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。     

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.     

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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。     

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。     

隆凸性皮肤纤维肉瘤的临床诊治

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2002年9月收治的163例DFSP病例,进行临床和病理资料的回顾性分析。结果 163例患者中有150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69例,占46．0％,其中49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45．1％,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142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∽2em,术后复发3例(8．3％)。46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(20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3275∽7000cGy。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,经手术治疗后存活至今。有13例(占8．0％)出现纤维肉瘤样改变(DFSP-FS),其恶性程度增加,其中11例见于局部复发的病例。结论 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;规范化的广泛切除是治疗DFSP、降低复发率的主要方法;对术后切缘阳性、身体状况不适合手术治疗的患者辅以放射治疗仍不失为一种有效的治疗方法;对DFSP-FS,临床医师必须引起重视并采用更积极的治疗方案。     

手背巨大隆突性皮肤纤维肉瘤1例报告

隆突性皮肤纤维肉瘤（dermato fibrosarcoma protuberans,DFSP）临床上少见,术后容易复发。2009年3月我院收治手背巨大DFSP1例,经外院多次手术切除均复发,治疗难度大。本次治疗采用肿物扩大根治切除术＋皮瓣转移术＋植皮术,疗效满意,随访1年未复发,现报告如下。     

韧带样型纤维瘤病的诊断和治疗

目的:探讨韧带样型纤维瘤病(DTFT)的诊断和治疗方法。方法:回顾性总结收治的DTFT11例患者的临床资料。肿瘤位于腹壁和盆腔4例，腹腔内2例，腹部以外5例。病程平均为5.7年(1个月至41年)。术前均误诊。2例行局部切除，其余9例均行扩大切除术; 3例术后行放射治疗。结果:11例术后病理均诊断为DTFT，其中波形蛋白阳性10例，阴性1例。术后随访1~10年(平均4.2年)，复发4例，平均复发时间2.2年(8个月至5年)。11例均健康生存，未见有远处转移。结论:DTFT在临床上易与其他软组织肿瘤相混淆; 诊断主要依据病理报告; 治疗DTFT最主要的方法是扩大的手术切除。该病预后较好，复发者可再次手术。单独使用放疗可以作为无法切除肿块的治疗方式。     

隆突性皮肤纤维肉瘤的MRI和CT表现

目的探讨隆突性皮肤纤维肉瘤（DFSP）的MRI与CT特征。方法回顾性分析11例经手术及病理证实的隆突性皮肤纤维肉瘤患者的MRI及CT资料。11例中,8例接受MR检查,其中5例接受增强扫描;4例接受CT扫描,其中1例同时接受增强扫描。结果10例DFSP位于皮下及皮下脂肪层,表现为边界清楚的椭圆形或分叶状肿块;1例位于左侧大腿中上段深部。DFSP在T1WI图像上主要为低至稍高信号,T2WI图像上呈稍高或高信号;CT平扫呈等密度。MR和CT增强扫描病灶均明昂强化。结论隆突件皮肤纤维肉瘤的MRI和CT嘉孤且右一宦特衍．右助千明确诊断．     

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。     

A case of aggressive giant dermatofibrosarcoma protuberance occurring in the parotid gland

IntroductionDermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that arises from the dermis and invades deeper tissue. The cellular origin of DFSP is not clear. Evidence supports the cellular origin being fibroblastic, histiocytic, or neuroectodermal.Presentation of caseA 38 years old, women presented with slow-growing large right parotid mass. A total parotidectomy performed with auriculectomy and reconstruction using ALT flap. Diagnosis confirmed by pathology and immunohistochemistry. Tumor recurred in 10 months, the second surgery with subsequent chemoradiotherapy performed. Patient initially treated with wide resection, 4 cycles of chemotherapy and postoperative radiotherapy 40 Gy, with the recurrence in 10 months. We performed a second surgery followed by radiotherapy. She is disease-free for more than two years under follow-up.DiscussionThe main treatment of DFSP is surgical resection with wide negative margins or Moh’s surgery. Advanced cases treated with addition of radiotherapy or chemoradiotherapy, but with unclear benefits. In our case, huge tumor located in the parotid region recurred after initial surgery and adjuvant treatment.ConclusionClinically, DFSP usually manifest as well circumscribed, slow-growing, smooth, and painless masses. In cases with advanced tumor in parotid region, recurrence may occur despite aggressive initial treatment with wide resection and chemoradiotherapy.     

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.     

非典型脑膜瘤术后复发影响因素分析

目的分析影响非典型脑膜瘤复发的影响因素。方法回顾性分析2013-01—2016-12间在郑州大学第一附属医院行手术治疗且经病理学证实的94例非典型脑膜瘤患者。通过多因素Cox回归分析方法评价影响术后复发的临床因素。结果94例患者发病年龄12~78岁,男∶女=1∶1.29。82例肿瘤全切除,12例肿瘤次全切除。所有患者术后随访11~66个月,中位时间36个月。其中行手术及辅助放疗43例,仅行手术治疗51例。其间31例肿瘤复发。1 a、3 a无进展生存率分别为87.2%、57.1%,1 a、3 a、5 a总生存率为98.2%、78.1%、73.4%。单因素分析表明年龄≥50岁、非原发性、位于颅底、脑侵犯、Ki67指数>10%及次全切除,与非典型脑膜瘤术后高复发率有关。多因素分析结果表明,年龄、脑侵犯、Ki67指数及切除程度是影响肿瘤复发的独立危险因素。结论非典型脑膜瘤具有高复发率和高病死率,年龄≥50岁、非原发性、位于颅底、脑侵犯、Ki67指数>10%及次全切除与非典型脑膜瘤术后高复发率有关,其中年龄、脑侵犯、Ki67指数及切除程度是影响肿瘤复发的独立危险因素。最大安全范围切除肿瘤是最主要的治疗措施,对于不完全切除的肿瘤和位于矢状窦旁或颅底的肿瘤推荐接受辅助放疗。     

Extraventricular neurocytomas: pathologic features and clinical outcome.

Neurocytic neoplasms usually arise within the lateral ventricles, generally as circumscribed, slowly growing masses curable by total resection. Both subtotal resection and histologic atypia are associated with an increased risk of recurrence. In contrast, neurocytic neoplasms situated within brain parenchyma, so-called "extraventricular neurocytomas" (EVNs), are not as well characterized. The relationships between histologic features and extent of resection versus clinical behavior have not been defined. We evaluated pathologic features, clinical data, and neuroimaging of 35 examples. The tumors occurred in 18 males and 17 females, age 5-76 years (median 34 years). All tumors involved the cerebrum. On imaging, EVNs were solitary, variably contrast-enhancing, and often (57%) cystic. Tumor cells were arranged in sheets, clusters, ribbons, or rosettes, in association with fine neuropil dispersed in broad zones that separated cell aggregates. Ganglion cell differentiation was seen in 66%. All tumors showed strong synaptophysin immunoreactivity. Despite the lack of apparent astrocytes in hematoxylin and eosin-stained sections, focal glial fibrillary acidic protein reactivity was seen in 46%. Eleven EVNs were designated "atypical" based on the presence of necrosis, vascular proliferation, or elevated mitotic activity (> or = 3 mitoses/10 high power fields). Nineteen tumors were subtotally resected or biopsied, whereas 14 were totally resected grossly. Seventeen patients underwent radiotherapy (mean 55 Gy). In 30 cases with follow-up, 10 tumors recurred, 3 causing death at 6, 14, and 43 months. All 10 recurrences followed subtotal resection. No totally resected tumors recurred. Thus, the majority of EVNs are well differentiated and appear unlikely to recur after gross total resection. Subtotal resection, atypical histologic features, and high cell proliferation rates correlate with recurrence.     

A Multi-Institutional Phase II Trial of Preoperative Full-Dose Gemcitabine and Concurrent Radiation for Patients With Potentially Resectable Pancreatic Carcinoma

Background We report the results of a multi-institutional phase II trial that used preoperative full-dose gemcitabine and radiotherapy for patients with potentially resectable pancreatic carcinoma. Methods Patients were treated before surgery with three cycles of full-dose gemcitabine (1000 mg/m² intravenously), with radiation during the second cycle (36 Gy in daily 2.4-Gy fractions). Patients underwent surgery 4 to 6 weeks after the last gemcitabine infusion. Results There were 10 men and 10 women, with a median age of 58 years (range, 50–80 years). Nineteen patients (95%) completed therapy without interruption, and one experienced grade 3 gastrointestinal toxicity. The mean weight loss after therapy was 4.0%. Of 20 patients taken to surgery, 17 (85%) underwent resections (16 pancreaticoduodenectomies and 1 distal pancreatectomy). The complication rate was 24%, with an average length of stay of 13.5 days. There were no operative deaths. Pathologic analysis revealed clear margins in 16 (94%) of 17 and uninvolved lymph nodes in 11 (65%) of 17 specimens. One specimen contained no residual tumor, and three specimens revealed only microscopic foci of residual disease. With a median follow-up of 18 months, 7 (41%) of the 17 patients with resected disease are alive with no recurrence, 3 (18%) are alive with distant metastases, and 7 (41%) have died. Conclusions Preoperative gemcitabine/radiotherapy is well tolerated and safe when delivered in a multi-institutional setting. This protocol had a high rate of subsequent resection, with acceptable morbidity. The high rate of negative margins and uninvolved nodes suggests a significant tumor response. Preliminary survival data are encouraging. This regimen should be considered in future neoadjuvant trials for pancreatic cancer.     

Treatment outcome of resected and nonresected primary adenoid cystic carcinoma of the lung.

BACKGROUND AND OBJECTIVE: The incidence of primary adenoid cystic carcinoma of the lung is relatively rare and the optimal treatment strategy is still unclear. METHODS: Sixteen adenoid cystic carcinoma patients were treated at our institute from 1972 to 1998 and their clinical features, treatments and survivals were reviewed. RESULTS: Half of all patients were female and the median age was 46 ranging from 30 to 64. All primary lesions were located in the central bronchial tree and 80% of the patients had some symptoms. Eleven patients underwent a resection of the tumor with/without plasty of the trachea or bronchus. Although 6 (55%) of 11 patients had a microscopic residual tumor after resection, 5 patients who received postoperative radiotherapy survived without recurrence from 3 to 17 years. Five patients received radiotherapy as their initial treatment and all tumors responded well to the treatment. The 5-year and 10-year survival rates were 91 and 76% without local recurrence in the resected group and 40 and 0% in the nonresected group, respectively. CONCLUSION: These observations suggest that surgical resection should be selected first whenever possible, and, in addition, adenoid cystic carcinoma is sensitive to radiotherapy.     

Postoperative radiotherapy in the management of spinal cord ependymoma

Fifty-eight patients with histologically verified spinal cord ependymomas were treated at the Royal Marsden Hospital and Atkinson Morley's Hospital between 1950 and 1987. The median age in this series was 40 years (range 1 to 79 years) and the male:female ratio was 1.8:1. Ten patients had tumors in the cervical cord and 10 in the thoracic cord; 14 tumors involved the conus medullaris and 24 the cauda equina. Forty ependymomas were grade I and 13 were grades II to IV (in five patients there was insufficient material for grading). Eleven patients underwent biopsy only, 33 had partial or subtotal resection, and 14 had complete resection. Forty-three patients received postoperative radiotherapy. The median follow-up period was 70 months (range 3 to 408 months). Cause-specific survival rates were 74% and 68% at 5 and 10 years, respectively. On univariate analysis, age, histological grade, postoperative neurological function, and era of treatment were significant prognostic factors for survival. The histological grade was the only significant independent prognostic factor. The relative risk of death from ependymoma was 9.0 for patients with tumor grades II to IV compared to grade I (p less than 0.005, 95% confidence interval 2.7 to 30). The survival rates of patients following complete excision were significantly better compared to those after incomplete surgery (p less than 0.025). The majority of completely resected neoplasms were low-grade cauda equina tumors. Despite incomplete surgery, 5- and 10-year progression-free survival rates following radical radiotherapy were both 59%, and cause-specific survival rates were 69% at 5 years and 62% at 10 years. This suggests that radiotherapy may achieve long-term tumor control in over half of those patients with residual spinal ependymoma.     

Desmoid tumors in adults: the role of radiotherapy in their management

Twenty-six adult patients with the pathologic diagnosis of desmoid tumor were treated between 1964 and 1983 at the Institut Curie in Paris with megavoltage irradiation. Twenty of these patients (76 percent) had extraabdominal tumors. Definitive surgical resection was performed on nine patients (one received preoperative radiotherapy). At last follow-up 1 1/2 to 10 years after treatment, all of the patients had no evidence of disease. Seven of the nine had follow-up examinations from 5 to 10 years after treatment. Seven patients had postoperative radiotherapy with doses from 4,700 to 6,500 rads (47 to 65 Gy) for either microscopic (three patients) or gross (four patients) residual disease. All but one patient had no evidence of disease from 2 to 8 years after treatment. Nine patients had radiotherapy for recurrent inoperable tumors and six had no evidence of disease from 3 to 20 years after treatment. Recurrences developed in three patients; outside the treatment portal in one, and the other two had received less than 5,000 rads (50 Gy). Clinical regression of tumors after treatment was slow, with complete regression taking up to 2 years. Postoperative radiotherapy with doses of at least 5,000 to 6,000 rads (50 to 60 Gy) was effective in achieving local control of inoperable or incompletely resected tumors, thus the need for repeated resections was avoided. Computerized tomography has greatly improved the assessment of tumor extension and should be used routinely before either operation or radiotherapy to obtain adequate margins and minimize the chance of missing disease.     

Video-assisted wedge resection and local radiotherapy for peripheral lung cancer in high-risk patients: the Cancer and Leukemia Group B (CALGB) 9335, a phase II, multi-institutional cooperative group study

OBJECTIVES: This study examined the feasibility of thoracoscopic wedge resection and radiotherapy for clinical T1 lesions in patients with compromised cardiopulmonary status. METHODS: In this phase II, prospective, multicenter, cooperative group trial, high-risk patients had one or more of the following risk factors: forced expiratory volume in 1 second less than 40%, carbon monoxide diffusing capacity in lung less than 50%, and maximum oxygen consumption less than 45 mm Hg. Patients underwent video-assisted wedge resection followed by local (56 Gy) radiotherapy. The primary end point was the proportion of patients whose disease could be completely resected and who received radiotherapy without treatment complications. RESULTS: Between September 1995 and September 1999, a total of 65 patients were accrued, of which 58 were eligible (52% male, median age 69 years). Pathologic staging resulted in upgrading to T2 or greater in 16 of 58 cases (28%) and in reassessment as benign in 10 of 58 cases (17%). Conversion to thoracotomy was required in 10 cases (17%), including 1 of 10 benign T1-size lesion (10%), 4 of 35 non-small cell lung cancer T1 lesions (13%), and 5 of 14 non-small cell lung cancer T2 lesions (31%). Resection margins were positive in 5 patients: 6% of T1 and 23% of T2. Surgery was aborted in 2 cases (3.5%), and operative mortality was 4%. Overall operative failure rates of video-assisted wedge resection were 20% for benign T1-size lesions, 22% for T1 non-small cell lung cancer, 21% for all T1 lesions, 50% for T2 non-small cell lung cancer, and 29% for all lesions in this study (clinical T1). Prolonged air leaks occurred in 10%, pneumonia in 6%, and respiratory failure in 4%. Thirty-one patients were eligible for radiotherapy; 3 of them refused, and 1 died before treatment. Among the 28 patients who received radiotherapy, severe dyspnea was noted in 3 patients (11%) and moderate pneumonitis in 4 (14%). CONCLUSIONS: Clinical staging in high-risk patients is often inaccurate (45% difference from pathologic staging). Intention to treat clinically staged T1 disease by video-assisted wedge resection is associated with a high failure rate. Pathologically staged T1 lesions can be successfully resected in 75% of cases; however, narrow resection margins remain a concern.