左股骨下段原发性恶性纤维组织细胞瘤脑室内转移1例报告及文献复习

目的: 探讨骨原发性恶性纤维组织细胞瘤(MFH)的临床特点及诊断.方法: 报告1例原发性股骨下端MFH并脑室转移病例结合文献复习.结果: MFH临床表现无特征性,临床及影像学诊断困难,MFH的诊断主要依赖于组织病理学资料,治疗后易出现局部复发和远处转移.结论: 若患者出现骨痛,局部肿块,影像学上如出现虫蚀状或大片状溶骨性破坏,巨大软组织肿块,无骨膜反应,应考虑到本病.     

软组织恶性纤维组织细胞瘤——临床变化与组织学恶性程度之间的关系

Stout和Lattes在1967年对软组织恶性纤维细胞瘤(malignant fibrous histoegtoma,MFH)进行了定义。此后,又有几种类型的肉瘤重新分类为MFH。最近的报导中,MFH是软组织肉瘤中最常见的类型。其预后不仅与组织学恶性程度,而且与肿瘤的大小、深浅和发生的部位都密切相关。作者对瑞典南部130万居民1964～1978年已确诊的全部软组织肉瘤(278例)进行了分析,对所有组织学材料进行了重新评价。肿瘤的恶性程度分为4级,其中Ⅳ级恶性程度最高。根据Weiss和Enzinger的     

肺原发性恶性纤维组织细胞瘤

 恶性纤维组织细胞瘤(MFH)是一类好发于成人深部软组织的多形性肉瘤,多见于四肢、躯干、腹腔和腹膜后,肺原发少见。该瘤是近年渐被注意的一类预后不良的软组织肿瘤。最早发现于肢体软组织.继而发现于骨组织。     

肺原发恶性纤维组织细胞瘤20例

背景与目的肺原发恶性纤维组织细胞瘤是一种罕见的肿瘤.本文研究肺原发恶性纤维组织细胞瘤的临床特点及预后影响因素.方法回顾性分析肺原发恶性纤维组织细胞瘤20例的临床特点、治疗方式及生存情况等资料,采用SPSS 16.0统计学软件,用Kaplan-Meier法及COX回归分析性别、肿瘤大小、肿瘤位置及化疗对术后生存期的影响.结果肺原发MFH的临床症状主要有咳嗽咳痰、痰中带血、胸痛、发热和胸闷.本组患者1年生存率为55.0%,2年生存率为25.096,死亡原因多为局部复发及远处转移.肿瘤的大小和位置是肺原发MFH总生存率的独立预后因素.结论肺恶性纤维细胞瘤恶性程度高,预后差.临床治疗以手术为主,术后辅助化疗疗效尚不确切,肿瘤大小及位置和预后相关.     

肺转移性恶性纤维组织细胞瘤3例

[目的]探讨肺转移性恶性纤维组织细胞瘤(MFH)的临床影像学特征、治疗及预后。[方法]收集3例MFH伴肺转移病例,并进行相关文献复习。[结果]3例均为男性,1例64岁,因胸痛伴咯血入院,病理证实骨MFH伴肺转移,表阿霉素联合环磷酰胺化疗后症状缓解,肺部病灶明显吸收,6个月后骨MFH复发。1例42岁,因胸闷气急入院,病理为皮下炎症型MFH,肺部、纵隔、腹膜后均见转移灶,很快死亡。1例65岁,因咳嗽、低热、胸痛入院,病理证实肾MFH伴肺转移,手术及放疗治疗后恢复可,9个月后死于广泛转移。[结论]MFH易发生肺转移,肺转移性MFH临床及影像学表现多样,对于肺转移的MFH,尚无可靠的治疗方案,表阿霉素、异环磷酰胺联合化疗可能起到缓解症状、提高患者生活质量的效果。     

儿童软组织恶性纤维组织细胞瘤

软组织肉瘤构成儿童大部分恶性间叶肿瘤,其中以横纹肌肉瘤为最常见,发病年龄通常在21岁以下。但是,恶性纤维组织细胞瘤(MFH)则常见于成年以后,多见于手、足、腹膜后或肾盂。有关儿童的MFH,已发表的资料很少,又因少见,故在儿科领域内的发展及治疗尚不清楚。美国费城儿童医院癌症研究中心,于1975年1月至1983年7月收治121名患有躯体软组织恶性肿瘤的儿童。7例为MFH,患儿在诊断时年龄为6月至11岁(平均3岁),均为男性,其中5例白人,2例黑人。     

LN-R mRNA及其蛋白在恶性纤维组织细胞瘤中的表达

目的 探讨层粘连蛋白受体(laminin receptor,LN-R)mRNA及其蛋白在恶性纤维组织细胞瘤(malignant fibrous histiocytoma,MFH)组织中的表达及意义.方法 采用原位杂交和免疫组化法检测LN-R mRNA及其蛋白在65例MFH组织的表达,并与10正常纤维组织对照.结果 LN-RmRNA及其蛋白阳性表达率分别为60﹪和66.2﹪.LN-RmRNA及其蛋白阳性表达率与MFH的分级、临床分期及预后密切相关(P＜0.05);与性别、年龄、组织学类型无关(P＞0.05).LN-R mRNA及其蛋白阳性表达具有明显的相关性(P＜0.01).结论 LN-R与MFH的发生有关,能较好反映MFH的恶性生物学行为,作为判断其预后的参考指标.     

恶性骨纤维细胞瘤的预后因素

恶性骨纤维细胞瘤(MFH)是一种罕见的肿瘤,其预后至今仍有争议。对34例骨MFH 患者做了临床病理检查,确定影响预后因素。 该病最常见的发病部位是股骨(15例)。其次为骨盆(6例)、胫骨(5例)和腓骨(5例)。可随访的29例患者,其中接受适当手术治疗的17人5年生存率63％,其余12例,包括5例接受姑息性手术治疗的骨盆MFH患者,5年生     

卵巢恶性纤维组织细胞瘤1例

恶性纤维组织细胞瘤(MFH)是来源于原始间叶组织的恶性程度极高的软组织肉瘤,好发于肢体[1],原发于卵巢者极为罕见.现将经病理证实的卵巢MFH,报道如下.     

喉部恶性纤维组织细胞瘤1 例

恶性纤维组织细胞瘤( malignant fibrous histocytoma ,MFH )是起源于间叶组织的恶性肿瘤, 1964 年由O 'Brien 首次描述.MFH 可发生于任何年龄,多见于四肢、躯干深部软组织和腹膜后区,发生于喉部的较少见.现报告两次误诊为声带息肉的病例1 例.     

Postradiation malignant fibrous histiocytoma and osteosarcoma of a patient with high telomerase activities

BACKGROUND: An extremely rare case of postradiation malignant fibrous histiocytoma (MFH) and osteosarcoma (OS) secondary to radiation therapy for leukemia-related osteolytic lesions is presented. In addition, the telomere biology of these tumors was investigated. CASE REPORT: A 14-year-old boy was diagnosed with acute lymphocytic leukemia. The right tibia was irradiated at a total dose of 60 Gy, and the left tibia was irradiated at a total dose of 40 Gy. The left tibia developed MFH and the right tibia developed OS. RESULTS: Telomere reduction (MFH 70.2, OS 70.0%) and high telomerase activities (MFH 12.1, OS 17.7 TPG) were observed. These results reflect an aggressive feature of postradiation sarcomas. CONCLUSION: Prognosis for patients diagnosed with postradiation sarcoma is poor due to its aggressiveness. However, even if sarcoma occurs after irradiation in more than two fields in a single patient, improvements in prognosis are anticipated with appropriate chemotherapies and wide resection.     

腹部恶性纤维组织细胞瘤47例临床和预后分析

目的探讨腹部恶性纤维组织细胞瘤(MHF)的临床病理特点、治疗策略及预后相关因素。方法回顾性分析1970年1月至2000年1月间天津医科大学附属肿瘤医院47例腹部恶性纤维组织细胞瘤患者手术切除治疗情况。所有患者均经手术病理证实,病变单一。结果术后随访中共有32例患者出现复发、转移,术后单纯局部复发13例(29.5%),出现肺及胸膜转移7例(15.9%),肝转移4例(9.0%),骨转移6例(13.6%),肾脏转移2例(4.5%),复发 转移5例(11.4%)。术后1,3,5年无瘤生存率分别为65.9%、40.9%和29.5%,累计生存率分别为70.5%、52.3%和31.8%,中位生存期为18个月。影响预后的主要因素为肿瘤发生的部位、治疗方式、病理类型、肿瘤的局部复发等。以手术为主的综合治疗较单纯手术及放化疗预后好,术后辅以放疗可降低局部复发率,尤其对那些切端阳性患者。结论腹部MHF侵袭性强,复发率高,病理分级差,以手术切除为主辅以放疗有助于提高患者的远期生存率。     

iNOS-dependent DNA damage in patients with malignant fibrous histiocytoma in relation to prognosis

Malignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas. MFH has been proposed to be a lesion accompanied with inflammatory responses. During chronic inflammation, reactive nitrogen and oxygen species generated from inflammatory cells are considered to participate in carcinogenesis by causing DNA damage. 8-nitroguanine is a mutagenic nitrative DNA lesion formed during chronic inflammation. We examined whether nitrative DNA damage is related to the prognosis of MFH patients. We performed immunohistochemical analyses to examine the distribution of DNA damage and the expression of inflammation-related molecules including inducible nitric oxide synthase (iNOS), nuclear factor-kappaB (NF-kappaB), and cyclooxygenase-2 (COX-2) in clinical specimens from 25 patients with MFH. We also analyzed the correlation of DNA damage or the expression of these genes with the prognosis of MFH patients. Immunohistochemical staining revealed that the formation of 8-nitroguanine and 8-oxo-7,8-dihydro-2'-deoxyguanosine (8-oxodG), an oxidative DNA lesion, occurred to a much greater extent in MFH tissue specimens from deceased patients than in live patients. iNOS, NF-kappaB and COX-2 were colocalized with 8-nitroguanine in MFH tissues. It is noteworthy that the statistical analysis using the Kaplan-Meier method demonstrated strong 8-nitroguanine staining to be associated with a poor prognosis. In conclusion, 8-nitroguanine appears to participate in not only the initiation and promotion of MFH, but also in the progression of MFH, and could therefore be used as a promising biomarker to evaluate the prognosis of cancer patients.     

软组织恶性纤维组织细胞瘤术后辅助放化疗预后因素分析

、68.0%、53.0%和62个月.单因素分析显示肿瘤大小、分期及术后放疗等均和预后明显相关,而性别、年龄、肿瘤部位、术后化疗对总生存率无影响,多因素回归分析显示术后放疗为独立预后因素.单纯化疗、单纯放疗及化疗+放疗组疗效相似,但它们均优于无放化疗组.结论 体积较小、术后无转移的肿瘤预后较好,而术后治疗以术后辅助放疗者预后最好.     

Primary malignant fibrous histiocytoma of the lung. A clinicopathologic and ultrastructural study of five cases

Primary sarcomas of the lung are rare malignant neoplasms which have usually been classified as fibrosarcomas or as leiomyosarcomas. Only five cases of primary malignant fibrous histiocytoma (MFH) of the lung have previously been reported in the literature. The authors reviewed 10 cases of primary pulmonary sarcoma of the lung seen at their institution during the last 12 years, and five of these cases met the morphologic criteria for the diagnosis of MFH. The morphology in these cases is identical to MFH of other body locations, and shows a combination of fibroblast-like and histiocyte-like cells at both the light and electron microscopic level. By electron microscopic examination there was no evidence for anaplastic carcinoma, rhabdomyosarcoma, or leiomyosarcoma. Of the three patients with adequate follow-up, one patient is alive without evidence of disease at 10 years, one died at 5 years without evidence of recurrent sarcoma, and one died at 7 months with liver metastasis. Two other patients are alive without recurrence at 8 months and 1 year, respectively, following surgery. Because MFH is primarily a tumor of the soft tissues of the extremities, patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin. The reported prognosis in patients with primary MFH of the lung is poor, but early surgical resection does offer the possibility of a cure.     

纵隔原发性恶性纤维组织细胞瘤

为了研究原发性纵隔恶性纤维组织细胞瘤的临床病理特点。采用常规石蜡包埋ＨＥ 染色及免疫组织化学方法研究３例ＭＦＨ。３ 例ＭＦＨ 均位于中纵隔,肿瘤界限清楚有包膜或假包膜,术后无局部复发及转移,最长无瘤生存者已达９ ａ。纵隔原发ＭＦＨ的病理特点与软组织ＭＦＨ 相同,但局部复发率及转移率低,提示其预后好于软组织ＭＦＨ。     

乳腺恶性纤维组织细胞瘤1例报告并文献复习

目的:探讨乳腺原发性恶性纤维组织细胞瘤(MFH)的临床病理学、免疫组化特征和预后。方法:对手术切除一例乳腺MFH进行病理常规切片及免疫组化观察,并结合文献复习。结果:瘤组织由纤维细胞、纤维母细胞、黄色瘤样细胞组成。免疫组化CD68、Vimentin阳性表达,Ki67增殖指数38.38%。结论:MFH根据其分类及亚分类、分级、瘤体大小、部位、肿瘤深度,评估预后。     

A single-team experience of limb sparing approach in adults with high-grade malignant fibrous histiocytoma

Malignant fibrous histiocytoma (MFH) is the most common subtype of soft-tissue sarcoma (STS). When located in a limb, MFH, is currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT). During 8 years, 42 adult patients with high-grade limb MFH were approached by LSS and RT. Our results reflect a single-team experience and point to several important conclusions. High grade MFH, treated by conservative approach, lead to a 10-year relapse-free survival of 62% and a 10-year overall survival rate of 80%. Recurrences of MFH tend to occur during the first 2 years. Relapse-free survival was affected mainly by location in the lower limb vs. the upper limb, irrespective of the tumor size. Patients who had their diagnostic biopsies in another medical center had a greater tendency to local and systemic relapse. It seems that the most important clues for disease-free survival are the team experience and cooperation. All other factors are tumor-biology dependent, and thus far are beyond our control.     

Malignant fibrous histiocytoma of the lung

Twenty-two cases of primary malignant fibrous histiocytoma (MFH) of the lung are described, and a review of the literature is presented. As in the soft tissue, this sarcoma is found primarily in older individuals, usually as a solitary peripheral mass. Chest wall invasion at diagnosis was seen in four cases. The most common histologic subtype was the storiform-pleomorphic type of MFH. Vascular invasion was seen in 50% of cases. Histologic features did not correlate with prognosis, and subsequent aggressive behavior was related primarily to symptomatic presentation, advanced stage at diagnosis, incomplete excision, invasion of the chest wall or mediastinum, and subsequent recurrence or metastasis. Primary MFH of the lung should be distinguished from spindle cell carcinoma and inflammatory pseudotumors, and this histologic differential diagnosis is discussed.