treated by wide local excision with negative margins; three additionally received radiation. Four patients (22%) developed recurrences, but none developed metastasis during the follow-up period of 62 months to 17 years (median, 81.5 mos). In contrast to earlier studies, we demonstrate that patients with sarcomas arising in DFSP do not have an increased risk of distant metastasis within a 5-year follow-up period, provided they are treated by wide local excision with negative margins. This probably reflects the fact that wide local excision results in eradication of local tumor, thereby eliminating the source for subsequent dissemination. However, we cannot completely exclude the possibility that tumors in which clear margins are achieved represent a less aggressive subset, as has been suggested for high-grade extremity sarcomas. Previous studies showing increased metastasis for sarcomas arising in DFSP should be re-evaluated to determine if, with treatment stratification, metastatic rate varies.     

Dermatofibrosarcoma protuberans. A clinicopathologic review with emphasis on fibrosarcomatous areas.

We reviewed 75 cases of dermatofibrosarcoma protuberans (DFSP) from the University of Texas M.D. Anderson Cancer Center. All accessions were examined for areas of giant cell fibroblastoma (GCF), but none was found. The 30 cases having a minimum of 5 years follow-up were studied in more detail. The histologic findings were typical of DFSP in 24 cases, whereas in six cases discrete areas with a fascicular or "herringbone" growth pattern, considered to represent fibrosarcomatous change (DFSP-FS), were evident. The mitotic rate was usually but not always higher in fibrosarcomatous areas, and occasional examples of typical DFSP demonstrated relatively numerous mitotic figures (up to 35 per 10 high-power fields). Other histologic findings of interest were the presence of melanin in two cases of DFSP and the focal presence of a distinctive type of multinucleated giant cell similar to those seen in GCF in six cases. Patients with DFSP-FS differed from those with DFSP in that they had a higher median age (56 years vs. 37 years). Tumor location was similar in both groups, with the trunk being the most common site. No significant difference in either the rate of local recurrence or the interval until recurrence between DFSP and DFSP-FS was evident; the only factor strongly related to local recurrence was adequacy of surgical margins. However, the only two patients who died of tumor, including the sole patient with distant metastasis, had DFSP-FS. We conclude that DFSP-FS deserves recognition as a variant of DFSP.     

Dermatofibrosarcoma Protuberans: Reappraisal of Wide Local Excision and Impact of Inadequate Initial Treatment

Background: The extent of local invasion in dermatofibrosarcoma protuberans (DFSP) is often clinically difficult to appreciate, and this leads to inadequate resections. We examined the effect of inadequate initial treatment and the efficacy of wide resection.Methods: We performed a retrospective analysis of the records of 35 patients with DFSP treated at our institution (1985 and 2001). Data were analyzed with Wilcoxons ranked sum test and Fishers exact test.Results: Of the 24 patients eligible for analysis, 11 had definitive wide resection after diagnostic excisions elsewhere (primary group), and 13 had recurrent tumors after previous surgical treatment elsewhere (recurrent group). Twenty-three patients were treated with wide resection only, and adjuvant radiation was administered to one patient who had a fibrosarcoma. At a median follow-up of 54 months, patients definitively treated at our institution had a 100% local recurrence–free survival. In comparison to the primary group, recurrent DFSPs were significantly larger and deeper and occurred in the head and neck region. Five cases had bone involvement, and of these, 80% occurred in the recurrent group.Conclusions: Inadequate initial treatment results in larger, deeper recurrent lesions, but these can be managed by appropriate wide excision. Wide resection of DFSP (whether recurrent or primary) with negative histological margins predicts a superior local recurrence–free survival.     

Modified Mohs Micrographic Surgery in the Therapy of Dermatofibrosarcoma Protuberans: Analysis of 22 Patients

Background: Dermatofibrosarcoma protuberans (DFSP) is a very rare, low-grade malignant tumor. Local recurrence is frequent after incomplete resection either because of false diagnosis or inadequate standard surgical excision. This study evaluated the clinical outcome of micrographic surgery with paraffin sectioning in the therapy of DFSP.Methods: Medical charts of 22 patients with histologically confirmed DFSP treated at our institution between 1987 and 2002 were reviewed.Results: For 13 patients with primary and 9 with recurrent disease, tumor-free excision margins could be achieved, and all patients remained free of local recurrence during a mean follow-up of 54 months.Conclusions: The results of our study and a review of the literature confirm the successful and recurrence-free treatment of DFSP with micrographic surgery as the treatment of choice for this locally invasive tumor. Particularly in recurrent lesions, we recommend the use of paraffin sectioning and three-dimensional histological evaluation as an accurate additional tool for treatment optimization.     

Mohs Micrographic Surgery for Dermatofibrosarcoma Protuberans: University of Miami and NYU Experience

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare cutaneous malignancy with the potential for significant local destruction and morbidity. This neoplasm has a tendency for recurrence following excision due to its infiltrative nature. Surgical excision with wide margins has been considered the standard therapy for DFSP but has had reported recurrence rates of up to 60%. OBJECTIVE: The results of 20 patients with DFSP treated with Mohs micrographic surgery (MMS) are reported. We also reviewed the world literature on the role of MMS for treatment of this tumor. METHODS: Twenty patients between the ages of 20 and 82 years with DFSP were treated with MMS. The patients were followed for recurrences between 4 and 216 months (mean 56.4 months). RESULTS: None of the 20 patients had a recurrence of DFSP following MMS. Of 221 DFSP patients in our review of the world literature treated with MMS, only 5 (2.3%) had a recurrence. CONCLUSION: MMS is an effective treatment of DFSP.