5例罕见部位腺泡状软组织肉瘤临床病理分析

分析5例罕见部位腺泡状软组织肉瘤的临床病理特征。     

宫颈原发性腺泡状软组织肉瘤临床病理观察

目的：探讨腺泡状软组织肉瘤（alveolar soft part sarcoma,ASPS）的临床病理学特征。方法：对1例ASPS进行大体、组织病理学和免疫组化染色观察,并复习相关文献。结果：患者,女,33岁,ASPS发生于宫颈。镜下肿瘤细胞排列成腺泡状,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。PAS染色,瘤细胞胞质内可见棒状结晶体。免疫组化：Vim阳性,desmin、SMA、myoglobin、CgA、S-100、EMA、AEI/AE3、CD68、和HMB45（-）全部为阴性。结论：宫颈ASPS是一罕见的恶性软组织肿瘤,结合临床病理学特征及免疫组化,可作出正确诊断。     

宫颈原发性腺泡状软组织肉瘤临床病理观察

目的:探讨腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征。方法:对1例ASPS进行大体、组织病理学和免疫组化染色观察,并复习相关文献。结果:患者,女,33岁,ASPS发生于宫颈。镜下肿瘤细胞排列成腺泡状,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。PAS染色,瘤细胞胞质内可见棒状结晶体。免疫组化:Vim阳性,desmin、SMA、myoglobin、CgA、S-100、EMA、AEI/AE3、CD68、和HMB45(-)全部为阴性。结论:宫颈ASPS是一罕见的恶性软组织肿瘤,结合临床病理学特征及免疫组化,可作出正确诊断。     

腺泡状软组织肉瘤三例报告

腺泡状软组织肉瘤三例报告广西医科大学附属肿瘤医院（南宁市５３００２７）朱小东，陈铭忠腺泡状软组织肉瘤临床少见，我院１９８５年以来共收治３例，现报告以下。１病例介绍例１．女性，５岁，左胸壁肿物切除术后３周于１９８９年５月入院。患儿于１９８６年１月起发现...     

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。     

大腿腺泡状软组织肉瘤临床病理分析及文献复习

目的:探讨腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征。方法:对1例ASPS进行大体、组织病理学和免疫组化染色观察,并复习相关文献。结果:患者女性,39岁,肿瘤发生于宫颈。镜下肿瘤细胞排列成腺泡状,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。结论:ASPS是一罕见的高度恶性软组织肿瘤,结合临床及病理学特征,可作出正确诊断。     

腺泡状软组织肉瘤13例临床病理分析及文献回顾

目的:探讨腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征、鉴别诊断、分子遗传学特点以及治疗与预后等。方法:回顾性分析13例ASPS患者的临床病理资料,总结其组织病理学特点、免疫组化等,并复习相关文献。结果:13例ASPS患者中男性8例、女性5例,男女比例为1.6∶1;发病中位年龄30岁。临床症状主要表现为深部软组织内肿块无痛性增大。典型的ASPS具有特征性的病理形态,即肿瘤细胞排列成腺泡状或器官样结构,细胞巢被纤细的窦隙样毛细血管包绕,瘤细胞体积大,界限清楚,胞浆丰富。PAS染色显示瘤细胞胞浆内见菱形、棒状结晶体。免疫组化:13例ASPS中MyoD1阳性率76.9%,且均为胞浆着色,其他抗体未见一致性阳性表达。结论:ASPS是一种罕见的组织来源不明的恶性软组织肿瘤,好发于年轻人。肿瘤生长缓慢,但最终预后较差。结合临床和组织病理学特征,可作出准确的病理诊断。     

纵隔原发腺泡状软组织肉瘤1例

腺泡状软组织肉瘤( alveolar soft part sarcoma,ASPS)是一种很少见的软组织来源的恶性肿瘤.其生长缓慢,早期症状不明显,通常发现时肿瘤已较大,而且容易早期转移.目前临床上对于ASPS认识不足,为提高对此肿瘤的认识及诊疗水平,现将我科1例纵隔原发ASPS患者的诊治经过报告如下.     

Activity of Pazopanib and Trabectedin in Advanced Alveolar Soft Part Sarcoma

Background

Alveolar soft part sarcoma (ASPS) is an exceedingly rare and orphan disease, without active drugs approved in the front line. Pazopanib and trabectedin are licensed for sarcoma treatment from second‐line, but very little and contradictory data are available on their activity in ASPS. Lacking ongoing and/or planned clinical trials, we conducted a multi‐institutional study involving the reference sites for sarcoma in Europe, U.S., and Japan, within the World Sarcoma Network, to investigate the efficacy of pazopanib and trabectedin.

Materials and Methods

From May 2007, 14 of the 27 centers that were asked to retrospectively review their databases had identified 44 advanced ASPS patients treated with pazopanib and/or trabectedin. Response was evaluated by Response Evaluation Criteria in Solid Tumors 1.1. Progression‐free survival (PFS) and overall survival (OS) were computed by Kaplan‐Meier method.

Results

Among 30 patients who received pazopanib, 18 were pretreated (13 with other antiangiogenics). Response was evaluable in 29/30 patients. Best responses were 1 complete response, 7 partial response (PR), 17 stable disease (SD), and 4 progressions. At a 19‐month median follow‐up, median PFS was 13.6 months (range: 1.6–32.2+), with 59% of patients progression‐free at 1 year. Median OS was not reached. Among 23 patients treated with trabectedin, all were pretreated and evaluable for response. Best responses were 1 PR, 13 SD, and 9 progressions. At a 27‐month median follow‐up, median PFS was 3.7 months (range: 0.7–109), with 13% of patients progression‐free at 1 year. Median OS was 9.1 months.

Conclusion

The value of pazopanib in advanced ASPS is confirmed, with durable responses, whereas the value of trabectedin appears limited. These results are relevant to defining the best approach to advanced ASPS.

Implications for Practice

This retrospective study, conducted among the world reference centers for treatment of sarcoma, confirms the value of pazopanib in patients with advanced alveolar soft part sarcoma (ASPS), with dimensional and durable responses, whereas trabectedin shows a limited activity. Alveolar soft part sarcoma is resistant to conventional cytotoxic chemotherapy. Pazopanib and trabectedin are licensed for treatment of sarcoma from second line; in the lack of prospective clinical trials, these results are relevant to defining ASPS best management and strongly support initiatives aimed at obtaining the approval of pazopanib in the front line of the disease.     

Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group

Background:Alveolar soft part sarcoma (ASPS) is a rare malignanttumor and little is known about its clinical features and management. Wereport on a series of 19 pediatric patients managed over 20 years. Patients and methods:Primary conservative surgery was performedin all patients and was radical in nine, non-radical in three; seven patientsunderwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In twocases radical surgery was performed after primary chemotherapy. Radiotherapywas delivered to 8 patients, chemotherapy to 15. Results:After a median follow-up of 74 months, the five-yearsurvival was 80% for the whole series, 91% for patients withlocalized disease, 100% for patients with tumor 5 cm, and31% for those >5 cm; 16 of 19 patients were alive (12 of 12 withgrossly-resected tumor in first continuous remission). Chemotherapy achievedtwo partial remission among seven evaluable patients. Conclusions:Pediatric ASPS has a more favorable prognosis thanits adult counterpart. In this series, tumor size correlates with metastaticdisease at onset and is the major factor influencing survival. Surgery is themainstay of therapy. The effectiveness of adjuvant therapy remains to beestablished, though radiotherapy may be advisable in cases of inadequatesurgery.     

肢体软组织肉瘤709例外科治疗分析

目的探讨肢体软组织肉瘤(STS)的诊断与规范化的手术方式,以提高治疗效果。方法回顾性分析行手术治疗并经病理证实的709例肢体STS患者的临床资料。结果全组709例肢体STS患者中,术前行B超709例,CT 135例,MRI 587例。本组除216例行补充广泛切除术患者术前未再行术前活检,其余493例均进行术前活检,其中细针穿刺活检350例,粗针穿刺活检89例,切检54例。手术方式:434例行广泛切除术,125例行根治性间室切除术,113例行屏障切除术,37例行截肢术。术后病理检查结果显示,恶性纤维组织细胞瘤103例,滑膜肉瘤89例,脂肪肉瘤78例,横纹肌肉瘤74例,隆突性皮肤纤维肉瘤67例,纤维肉瘤62例,平滑肌肉瘤46例,腺泡状软组织肉瘤41例,透明细胞肉瘤36例,韧带样瘤型纤维瘤病31例,上皮样肉瘤29例,恶性神经鞘瘤27例,骨外尤文肉瘤11例,骨外骨肉瘤10例,血管肉瘤5例。531例术后随访0.3~4.5 a,73例复发(13.7%),复发时间11~32个月,中位复发时间为21个月。127例(23.9%)出现远处转移,其中86例为肺转移,31例为肝转移,10例为骨转移。结论 MRI或CT应为术前常规的辅助检查,活检有助于明确诊断,手术是肢体STS最重要的治疗手段,规范化的手术方式可以降低复发率。     

15例腺泡状软组织肉瘤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,常伴有远处转移（肺转移,脑转移及骨转移等@。本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素。方法：回顾性研究了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例腺泡状软组织肉瘤患者的临床资料,并随访了他们的预后情况。15例患者中,女性9例,男性6例;小于或等于30岁者8例,大于30岁7例;肿瘤大于5cm者11例,小于5cm者4例;原发于软组织12例,原发于骨骼3例;未见其他部位转移6例,肺转移6例,骨转移3例,脑转移2例;术后局部复发2例;术后9例接受放疗,6例未接受放疗;术后接受化疗11例,未接受化疗4例,化疗方案MAID：多柔比星,氮唏米胺及异环磷酰胺。结果：患者预后情况较差,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%。伴有远处转移（肺转移、脑转移及骨转移@的患者5年生存率远低于整体生存率。患者个人性别、年龄对预后无显著性差异。肿瘤大小、部位及有无远处转移是影响预后的重要因素。结论：腺泡状软组织肉瘤是高度恶性软组织肿瘤。尽早评估病情,制定合适的治疗方案,原发病灶采取广泛切除,以降低复发及转移风险。但由于本组数据较少,放化疗是否有效还需进一步研究。