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。     

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。     

腺泡状软组织肉瘤临床病理及其组织发生的研究

报告３１例腺泡状软组织肉瘤。此瘤最常发生于年轻人下肢，５年生存率为５４．５％。２８例作免疫组化，２３例肌红蛋白阳性（８２．１％），１８例结蛋白阳性（６４．３％），３例Ｓ－１００蛋白阳性（１０．７％）；而神经原特异性烯醇化酶、神经微丝、溶菌酶、α１－抗胰蛋白酶、第Ⅷ因子相关抗原、角蛋白、上皮膜抗原、波形蛋白均为阴性．２例作电镜观察，瘤细胞浆内可见特殊的菱形结晶。作者认为腺泡状软组织肉瘤与肌细胞性肿瘤的常见类型之间可能存在着内在联系。     

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30例临床及病理分析

目的：探讨腺泡状软组织肉瘤（alveolar soft part sarcoma，ASPS）的临床病理学特征及其鉴别诊断。方法：对30例ASPS的临床资料进行回顾性分析，对标本进行HE染色，光镜观察，其中20例做免疫组织化学（SP法）染色结果：30例ASPS中，男性15例，女性15例；年龄5～67岁，平均30．2岁；病变主要位于下肢深部骨骼肌组织内。临床症状在软组织者主要表现为无痛性肿块，位于骨组织者均见骨质破坏。肿物呈结节状，部分为圆形、椭圆形，质实，切面黄白色到灰红色，常有大量出血和坏死。组织学可见具有特征性的腺泡状或巢状结构，其周围由薄壁样、窦隙样血管环绕，形成器官样结构；瘤细胞体积大，胞质丰富，可见伊红色细颗粒状及红染的棒状结晶；细胞核呈圆形、卵圆形或不规则形，染色质细颗粒状，着色较淡，并可见明显的核仁；可见近包膜处血管内瘤栓形成，肿瘤穿透包膜浸润周围骨骼肌组织；间质内纤维组织较少，病程长者纤维组织较多，并可发生透明变性免疫组织化学：desmin 10例阳性，actin 5例阳性，SMA 3例局灶阳性，vimentin 2例局灶阳性。CK、S-100均阴性。结论：ASPS多见于青少年和青年，生长缓慢，但多数早期出现血液转移，切除后易复发，最终预后欠佳结合临床和病理学特征，可作出正确的病理诊断。     

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。     

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。     

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.     

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。     

Successful treatment for solitary brain metastasis from alveolar soft part sarcoma

Following pulmonary metastases, alveolar soft part sarcoma has an unexplained predisposition to metastasize to the brain. Herein, a case of alveolar soft part sarcoma solitarily metastatic to the brain is described. A 23-year old female underwent the resection of alveolar soft part sarcoma from the right thigh. Three years after the surgery, a brain tumor was resected and pathology proved it was a metastatic alveolar soft part sarcoma. She is currently alive and well 6 years after brain surgery. Our report suggests that surgical resection for solitary brain metastasis from alveolar soft part sarcoma is an effective treatment modality.     

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.     

Alveolar soft part sarcoma

This paper presents the case history of a 22-year-old woman with an alveolar soft part sarcoma of the scapular region. Treatment consisted of resection. Cytostatic medication with adriamycin and dacarbazine (DTIC) failed to prevent rapid general metastatic growth.     

Cytopathology of alveolar soft part sarcoma

BACKGROUND:

Alveolar soft part sarcoma (ASPS) rarely is subjected to cytopathologic evaluation. With the exception of some very small series, the literature is limited to case reports. The objectives of the current study were to evaluate the cytomorphology of 10 ASPS cases on fine‐needle aspiration (FNA) or imprint cytology, review the literature, and highlight potential diagnostic pitfalls.

METHODS:

The authors searched their files for all lesions that were signed out as ASPS or suspicious for ASPS and searched the surgical pathology files for any cases of ASPS that had corresponding cytology. FNA was performed using the standard technique. Scrape preparations were performed on tissues that were sent fresh for frozen section examination.

RESULTS:

Ten cases of ASPS were retrieved from 7 patients (male‐to‐female ratio, 4:3; mean age, 22 years). All had subsequent tissue confirmation. Six specimens were from primary lesions, 3 specimens were from metastases, and 1 intraoperative smear was from a previously aspirated primary mass in a newly diagnosed patient. Anatomic sites of involvement included extremities (5 specimens), trunk/pelvis (3 specimens), oral cavity (1 specimen), and lung (1 specimen). Eight specimens were aspirates, and 2 specimens were intraoperative smears. Nine cases were diagnosed correctly as ASPS/consistent with ASPS, and 1 specimen was diagnosed as “tumor, not otherwise specified”. Cytomorphology included variably cellular smears composed of large cells with an enormous amount of finely granular or vacuolated cytoplasm, markedly enlarged nuclei/nucleoli, and bare nuclei. The cytomorphology of some cases revealed a strong similarity to renal cell carcinoma, clear cell type.

CONCLUSIONS:

The results of the current study indicated that ASPS has cytomorphology that overlaps with several other neoplasms, including renal cell carcinoma. Nonetheless, the morphologic features, when combined with the clinical presentation, radiologic findings, and ancillary testing, may allow for a specific diagnosis. Cancer (Cancer Cytopathol) 2009. © 2009 American Cancer Society.     

Alveolar soft part sarcoma metastatic to the breast

Metastasis to the breast is uncommon, with an incidence of 0.5–3%. Alveolar soft part sarcoma is rare, accounting for <1% of malignant soft tissue tumors, which are themselves unusual. Excluding contralateral breast and hematologic malignant disease, the primary lesion in most cases of metastasis to the breast is melanoma, small cell carcinoma of the lung, or ovarian carcinoma, although rhabdomyosarcoma is the most common primary tumor in children. We describe a 26-year-old woman with no history of malignant disease who presented with two masses in the right breast that clinical evaluations and ultrasonography indicated were fibroadenomas. Pathological studies after excisional biopsy, however, indicated alveolar soft part sarcoma. Subsequent computed tomography showed the primary tumor in the anterior left thigh and multiple bilateral lung metastases. Because of the presence of distant metastases, the patient was treated with chemotherapy. © 1996 Wiley-Liss, Inc.     

Pancreatic metastasis of alveolar soft-part sarcoma: A case report and review?of the literature

Alveolar soft-part sarcoma is a rare tumour. Patients commonlypresent with distant metastases both at the time of diagnosis and latein the course of disease. We present a case of pancreatic metastasis,occurring more than six years after diagnosis. Treatment consistedin subtotal pancreatoduodenectomy with pylorus resection. Both specificpatterns of relapse and treatment opportunities of this uncommon featureare discussed.