腹膜后低度恶性肌纤维母细胞肉瘤的诊治

目的:探讨腹膜后低度恶性肌纤维母细胞肉瘤(LGMS)的临床特征及其诊治。方法:回顾性分析2000年1月～2010年5月我院收治3例经病理确诊的腹膜后LGMS患者的临床资料。其中男2例,女1例,患者平均发病年龄40.6(28～70)岁。并结合文献复习探讨本病的临床特征、组织病理学特点和诊治、预后。结果:本组3例均行完整的手术切除,切缘阴性。病理确诊3例均为腹膜后LGMS,随访2年未见复发。结论:腹膜后LGMS临床罕见,术前易误诊,确诊依赖于病理检查。综合分析临床资料,提高对此病的临床认识是避免误诊的关键。完整的手术切除和严密随访以避免复发。     

肝脏肌纤维母细胞肉瘤自发破裂一例

患者男性,29岁,既往无肝炎肝硬化病史,因"上腹部间断性疼痛4 d"入院,影像学检查提示右肝囊实性肿瘤。术前肺功能检查过程中因腹压增高诱发肿瘤破裂出血,急诊手术行肝肿瘤切除、胆囊切除术。术后病理提示肝脏肌纤维母细胞肉瘤。     

膀胱假肉瘤性肌纤维母细胞增生一例并文献复习

目的提高膀胱假肉瘤性肌纤维母细胞增生的诊治水平。方法报道1例膀胱假肉瘤性肌纤维母细胞增生并复习文献。患者女性,38岁,因“尿频、尿急、尿痛1月”入院,术前超声及CT提示膀胱右侧壁实性占位性病变。结果入院行膀胱镜检,见肿瘤位于膀胱右侧壁,球形凸向膀胱腔,质硬,基底宽,不活动,大小约4cm×5cm,被覆尿路上皮,呈滤泡样改变,无破溃出血。肿物电切两处活检,病理报告示膀胱假肉瘤性纤维母细胞增生。遂行膀胱部分切除术,见肿瘤侵犯膀胱全层,膀胱外脂肪组织少量浸润,病理证实为膀胱假肉瘤性肌纤维母细胞增生。结论膀胱假肉瘤性肌纤维母细胞增生为一种肿瘤性病变,部分病例呈低度恶性,病理可确诊,多采用外科手术治疗,术后应密切随访。     

胸骨肌纤维母细胞肉瘤一例

患者男性,52岁,农民.因"持续前胸部疼痛4个月,加重1个月"入院.疼痛位于胸骨柄,针刺样痛,夜间明显,不伴发热、咳嗽、咳痰、咯血等其他症状.入院查体:胸骨柄有明显压痛、叩击痛.实验室检查:血常规、血沉及C反应蛋白均正常.     

腹膜后巨大脂肪肉瘤一例

腹膜后脂肪肉瘤是一种少见的恶性肿瘤，早期不易发现，手术彻底切除较困难，术后易复发。现将我院收治的1例腹膜后巨大脂肪肉瘤报道如下。[第一段]     

腹膜后横纹肌肉瘤一例

横纹肌肉瘤(rhabdomyosarcoma,RMS)是儿童软组织肉瘤中最常见的一种,成人少见。RMS主要发生在头颈部,发生于腹膜后者较罕见。其恶性程度高,发生于成人的RMS预后更差。尤其是腹膜后RMS早期无特异性症状,不易被发现,一旦出现症状已属中、晚期,治疗难度相当大。本文报道1例腹     

肌纤维母细胞肉瘤(附2例报告并文献复习)

[目的]总结肌纤维母细胞肉瘤的临床及组织病理特点,探讨其诊疗方法及预后情况。[方法]分析2例罕见的肌纤维母细胞肉瘤的临床特征、组织病理学特点、诊断治疗方法,并对患者的预后情况进行随访。同时复习近年来的国内外相关文献。[结果]患者1在活检诊断后,行大腿中段截肢术,术后在当地医院行化疗(具体方案不详)。术后1年,患者因肺转移,死亡。患者2行肿瘤切除术,术后3个月肿瘤复发,再次行手术切除并行化疗,术后11个月发生肺转移,3个月后死亡。两例最终病理诊断均为肌纤维母细胞肉瘤。复习近30年的文献,共有54例同样的病例报道。患者中以成年男性多见,肿瘤好发于头颈部、躯干以及四肢。逐渐增大的无痛性包块是其最常见的临床表现。依照特殊的形态学特征及肌源性标记物,即可以作出诊断。[结论]肌纤维母细胞肉瘤是一种罕见的恶性梭形细胞肉瘤,具体发病率不详。诊断时应与滑膜肉瘤、平滑肌肉瘤和纤维肉瘤等鉴别。治疗以手术切除为主,化疗效果不肯定。该肿瘤为低度恶性,具有侵袭性,术后局部复发率高,可以发生远处转移。预后尚不肯定。     

腹膜后肌纤维母细胞瘤1例

<正>病人男性,61岁。以主诉"间断右上腹疼痛4年"于2008-10-14入院。查体:上腹部轻压痛,未及腹部包块。腹部CT示慢性胆囊炎,胰腺尾部上方见类圆形高密度结节影,CT值1357Hu,最大直径5cm,内见不均匀钙化,增强扫描病灶无明显强化(图1)。MRCP示胆囊结石和胆囊炎,胰尾部上方见类圆形长T1短T2信号影,边界清晰,其内见环状高信号影。术前诊断:(1)胰腺肿物,性质待查;(2)慢性     

A case of retroperitoneal extraosseous osteogenic sarcoma

A case of primary osteogenic sarcoma in retroperitoneal space is reported. A 33-year-old man was admitted to our hospital with complaints of left flank pain. On examination, a large tumor was found in the left retroperitoneal space. After resection of the tumor, the patient was treated with combined chemotherapy by the regimen of cyclophosphamide, vincristine, adriamycin and dimethyl triazeno-imidazole carboxamide (CYVADIC). He has been well for more than ten months without recurrence. Extraosseous osteogenic sarcoma of the soft tissue is extremely rare, and only 7 cases have been reported in Japan. In general, the prognosis of patients has been very poor with surgery, radiotherapy or chemotherapy alone. A suitable combination chemotherapy or radiotherapy or both following radical surgery should be performed for prolonging survival, and even obtaining a possible cure.     

A case report of retroperitoneal malignant schwannoma

A case of retroperitoneal malignant schwannoma is reported. The patient was a 66-years-old man who was referred to our clinic because of an abdominal lump. CT scan and renal arteriography pointed out right renal cyst, but the tumor was separate from the right kidney at operation. Pathological diagnosis of this retroperitoneal tumor was malignant schwannoma. After 10 months he developed local recurrence and had transabdominal excision. He has been doing well 12 months after the second operation. Thirty-one cases of retroperitoneal malignant schwannoma in Japan are reviewed.     

复发性腹膜后恶性纤维组织细胞瘤一例报告

正恶性纤维组织细胞瘤(malignant fibrous histiocytoma,MFH)指起源于原始间叶组织的恶性肿瘤,常见于四肢深部软组织,而腹膜后者少见。本文报告一例我院诊治的复发的腹膜后恶性纤维组织细胞瘤。临床资料患者女性,74岁。2015年3月因"洗澡时发现右上腹包块5 d"入院,患者既往有腰部钝痛不适,无     

Rare case of retroperitoneal malignant Schwannoma

Malignant Schwannoma, recently renamed malignant peripheral nerve sheat tumor retroperitoneally localized, represents 0.01 of retroperitoneal tumours. A 41-year old woman, without pathological record--and without cutaneous neurofibromatosis--hospitalized for increased volume of the abdomen, without symptomatology, is diagnosed after the imaging and biological tests--without CT and RMN--with retroperitoneal tumour. The unusual size of tumour--6000 gr.--the macroscopic aspect suggesting malignancy (histopathologically and immunohistochemically confirmed), the relatively easy extirpation which, nevertheless, required caudal spleen- and pancreatectomy as well as transvers colectomy, the absence of proximity adenopathy and remote secondary determinations, the simple postoperative evolution represent overwhelming elements in this case. Two years after the operation, during which the patient was under chemotherapy, on a routine control we found a relapse at a distance from the initial tumour (primitive tumour ?), totally operable. For the time being, after almost five years from the first operation, there are no clinical, biological and imaging changes. Conclusions: the retroperitoneal space is quite enough for the development of large tumour masses, without symptomatology. The present case combines most characteristics of retroperitoneal neoplasms: large or very large size, quasi-absent symptomatology, difficulty in preoperative diagnosis, surgical tactics and techniques--quite often, the total extirpation of tumour mass led to the sacrifice of other organs within the limits of a justified risk--and unforeseeable evolution with relapses having the same characteristics.     

Low-grade myofibroblastic sarcoma of the palm

We report here a case of an unusual spindle cell tumour of the palm with myofibroblastic differentiation, which was surgically excised. Histologically and immunohistochemically, it was a low-grade myofibroblastic sarcoma. After 25 months follow-up the patient is well and free of recurrence.