腺泡状软组织肉瘤的临床特点和治疗疗效分析

  目的  分析腺泡状软组织肉瘤的临床特点和治疗疗效。  方法  总结1962年5月至2008年8月接受治疗的腺泡状软组织肉瘤42例, 男性19例, 女性23例, 中位年龄27岁, 原发肿瘤部位以四肢为主(83.3%), 原发肿瘤中位直径为4.2 cm, 5例病例(12%)就诊时存在远处转移, 38例接受了原发肿瘤的扩大切除。  结果  中位随诊57个月, 全组5、10、15年总生存率分别为81.7%、63.6%、31.8%。随诊期间27例(64.3%)复发或转移, 其中单纯局部复发1例, 局部复发合并远处转移3例, 远处转移23例。远处转移病例中21例出现肺转移, 7例脑转移, 5例骨转移。远处转移到死亡的中位时间为36个月。20例转移病例接受了化疗, 6例还接受了肺内转移灶的切除, 有化疗疗效评价的14例中8例进展, 6例稳定。  结论  腺泡状软组织肉瘤发病年龄轻, 四肢多见, 病程发展缓慢, 手术为主要治疗手段腺泡状软组织肉瘤远期预后差, 容易出现肺、骨、脑转移。      

腺泡状软组织肉瘤的预后影响因素分析

目的探讨腺泡状软组织肉瘤的预后影响因素。方法回顾性分析1962年5月至2008年8月接受治疗的腺泡状软组织肉瘤患者42例,男性19例,女性23例,中位年龄27岁。肿瘤原发于四肢35例,原发于躯干或其他部位7例,原发肿瘤最大中位直径为4.2cm。就诊时局限的37例患者接受了原发肿瘤的扩大切除,其中10例还接受了术后的辅助放疗。结果患者中位随诊57个月,接受了原发肿瘤扩大切除的37例患者随诊期间局部复发4例(10.8%),远处转移20例(54.1%)。全组患者5、10、15年总生存率分别为81.7%、63.6%和31.8%;5和10年总生存率与性别、就诊时年龄(<27岁与≥27岁)、原发肿瘤部位(四肢与其他部位)明显相关,与原发肿瘤大小及术后辅助放疗的关系不明显,但原发肿瘤大小与远处转移明显相关。结论局部扩大切除为腺泡状软组织肉瘤主要治疗手段,患者就诊时年龄和原发肿瘤部位是腺泡状软组织肉瘤重要预后影响因素,原发肿瘤大小与远处转移密切相关,术后辅助放疗对生存率无明显影响。     

15例腺泡状软组织肉瘤的临床特点及预后分析

背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有肺转移、脑转移或骨转移等远处转移。本研究旨在初步探讨ASPS的临床特点,评估影响其预后的因素。方法:收集2003年1月—2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例ASPS患者的临床资料,对患者的性别(女性9例,男性6例)、年龄(≤30岁8例,>30岁7例)、肿瘤大小(直径>5 cm 11例,≤5 cm 4例)、肿瘤原发部位(软组织12例,骨骼3例)、有无远处转移(6例未见转移,9例有肺、骨或脑转移)、术后局部复发情况(2例)、术后是否接受放疗(9例接受放疗,6例未接受放疗)及术后是否接受化疗(11例接受化疗,4例未接受化疗,方案MAID:多柔比星,氮唏米胺,异环磷酰胺)等资料作回顾性分析,并进行随访。结果:患者性别、年龄对预后的影响差异无统计学意义(P>0.05),肿瘤大小、部位及有无远处转移是影响预后的重要因素(P<0.05)。15例患者随访时间为7～76个月,接受广泛或边缘切除后只有2例局部复发,但远处转移率较高。患者预后较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%。在本研究中,患者并没有从术后放化疗中受益。结论:ASPS是高度恶性软组织肿瘤。患者预后较差,伴有远处转移的患者5年生存率远低于整体生存率。但由于本研究病例较少,还需扩大样本量进一步深入研究。     

腺泡状软组织肉瘤的临床治疗分析

目的探讨腺泡状软组织肉瘤手术切除及辅助放、化疗的临床治疗效果。方法对1993～2006年间收治的腺泡状软组织肉瘤12例回顾性分析。男5例,女7例。年龄11～37岁。肿瘤直径平均5.5cm。结果12例手术后切除缘评价中,达到广泛切除缘者10例,边缘切除缘者2例,术后辅助放疗2例,全组均予辅助化疗。12例手术后复发3例,占25%。有7例发生转移,首诊时转移2例,其中肺转移瘤5例,脑转移瘤2例。转移率58.3%。转移瘤单纯化疗5例中PR3例,NC1例,PD1例,3例PR患者均采用MAI方案。随访时间10个月～10年6个月,12例中7例生存,5例死亡,经过Kaolan-Meire生存率计算,3年生存率81.2%,5年生存率52.8%。结论腺泡状软组织肉瘤外科治疗应以广泛切除术为基本原则,术后辅助放疗可以减少复发,辅助化疗是转移瘤的主要治疗方法。     

单中心高度恶性软组织肉瘤的诊疗经验

背景与目的：高度恶性软组织肉瘤是一类转移率高、预后差的恶性肿瘤。该研究总结单中心高度恶性软组织肉瘤的治疗经验,以指导制定这类肿瘤患者的个体化治疗方案。方法：对2000年7月—2014年7月在北京大学人民医院骨与软组织治疗中心接受手术及辅助治疗的473例高度恶性软组织肉瘤患者的临床资料进行回顾性分析,随访这些患者复发、转移及生存情况,分析影响预后的因素。结果：共有400例（84.6%）患者接受了保肢治疗。103例患者出现局部复发,148例患者出现肺、骨等远处转移,419例（88.6%）患者接受广泛性切除。370例患者接受术后化疗,153例患者接受局部放疗。平均随访时间为46.9个月（8.0~127.0个月）。随访期间114例患者死亡。3、5及10年总生存率分别为82.6%、69.0%及58.7%。统计学分析显示,肿瘤的组织学类型、辅助化疗及转移情况是影响患者预后的危险因素。结论：对高风险的肿瘤患者应进行积极的辅助治疗,包括通过化疗降低转移风险和局部放疗降低复发率,以期提高这些患者的生存率。     

腺泡状软组织肉瘤的临床和预后分析

目的探讨腺泡状软组织肉瘤(ASPS)的临床特点、治疗方法和预后情况。方法58例ASPS患者除6例就诊时即发现远处转移未行手术治疗外,其余52例均行手术治疗,其中19例行局部切除术,33例行扩大切除术。19例术后接受辅助性放疗或化疗。58例患者均获得随访,中位随访时间为52个月。结果50例肿瘤完整切除患者中,11例(22.0%)出现局部复发。全组58例患者中,31例(53.4%)发生肺转移。全组总的3、5和10年生存率分别为89.5%、74.1%和57.7%。中位生存时间为125个月。男性患者的3、5和10年生存率分别为79.6%、67.2%和49.7%,女性患者分别为100.0%、81.6%和65.3%(P=0.026)。结论ASPS恶性程度不高,肿瘤生长较为缓慢,局部复发率不高,但远处转移较为常见,肺是其最常见的转移器官。手术切除仍是目前治疗局限期ASPS的惟一有效手段。术后辅助性放化疗对控制局部复发和远处转移效果并不令人满意。女性患者的预后好于男性。     

54例原发鼻咽涎腺型癌的临床分析

目的 分析原发鼻咽涎腺型癌临床特点、治疗方法及预后因素。方法 回顾分析1963—2006年本院收治的 54例原发鼻咽涎腺型癌患者资料,其中腺样囊性癌 36例、黏液表皮样癌 11例、腺癌 7例,单纯手术 2例、单纯放疗 30例、综合治疗 22例(手术加放疗 15例,放疗加手术 7例)。8例接受化疗(术后辅助化疗 1例,同期放化疗 1例,复发后化疗 6例)。用Logrank法单因素分析。结果 随访率为89%。2、5年总生存率分别为85%、61%,无局部区域失败生存率分别为74%、55%,无远处转移生存率分别为92%、70%。治疗失败 35例,其中 26例局部区域失败(1例鼻咽复发加颈部淋巴结转移,23例鼻咽复发,2例颈部淋巴结转移)。发生远处转移 13例(4例出现多脏器转移),其中肺转移 10例、肝转移 3例、骨转移 2例、脑转移 1例、皮下转移 1例。单因素分析显示病理亚型与总生存率相关(χ²=15.29,P=0.000),而颈部淋巴结转移与无远转生存率相关(χ²=9.08,P=0.003)。结论 鼻咽涎腺型癌是一种局部侵袭性强、病程发展相对较慢的疾病,治疗上推荐手术与放疗的综合治疗。     

颅内转移性腺泡状软组织肉瘤4例临床病理分析

背景与目的：腺泡状软组织肉瘤颅内转移少见,容易与颅内其他原发性肿瘤混淆。本研究探讨4例颅内转移性腺泡状软组织肉瘤的临床病理特点、鉴别诊断以及预后情况。方法：复习4例颅内转移性腺泡状软组织肉瘤的病理切片及临床资料,并做免疫组化染色及PAS特殊染色,抗体包括Actin、Vimentin、Myosin、Myoglobin、S-100、Desmin、CgA、Syn、NSE、CK。结果：4例患者临床症状均主要表现为头痛及视物模糊等。颅内转移性病变均位于后颅窝．与脑膜关系密切。显微镜下肿瘤细胞胞浆透亮或嗜伊红,核仁明显,呈腺泡状排列,周围绕以血窦样结构。免疫组化染色2例表达Actin、Desmin和S-100,1例Vimentin和NSE弱表达。PAS染色4例均为阳性。1例术后1年内死亡,1例术后27个月死亡,1例30个月死亡,另1例术后已生存半年,目前无肿瘤复发。结论：颅内转移性腺泡状软组织肉瘤多数为转移性,需要与脑膜瘤、黑色素肿瘤、腺泡状横纹肌肉瘤以及副节瘤鉴别。患者预后差,术后5年生存率较低。     

46例首诊伴远处转移鼻咽癌的预后分析

目的 回顾性分析初诊时即伴有远处转移的鼻咽癌患者的治疗结果 并探讨其预后.方法 3年余共收治46例初诊伴有远处转移的鼻咽癌患者,其中43例为单器官转移,3例为多器官转移.肝转移19例,骨转移11例,肺转移7例,腋窝淋巴结和纵隔淋巴结各6例,脑转移1例.所有患者均接受了鼻咽部及颈部40～85 Gy放疗.41例接受了1～5周期PF方案化疗,23例接受了远处转移灶姑息性放疗.结果 随访率为100%.1、2、3、5年生存率分别为66%、47%、30%、19%,中位生存时间为20个月.转移灶是否放疗及KPS评分是影响生存的预后因素.转移灶放疗与末放疗的中位生存时间分别为39个月和13个月(X2=8.63,P=0.012),KPS评分≥80和<80的中位生存时间分别为26个月和12个月(X2=3.95,P=0.035).结论 首诊远处转移的鼻咽癌患者得到积极治疗后仍有长期生存可能,KPS评分高者预后好,在全身化疗、原发灶放疗及支持治疗的同时转移病灶局部治疗可能延长生存时间.     

三维适形放疗治疗骨与软组织肉瘤肺转移疗效分析

目的评价三维适形放疗对骨与软组织肉瘤肺转移患者的治疗价值。方法采用三维适形放疗技术治疗原发骨与软组织肉瘤肺转移患者7例,7例中孤立转移病灶1例,多发转移病灶6例,平均转移数目为3．4个。7例中4例接受化疗,2例拒绝化疗,1例因高龄未行化疗。放疗剂量为30—64Gy／7—30f／16—42d。结果7例的肿瘤靶区（GTV）体积为7．6～200．1cm^3,计划靶区（PTV）为89．2～523．9cm^3,患者的V20为6．31％～30．5％,平均肺剂量（MLD）为4—16．3Gy,放疗后CR3例,PR3例,SD1例。随访时间为4～32个月,其中4例存活,死亡3例,1例死于肺转移引起的呼吸衰竭,1例死于多发骨转移致截瘫后的坠积性肺炎,1例死于肾衰竭。7例平均存活13个月,按Kaplan—Meier寿命表法推断1、3年生存活率均为57．1％。放疗反应为1～2度,未出现2度以上的放射性肺炎。结论三维适形放疗治疗骨与软组织肺转移技术上可行,部分患者通过放疗可获得长期生存,与手术切除疗效相近,放疗反应可耐受。     

Salvage surgical resection after high-dose ifosfamide (HDIF) based regimens in advanced soft tissue sarcoma (ASTS): a potential positive selection bias--a study of the Spanish group for research on sarcomas (GEIS)

PURPOSE: To assess the impact of different factors on response rate (RR), time to tumor progression (TTP), and overall survival time (OS) in patients with locally advanced or metastatic soft tissue sarcoma (ASTS), included in three protocols with high-dose ifosfamide (HDIF). PATIENTS AND METHODS: One hundred fifty six ASTS patients included in three consecutive phase II trials with HDIF (>10 g/m(2)), alone or in combination with doxorubicin (DX), were analyzed. Cofactors were institution, trial, gender, age, performance status, histologic type, grade of malignancy, prior radiotherapy, presence of locoregional disease, metastatic site, salvage surgery, number of organs involved, and disease-free interval. RESULTS: By multivariate analysis performance status >0 and lack of salvage surgery correlated with a poorer survival. A good-risk and a poor-risk group were identified, with median survival time (OS) of 29, 5, and 10 months, respectively (P = 0.00001). The 1-, 2-, and 3-year OS for 83 good-risk patients (either with PS = 0 or receiving salvage surgery) was 83, 44, and 29%, respectively, those figures being 37, 7, and 3% for 73 poor-risk patients. CONCLUSION: The design of randomized trials in ASTS including HDIF should consider those prognostic factors as stratification variables.     

Malignant fibrous histiocytoma: an institutional review

Background: A thorough understanding of malignant fibrous histiocytoma (MFH), the most common subtype of soft tissue sarcoma, will lead to improved histologic-specific protocols. Methods: 126 patients with histologically confirmed MFH were analyzed. The median follow-up was 42 months (range 1-233 months). Results: Overall survival was 58% at 5 years and 38% at 10 years. Grade significantly influenced prognosis, with 10-year survival of 90%, 60%, and 20% for low, intermediate, and high grade tumors, respectively (p = 0.0007). Distant metastases at initial presentation (p = 0.0002) and size of the primary tumor (p = 0.0007) influenced outcome. Neither anatomic site nor depth of the primary tumor were significant prognostic factors. Positive microscopic margins were associated with a decreased disease-free survival (p = 0.006). Conclusions: Tumor grade, size, and distant metastases at initial presentation remain the most important prognostic factors for MFH. Resection with negative microscopic margins decreased the incidence of local recurrence.     

Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma. Brain metastases have been reported to be a common feature of Stage IV ASPS, and recent practice guidelines recommend routine intracranial imaging as part of the staging evaluation in all patients who present with ASPS. METHODS: The authors performed a comprehensive retrospective review of the clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized (American Joint Committee on Cancer [AJCC] Stages II/III) or metastatic (AJCC Stage IV) ASPS who presented to a tertiary care cancer center between 1959 and 1998. RESULTS: Seventy-four patients were identified from the database searches. The anatomic distribution of their primary tumors included: extremities, 44 patients (60%); trunk, 15 patients (20%); head and neck, 9 patients (12%); and retroperitoneum, 6 patients (8%). The median tumor size was 6.5 cm (range, 1.2-24 cm). The AJCC stage at presentation was Stage II or III in 35% of the patients and Stage IV in 65% of the patients. The 5-year actuarial local recurrence free, distant recurrence free, disease free, and overall survival rates among the 22 patients with localized ASPS were 88%, 84%, 71%%, and 87%, respectively. At a median follow-up of 9 years, 2 of 22 patients with localized disease had developed local recurrences and 3 had developed metastatic disease (all to the lung only). Brain metastases were noted in 9 of 48 patients who presented with Stage IV (M1) disease (19%) and always were noted in association with metastasis to other sites. The median survival of patients with M1 disease was 40 months, with a 5-year survival rate of 20%. CONCLUSIONS: Long term follow-up of patients with localized ASPS reveals a relatively indolent clinical course with relatively low rates of local and distant recurrence. In patients with Stage IV ASPS, brain metastases were observed only as part of more disseminated disease. The observations of the current study do not support current practice guidelines for the staging of patients with ASPS and suggest that selective rather than routine intracranial imaging should be used in patients presenting with ASPS.     

Alveolar soft-part sarcoma. A clinico-pathologic study of half a century

In the period from 1923 to 1986 our pathologists examined pathologic material from 102 patients with alveolar soft-part sarcoma (ASPS). Followup clinical data is available for 91. Median followup is 7 years (range 1 month to 27 years). Local recurrence was only found if residual disease was left at the time of the original excision. Survival in those patients who presented without metastases was 77% at 2 years, 60% at 5 years, 38% at 10 years and 15% at 20 years (median 6 years). No survival advantage could be demonstrated for patients who received chemo and/or radiotherapy, although numbers are small and staging not uniform. An evaluation by electron microscopy and immunohistochemistry cannot confirm recent claims that ASPS is a muscle tumor. ASPS is an unusual neoplasm; the primary therapeutic option is aggressive surgical excision. Survival even with the development of metastases can be long.     

Chemotherapy in alveolar soft part sarcomas. What do we know?

Alveolar soft part sarcoma (ASPS) is a rare tumour. Published series about treatment and outcome are scarce. Conclusive data about the response to chemotherapy are not available. The aim of this study was to analyse the efficacy of palliative chemotherapeutic treatment options and the incidence and mode of presentation of brain metastases. We retrospectively analysed our own sarcoma data-base and reviewed the literature. From our registry containing 757 patients, we identified 8 patients with ASPS. From the literature, 47 cases of adult patients and 13 children with sufficient data about chemotherapy were identified. Response to first-line chemotherapy in 68 patients was: complete remission (CR) 4%, partial remission (PR) 3%, stable disease (SD) 41%, progressive disease (PD) 51%. 285 patients with stage IV disease were evaluable for the analysis of metastatic sites. The incidence of brain metastases was 30.5% (87/285). Brain metastases were detected at a median interval of 48 months (range 0-396 months) after the primary diagnosis. Median survival after the diagnosis of brain metastases was 12 months. The median survival for patients with stage IV disease treated by chemotherapy was 36+ months (range 10-132 months) (31 patients evaluable) with a median follow-up of 46 months (range 10-135 months). ASPS shows a high incidence of brain metastases, at least 3 times higher than that of other soft tissue sarcomas. Chemotherapeutic regimens used for the treatment of other soft tissue sarcomas lack efficacy in ASPS. Staging investigations for ASPS should routinely include imaging of the brain. ASPS patients should not be treated with chemotherapy outside of controlled clinical trials. New targets for specific biologically-directed therapies need to be developed.     

腺泡状软组织肉瘤生物学特征及靶向治疗的研究进展

腺泡状软组织肉瘤(ASPS)是一种罕见的软组织肉瘤,具有高度侵袭性、易转移,患者预后差。ASPS对传统的化疗药物不敏感,虽然早期诊断和手术切除能有效改善患者预后,但是对发生转移的晚期患者,探索新型的治疗手段很有必要。分子靶向治疗在临床治疗中的作用越来越重要。ASPS的恶性表现主要是高度的血管生成效应,这是由血管生成因子过表达和过度活化造成。了解ASPS的生物学特征,包括其发生的分子事件,对于筛选新的分子靶向治疗方案很重要。本文就ASPS的生物学特征及靶向治疗的进展进行综述。     

Primary Alveolar Soft Part Sarcoma of the Scapula

Alveolar soft part sarcoma (ASPS) is an unusual soft tissue malignity, occurring in less than 1% of sarcomas and typically found in the head and neck tissues in children or, in adults, in the deep soft tissues of the lower extremities. In this report, we present a 33-year-old male with primary ASPS in the right scapular bone and discuss the radiologic features of this tumor in the context of the current literature.Key words: Alveolar soft part sarcoma, Scapula, Lung metastasis     

Pulmonary metastasectomy for osteosarcomas and soft tissue sarcomas

To evaluate the efficacy of aggressive pulmonary metastasectomy for treating osteosarcomas and soft tissue sarcomas, we reviewed 105 cases treated in our hospital between 1990 and 2002. There were 57 males and 48 females, 44 osteosarcomas (OS), 21 synovial sarcomas (Syno), 16 malignant fibrous histiocytomas (MFH), 4 leiomyosarcomas (Leio), 4 alveolar soft part sarcomas (ASPS) and 16 others (including chondrosarcoma and liposarcoma). A total of 904 metastases were resected (8.6(0-49)/patients) and 244 thoracotomies (2.3/patients) were performed. Mean number of initial metastasectomies was 3.7 (0-26), disease-free interval (DFI) was 13. 8 months (0-96), and mean diameter of maximum resected metastases for one patient was 20.4 (5-90) mm. Five-and 10-year survival rates of all cases were 44.9% and 32.0% respectively. Those of OS were 45.8% and 38.5%, and those of soft tissue sarcomas were 44.2% and 25.5%, respectively. The highest 5-and 10-year survival rates among soft tissue sarcomas was attained in ASPS and Leio (75%), and others (51.6% and 38.7%), followed by Syno (42.9% and 12.4%). Long-term survival was not attained in MFH (30.9%: 5 years). Analysis of histological types of the tumors and numbers of resected pulmonary metastases showed that the largest number of metastases were resected in ASPS (16/case) and Syno (13.8/case), followed by OS (9.0/case). MFH had the largest (27.1 mm) mean diameter of maximum resected metastases, followed by Leio (27.0 mm). Analysis of prognostic factors indicated that curativity was the most important prognostic factor: curative cases 42.2% (10-year survival) vs noncurative cases 4.2% (6-year survival). Number of resected metastases and mean diameter of maximum tumor size also affected the patient survival.     

Prognostic significance of grading and staging systems using MIB-1 score in adult patients with soft tissue sarcoma of the extremities and trunk

BACKGROUND: The predictive value of histologic grading and staging systems for overall survival in different types of adult soft tissue sarcoma of the extremities and trunk is unclear. METHODS: Histologic slides from 193 patients with primary tumors were reviewed for diagnosis, and Ki-67 (MIB-1) immunostaining was performed for grading in all patients. Univariate and multivariate analyses were conducted to analyze the results from patients with soft tissue sarcomas as a group and among the six main histologic categories: malignant fibrous histiocytoma (n = 49 patients), liposarcoma (n = 48 patients), synovial sarcoma (n = 30 patients), spindle cell sarcoma (n = 24 patients), small round cell sarcoma (n = 15 patients), and others (n = 27 patients). The median follow-up was 50 months. RESULTS: Univariate analysis of soft tissue sarcomas showed that tumor size and depth, histologic type, MIB-1 score, grades based on three criteria (tumor differentiation/histologic type, necrosis, and either mitosis or MIB-1 score) and disease stage, as assessed by tumor size, depth, and grade, were associated with overall survival. Among these variables, grading and staging systems using the MIB-1 score had better predictive value compared with the MIB-1 score and standard grading and staging models in the main histologic categories. Because survival curves for the different tumor grades and stages showed similar trends between the different histologic types, multivariate analysis was conducted adjusting for age, gender, disease site, surgical margin, tumor size and depth, grade, stage, and histologic type. Consequently, Grade 3 emerged as the most significant adverse prognostic factor. Additional adverse prognostic factors were Stage III, Grade 2, a histologic type of spindle cell sarcoma, and patient age > 50 years at the time of presentation. The histologic type liposarcoma was identified as a favorable prognostic factor. CONCLUSIONS: The current results indicate that grading and staging systems using the MIB-1 score are very strong prognostic factors in patients with the main histologic types of soft tissue sarcoma. Specific assessment of histologic type should be carried out before deciding on treatment strategies.