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。     

T1期肾肿瘤保留肾单位手术切缘安全范围的探讨

目的探讨T1期肾肿瘤保留肾单位手术切缘的安全范围。方法回顾性分析我院泌尿外科行保留肾单位手术的172例对侧肾脏正常的T1期肾肿瘤患者资料。分析切缘大小、围手术期并发症、术中冷冻切片病理、术后石蜡切片病理及肿瘤复发之间的关系。另取同期T1期根治性肾切除标本35例,于体外沿假包膜行肾肿瘤剜除术,取残肾距离切缘2、5和10mm处肾实质送石蜡切片病理检查。结果 172例中,切缘<2mm组13例,2~5mm组87例,>5mm组72例。<2mm组9例行切缘冷冻切片病理检查,1例阳性;2~5mm组38例行切缘冷冻切片病理检查,1例阳性。切缘阳性的2例肿瘤行根治性肾切除术,术后将2例残余肾行石蜡切片病理检查,均未见肿瘤残余。另外,术中行冷冻切片阴性或未行冷冻切片检查,<2mm组和2~5mm组术后石蜡切片病理检查各有1例切缘阳性,其中,<2mm组的病例3年后原位复发,行根治性肾切除术;2~5mm组阳性病例密切随访5年,未发现肿瘤复发。172例保留肾单位手术中10例围手术期有并发症发生,其中,2~5mm组3例(3.4%),>5mm组7例(9.7%)。平均随访33个月,术后石蜡切片病理切缘阴性的病例中,<2mm组1例出现影像学可疑复发,穿刺活检阴性,其他两组均无复发。根治性肾切除术后的35例肿瘤剜除后残肾标本中,2例距离切缘2mm处存在肿瘤浸润。结论 T1期肾肿瘤保留肾单位手术切缘控制在2~5mm较为安全合适。若肿瘤包膜完整,没有必要行术中冷冻切片病理检查。     

一例头部恶性肿瘤反复发作的治疗体会

目的探讨头皮恶性肿瘤的治疗方法。方法分别于2011年和2013年于外院行肿物切除治疗,均未作病理,切除后肿物复发;2015年1月来院,治疗中先后行肿物扩大切除术、颅骨外板磨削术、局部头皮瓣转移头皮缺损修复术,右大腿取皮术;术后切口愈合良好。术后病理"(头皮)低度恶性肿瘤"。同年12月复发且生长迅速。入院后查头颅3DCT及MRI均提示肿物已侵袭颅骨。在气管插管全身麻醉下行头顶部恶性肿瘤扩大切除+局部颅骨瓣、硬脑膜切除+人工硬脑膜及股前外侧游离皮瓣移植修复术。术后病理为梭形细胞肉瘤,符合黏液样纤维肉瘤,侵犯颅骨及硬脑膜。术后1个月行放射治疗,应用6 MeV电子线,200 cGy×30次。结果患者术后恢复良好,放疗过程中术区游离皮瓣皮肤始终完好,无破溃。随访1年,患者生活状态良好,肿瘤无复发迹象,治疗效果满意。结论足够大的切除范围是保证彻底消灭肿瘤的前提,应用游离皮瓣覆盖伴有颅骨缺损的创面是一种较好的手术方法,值得临床推广。     

局部皮瓣治疗面部基底细胞癌疗效观察

目的:观察局部皮瓣治疗面部基底细胞癌的疗效。方法:47例面部基底细胞癌患者,应用局部皮瓣的方法进行修复,术中做冰冻切片决定手术切缘。结果:所有病例均一期修复,皮瓣存活良好,颜面部外观满意。1例面部其他部位再发基底细胞癌,再次行手术切除,未见复发。结论:面部基底细胞癌切除术后,采用术中冰冻切片快速病理验证的方式可以保证肿瘤的完全切除,应用局部皮瓣修复术后缺损,简便、高效,效果满意。     

手部鳞癌再次手术治疗体会

目的 探讨手部鳞状细胞癌再次手术扩大切除的范围及修复方法.方法 2009年9月至2011年9月,对7例手部皮肤肿物在基层医院按良性肿瘤切除、病理诊断为鳞状细胞癌的患者,再次行扩大切除术.其中6例手背肿瘤于距切口边缘扩大切除软组织2.0 cm,1例创面行植皮术、5例创面行前臂背侧皮瓣转移修复;1例拇指肿瘤行截指术.结果 术后创面均Ⅰ期愈合,随访9～33个月,指伸肌腱无粘连,肿瘤无复发.结论 手部皮肤肿物切除后应常规行病理检查,诊断为鳞状细胞癌的应再次扩大切除,创面根据具体情况行植皮或皮瓣转移修复,侵及深部组织的应行截指术.     

术中冰冻病理检查外科切缘在机器人辅助根治性前列腺切除术保护尿控和勃起功能方面的应用价值

目的探讨机器人辅助根治性前列腺切除术(RARP)中行冰冻病理检查外科切缘对于保护尿控功能和勃起功能的临床应用价值。方法回顾性分析2022年2月至2023年7月北京和睦家医院收治的11例前列腺癌患者的病例资料。中位年龄64(61, 69)岁。中位前列腺特异性抗原(PSA)8.0(6.0, 11.2)ng/ml。中位国际勃起功能指数(IIEF-5)评分17.5(10.3, 22.0)分。11例均无尿失禁。所有患者均经术前穿刺活检确诊为前列腺癌。有适应证的患者术前行PSMA PET/CT检查均确定无远处转移。11例均行RARP, 采用达芬奇Si系统, 4孔法, 经腹部前入路, 筋膜内切除技术。经镜头孔切口取出前列腺标本, 送冰冻病理检查外科切缘。等待病理结果时行尿道-膀胱颈吻合和盆腔淋巴结清扫。如冰冻病理报告为切缘阳性, 则切除阳性切缘相应位置的前列腺周围筋膜和神经血管束(NVB)。术中3例冰冻病理报告为切缘阳性, 切除单侧NVB;8例保留双侧NVB。术后随访患者的PSA、尿控功能和勃起功能。结果本组11例手术均顺利完成, 无中转开放手术。中位手术时间260(225, 285)min。中位估...     

结肠癌术后局部复发的现状和进展

结肠癌是消化系统最常见的恶性肿瘤之一.手术是结肠癌最主要的治疗方式,而术后局部复发是决定结肠癌患者生存率及生活质量的重要因素,并且是目前评价手术质量的一项重要指标.因肠梗阻和肠穿孔行急诊手术是局部复发的独立危险因素,T和N分期、肿瘤大体形态、神经血管是否受侵也是影响局部复发的重要因素.目前认为对于有适应证的患者行再次手术仍可获得较好的预后,全部切除复发肿瘤并且达到阴性切缘(R0切除)是获得长期生存的首要条件.再手术后切缘病理状况、局部复发部位、复发肿瘤数目及大小、肿瘤分期、术前血清CEA的水平、是否存在远处转移均是影响术后肿瘤特异性生存率的独立因素.     

耻骨后前列腺根治性切除术后尖部切缘软组织的分析

作者通过术中活检和组织切片对保留神经的前列腺根治术的前列腺尖部切缘软组织进行了分析。研究包括 95例临床诊断为前列腺癌的患者 ,经耻骨后前列腺根治切除术后 ,分别取尖部切缘2～ 3ｍｍ组织进行冰冻和永久切片分析。同一病理医生镜检整个前列腺及尖部标本以了解良恶程度。尿管拔除时和术后 3个月评价控尿能力。结果发现 ,2 6 %的患者手术切缘阳性 ,其中 6 4%前列腺尖部切缘阳性。永久切片尖部39%未见前列腺组织 ,5 4%为良性前列腺组织 ,7%为前列腺癌 ,3例患者由于冰冻切片标本阳性而进一步切除 ,直至切缘取得阴性结果。根治切除的标本中…     

中下段胆管癌切除后切缘阳性的意义及预后因素分析

目的 分析中下段胆管癌切除术后切缘阳性的意义,研究影响中下段胆管癌切除术后的预后因素.方法 回顾性分析1990年1月至2006年12月收治的79例中下段且日管癌切除患者的临床病理资料.其中男性53例、女性26例,年龄30～79岁,平均61岁.中段胆管癌34例,下段胆管癌45例.行胰十二指肠切除术46例,行根治性胆总管癌切除术25例,行根治性胆总管癌切除联合肝部分切除术6例,行根治性胆总管癌切除联合门静脉部分切除术2例.5例于术后1个月内死亡,对其余74例患者的15项临床病理特征进行单因素及多因素分析.结果 74例患者总的5年生存率为30.7%,中位生存期为36个月.术后病理榆查为镜下切缘阳性(R1切除)16例(20.3%),其中肝脏端胍管切缘阳性6例,远端胆管切缘阳性3例,双侧胆管切缘阳性2例,环周切缘阳性5例.接受R0和Rl切除的患者的5年生存率分别为34.4%和15.5%.10例(17.2%)R0切除的胆管癌出现局部复发,10例(62.5%)R1切除出现复发,差异有统计学意义(X2=13.024,P<0.01).单因素分析显示术前血红蛋白水平、分化程度、肿瘤浸润深度、淋巴结转移、TNM分期及手术切缘为影响预后的因素.多因素分析显示淋巴结转移状况和切缘癌残留是影响预后的独立因素.结论 中下段胆管癌根治术中冰冻病理检查切缘达R0切除是提高长期生存的重要策略,辅助治疗的效果尚待进一步研究.     

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。     

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.     

A rare variant of scalp dermatofibrosarcoma protuberans: malignant fibrous histiocytomatous transformation

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon, local aggressive tumor. Tumor metastasis is rare, and it has equal sex distribution. DFSP usually develops on the trunk and extremities. Scalp DFSP composes less than 5% of all cases. Local recurrence rates are high. Wide local excision and Mohs surgery are options for treatment. Fibrosarcomatous or malignant fibrous histiocytomatous transformation of DFSP are rare conditions. In this article, we described a case of a giant DFSP over the scalp containing malignant fibrous histiocytomatous transformation areas.     

Dermatofibrosarcoma: a rare form of soft tissue. Management and review of the literature

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor of cutaneous origin of intermediate grade malignant potential. The incidence of DFSP is 0.1% of all cancers and 1% of all soft tissue sarcomas. We present the case of a 65years old female with a palpable, painful mass on the right thigh. A surgical excision of the lesion was done and the histopathology, as well as the immunohistochemical analysis with CD-34, confirmed the diagnosis of DFSP. Two years later, the patient is free of disease and no local recurrences or metastases have been found. Wide radical excision is the preferred surgical method for therapy of DFSP without distant metastasis. Furthermore, DFSP resists to conventional chemotherapy and radiation therapy, while, in cases of metastasis, therapy depends on cytogenesis and molecular biology of the tumor, so new therapeutic strategies are under research.     

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: 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.     

Extensive angiosarcoma of the scalp involving the cranium

. A case of angiosarcoma of the scalp involving the underlying bone is presented. Wide local excision of the scalp and full-thickness excision of the underlying calvarium and dura followed by reconstruction with latissimus dorsi free flap and skin graft was performed. Despite an initial negative frozen section of the margins, the final pathologic specimen showed microscopic involvement of the inferior margin. The positive margin was re-excised accomplished with the aid of mapping biopsies. This case demonstrates the typical nature of this rare tumor in terms of the difficulty of histologic diagnosis and surgical management.     

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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。     

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.     

Dermatofibrosarcoma Protuberans: Recent Clinical Progress

Background Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor of low malignant grade characterized by a pattern of slow, infiltrative growth and a marked tendency to recur locally after surgical excision. Wide surgical resection is generally accepted as optimal treatment for DFSP. However, despite optimal surgical management, distant metastases may develop in up to 5% of patients. More than 90% of DFSP are characterized by a reciprocal chromosomal translocation, t(17;22). This rearrangement leads to constitutive activation of the platelet-derived growth factor receptor (PDGFR) as a result of deregulated ligand expression, thus providing a rationale for targeted inhibition of PDGFR as a treatment strategy for patients with unresectable locally advanced or metastatic DFSP. Methods This article reviews the current understanding of DFSP, with emphasis on molecular-level pathogenetic events and their implications for management, and evidence for the role of tyrosine kinase inhibition in improving the outcomes of patients with unresectable locally advanced or metastatic DFSP. Results Surgery with wide margins remains the cornerstone in the management of DFSP. Recently, imatinib, a potent, selective inhibitor of the PDGFR alpha and PDGFR beta protein-tyrosine kinases, has been reported to induce complete or partial remissions in most patients treated for advanced DFSP. Conclusions Imatinib is approved for treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP who are not eligible for surgery. Future investigations will determine whether imatinib can also be used in the neoadjuvant setting to reduce tumor volume, thereby allowing resection of very large DFSP that would otherwise not be resectable.