罕见纵隔脂肪肉瘤1例诊治体会及文献复习

纵隔脂肪肉瘤（mediastinal liposarcoma,MLPS）临床罕见,文献报道发生率占纵隔肿瘤0．13％～0．75％。自1916年Pallase首次报告以来,世界文献报告仅百余例,多为个例报道。本院收治1例临床罕见、合并大片骨化的多结节性纵隔脂肪肉瘤,并复习相关文献,讨论纵隔脂肪肉瘤的诊断方法、病理类型、手术方式、切口选择、手术要点及术后治疗,以期提供对这种罕见肿瘤的临床诊治参考。     

成人前列腺非多形性横纹肌肉瘤的文献回顾

成人前列腺横纹肌肉瘤发病率低且预后不良,国内外仅有小样本的回顾性研究和个案报道,本文借鉴相关临床研究对该疾病的治疗进展进行梳理,其总的治疗原则是多学科综合治疗,根据危险分层选择化疗方案与局部治疗(手术和/或放疗)相结合.除长春新碱+放线菌素D+环磷酰胺(VAC)方案外,多柔比星(ADM)、依托泊苷(VP-16)和伊立替康(IRI)为基础的方案为转移和复发患者提供了更多治疗机会,探索靶向和免疫治疗药物是未来的发展方向.     

纵隔黏液样脂肪肉瘤1例报告并文献复习

<正>黏液样脂肪肉瘤(Myxoid liposarcoma,MLPS)是脂肪肉瘤(Liposarcoma)中的一种亚型,占20%～30%,好发于下肢,以大腿多见,而发生在纵隔者较为罕见,好发年龄为30～50岁^[1-2]。目前,国内外发表的关于纵隔黏液性脂肪肉瘤的文献均为个案报道,其临床症状和影像学特征均缺乏特异性,临床诊断准确率较差。因此,为了加深对纵隔黏液样脂肪肉瘤的认识,提高诊断治疗水平,现报告我科收治的1例患者情况,并复习国内外1984年10月至2016年8月间相关的个案报道文献12篇,对其临床表现、诊断、治疗进行分析和总结。     

纵隔脂肪肉瘤4例报告

纵隔脂肪肉瘤临床上较少见.现将我院收治4例报告如下. 1 临床资料 收集1967年6月至2001年6月经我科手术病理确诊纵隔脂肪肉瘤4例进行报道. 病例1,男性,36岁,胸闷20天伴胸背部不适,咳嗽.     

纵隔脂肪肉瘤9例临床分析

目的 结合9例纵隔脂肪肉瘤病历探讨纵隔脂肪肉瘤的临床特点和治疗.方法 我院1970年～2007年共收治9例纵隔脂肪肉瘤患者,男性6例,女性3例,年龄3663岁.临床均以胸闷、气短为主要表现,均接受过手术治疗,术后病理分型:5例为以粘液为主的脂肪肉瘤,2例为以梭型为主的脂肪肉瘤,2例为低度恶性脂肪肉瘤.其中4例患者接受放疗.结果 死亡4例,其中3例死于局部复发.复发后再治疗3例,其中2例长期生存.结论 脂肪肉瘤为恶性间叶性肿瘤中较常见的一种,多来源于退化的胸腺组织或纵隔胸膜脂肪组织,发生在纵隔极为罕见,占所有脂肪肉瘤的2.7%,不到所有纵隔肿瘤的1%.其病理学特点:主要成分为未成熟的脂肪细胞和成熟的脂肪细胞,以及纤维组织和性液组织.纵隔脂肪肉瘤的治疗应首选手术治疗,关键是尽早发现并及时彻底切除肿瘤.脂肪肉瘤对放疗和化疗的敏感性很低,复发和转移是较常见的失败原因.     

原发性纵隔脂肪肉瘤破裂并血胸1例

患者女性，２９岁，因突发性右侧胸痛３天并加重伴气促１天，于２００１年６月１１日入院，表现为右侧肩背部放射性锐痛，大汗淋漓，心悸气促，头晕眼花等症状，胸部X光照片显示右侧大量胸腔积液，右胸闭式引流８００ml血性液后仍有持续血性液引出，以自发性血胸行右剖胸探查术。见右侧中纵隔有１３cm×１０cm×９cm实性肿瘤，表面可见较多粗大血管分枝，肿瘤下方有３cm×４cm破裂口并持续渗血，负压抽吸肿瘤内脂质样坏死组织使瘤体缩小，切除肿瘤体部，肿瘤根部无包膜，位于右侧肺上静脉根部及其周围，呈浸润性生长，局部仔细切除后用银钉…     

卵巢原发性多形性脂肪肉瘤病理分析及文献复习

目的：探讨多形性脂肪肉瘤（pleomorphic liposarcoma,PLS）的临床病理学特征.方法：对1例卵巢PLS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：肿瘤发生于一28岁女性患者卵巢.临床主诉为腹部肿块.镜下见肿瘤由多形性的梭形细胞及小圆形细胞组成,混有少量多核巨细胞.免疫组化：肿瘤细胞Vimentin、S-100阳性,desmin、SMA、myoglobin、EMA、AE1/AE3和HMB45均阴性.结论：卵巢PLS是一罕见的高度恶性软组织肿瘤,结合临床病理学特征及免疫组化,可作出正确诊断.     

纵隔脂肪肉瘤5例的外科治疗

纵隔脂肪肉瘤较少见。我院自 1992年 8月至 1999年 10月共收治 5例 ,现报告如下。一、临床资料1 一般资料 :本组 5例均为男性 ,年龄 12～ 75岁 ,平均43岁。 5例均经手术治疗 ,术后病理诊断为脂肪肉瘤。主诉症状 :胸痛 1例 ,吞咽困难 2例 ,呼吸困难 2例。胸部Ｘ线检查均为纵隔巨大肿物阴影 ,多明显突向胸腔 ,有吞咽困难症状者经食管吞钡造影可见外压性改变。ＣＴ检查均显示占据纵隔不同部位的低密度 (脂肪密度 )肿瘤影 ,ＣＴ值在 - 80Ｈｕ至 - 12 0Ｈｕ之间 ,个别局部可达 6 0Ｈｕ。肿瘤形状多不规则 ,最大径均在 10ｃｍ以上 ,最大者延及并完…     

纵隔脂肪肉瘤急性破裂1例报告

患者女,41岁。胸闷、憋气半年,加重伴咳嗽、有胸部疼痛2周;胸片发现纵隔巨大块影,于1994年11月22日以纵隔肿瘤收住院。检查:贫血貌,气管左偏,右侧肋间隙饱满,第4肋以下叩诊呈浊音,语颤及呼吸音减低。肝、脾未触及。X线胸片(图1,2)示,中纵隔向右胸腔内突出一巨大肿块影(约15cm×13cm),边缘光滑,密度均匀,心影左移,两肋间膈角清晰。B型超声波探查示,右前纵隔探得一个巨大(约10.5cm×10.0cm)弱回声光团,类圆形,边界清,其内为均质的细小光点。患者于同年12月3日晚突发行胸剧痛,胸部透视示右胸腔大量积液,原块影轮廓不清,考虑为肿瘤溃破所致。于次日晨在全麻下行右侧剖胸探查术。术中见瘤体已破溃,胸内有血性及胶冻样液2200ml;肿瘤表面不光滑,包膜不完整;瘤体为大量脂肪样组织,其内尚存较多胶冻样物。肿瘤与胸壁、心包及右肺中、下叶浸润性连,瘤蒂位于腔与左无名静脉间,瘤蒂直径2cm。分离粘连,将肿瘤全部清除,瘤蒂根部予以缝扎。切除肿瘤组重2100g。病理诊断:纵隔粘液型脂肪肉瘤(图3)。手术后右肺复张,症状消失,2周出院。术后6个月X线胸片见右肺上叶前段近胸壁处有约6cm×5cm密度增高影。经皮穿刺活检证实为转移性脂肪肉瘤,虽然给予了放疗和化疗,但终因衰竭而死亡,术后仅存活10个月。     

原发性纵隔精原细胞瘤伴纵隔肉瘤1例并文献复习

原发性纵隔精原细胞瘤是极为罕见的性腺外生殖细胞恶性肿瘤,且伴肉瘤成分的纵隔精原细胞瘤更罕见。该病临床表现无特殊性,影像学特征与其他纵隔肿瘤及纵隔型肺癌等难以鉴别,容易误诊。本文详细报道1例原发性纵隔精原细胞瘤伴纵隔肉瘤。通过分析患者诊疗过程,对该病作一综述。     

原发性恶性纵隔肿瘤的诊断及治疗

纵隔肿瘤种类繁多 ,鉴别肿瘤为原发性或继发性、良性或恶性 ,对制定治疗方案极为重要 ,作者综述了常见原发恶性纵隔肿瘤的常规诊断方法及纵隔镜检查、纵隔穿刺活检的临床价值 ,介绍了常见原发性恶性纵隔肿瘤外科处理以及放疗、化疗的临床应用     

Endometrial Carcinoma with Cerebellar Metastasis: A Case Report and Review of the Literature

Background: Endometrial cancer is the most common malignancy involving the female genital tract in the United States. There is a paucity of reports of brain metastases in this disease, and most of these reports emphasize that this pattern of dissemination is rare. Case: We present a case of a 63-year-old woman who had high-grade endometrial carcinoma treated with surgery and radiotherapy. She had three separate episodes of relapse in the lungs, the first two relapses being treated surgically. Chemotherapy was also administered following surgery for the first relapse. The third pulmonary recurrence was treated with chemotherapy and then consolidated with thoracic radiation. Four years from the date of diagnosis, and a few weeks after completion of thoracic radiotherapy, she had evidence of a solitary cerebellar metastasis. This was treated surgically and followed by whole brain irradiation. She died 6 months after this central nervous system diagnosis with systemic dissemination of her cancer. Conclusion: The existing literature on brain metastases from endometrial cancer is reviewed together with the patterns of spread of endometrial cancer. We call attention to the unusually long course of this patient. Partially successful treatment for metastatic disease may have predisposed eventual development of brain metastases. This occurrence reinforces reports emphasizing their increasing incidence in association with endometrial cancer.     

Primary Colonic Liposarcoma Causing Colo-colic Intusussception: A Case Report and Review of Literature

Introduction

Liposarcomas are most common soft tissue sarcomas usually seen in deep soft tissues of extremities and retroperitoneum. Although secondary involvement of gastrointestinal system can occur in cases of retroperitoneal tumours, liposarcomas primarily involving the gastrointestinal tract are extremely uncommon. Intusussception refers to telescoping of contiguous segments of intestine causing obstruction. Colo-colic intusussception is rare in adults and usually associated with a malignant etiology, usually epithelial malignancies (adenocarcinomas).

Discussion

Colonic liposarcomas leading to colo-colic intussusception are extremely rare, with only six cases reported in the literature.

Conclusion

In this report, we describe this rare occurrence in a 66-year-old lady and highlight the diagnostic difficulties such cases can pose along with a review of relevant literature.     

A Pleomorphic Rhabdomyosarcoma Mimicking an Inguinal Hernia: A Case Report and Review of the Literature

A 59-year-old male presented with a painful right inguinal swelling and deep vein thrombosis at the ipsilateral leg. An inguinal hernia was initially diagnosed, but during surgery a large mass was found anteriorly to the peritoneal sheaths. Histology revealed a high-grade pleomorphic rhabdomyosarcoma. The mass advanced rapidly, occupying the whole right iliac fossa and metastasizing to the lung. Despite first- and second-line chemotherapy, the patient deteriorated rapidly and died. Rhabdomyosarcomas should be managed in specialized centres as they have prognostic factors and histologic features still controversial and poorly clarified.Key words: Rhabdomyosarcoma, Inguinal tumour, Chemotherapy     

Multi-Focal Gliosarcoma: A Case Report and Review of the Literature

Summary Gliosarcoma (GS) is an uncommon malignant brain tumor with biphasic tissue pattern consisted of both glial and sarcomatous components. It usually occurs in adult population of middle age. We report a rare case of multi-focal GS that was initially interpreted as metastases of extra-cranial tumor. The histological examination revealed the biphasic pattern of a GS. The patient was treated with postoperative external radiation therapy and had poor prognosis. To our knowledge this is the second published case of GS with multi-focal presentation. In this study we also review the literature on clinicopathological aspects of GS.