纵隔促纤维增生性小圆细胞肿瘤1例并文献复习

目的:探讨纵隔促纤维增生性小圆细胞肿瘤（DSRCT）的诊断、治疗及预后。方法:报告1例我科收治的纵隔DSRCT,结合以往报道的文献探讨纵隔DSRCT的诊断方法,以减瘤术、放化疗及靶向治疗等综合治疗为主的原则及预后。结果:回顾分析1例纵隔DSRCT患者,行诊断性穿刺检查,从影像学、病理学等确诊为纵隔DSRCT,并给予相应化疗及放疗方案,患者预后较差且病情进展迅速,结合以往文献分析并讨论DSRCT的确诊方法、治疗策略及预后情况。结论:DSRCT是一种罕见的间质组织恶性肿瘤,影像学无特异性表现,确诊多依靠组织学及基因检测结果,该疾病具有侵袭性高,进展快,复发率高等特征,有效的减瘤术及综合性联合治疗可达到部分缓解或提高患者生存时间。     

腹腔促纤维组织增生性小圆细胞肿瘤1例并文献复习

腹腔促纤维组织增生性小圆细胞肿瘤(Intraab-dominal desmoplastic small round cell tumor,IAD-SRCT)是一种近年来才有报道的极少见、高度恶性肿瘤。自Gerald1991年报道第1例以来至今全世界报道仅130余例[1],国内仅有个例报道[2-5]。本文将报道我院首例IADSRCT,并对国内外相关     

肝脏促结缔组织增生性小圆细胞肿瘤(1例报告并复习文献)

目的　探讨促结缔组织增生性小圆细胞肿瘤 (DSRCT)的临床特点、诊断及治疗。方法　回顾分析 1例原发于肝脏的促结缔组织增生性小圆细胞肿瘤的诊治经过及近期随访 ,并结合复习文献。结果　本例病理获正确诊断 ,肿瘤原发部位及所浸润器官联合切除并辅以化疗 ,随访 12个月效果良好。结论　充分认识和掌握 DSRCT的特点是临床诊断的关键 ;彻底切除肿瘤并辅以化疗可获良好效果。     

直肠小细胞未分化癌2例报告并文献复习

 直肠小细胞未分化癌罕见, 国外报道不多, 国内未见报道, 占直肠恶性肿瘤不足1%。 著者报告2例, 均经病理证实。 本文就其临床表现、诊断、病理、发病原因、治疗及预后进行讨论, 并强调对其积极进行包括手术、放疗、化疗等综合治疗。     

肺促纤维增生性小圆细胞肿瘤1例并文献复习

促纤维增生性小圆细胞肿瘤(desmoplatic small round cell tumor,DSRCT)是一种恶性程度较高的肿瘤,多见于腹腔及盆腔,其它一些部位如胸膜、睾丸、颅内、肝、卵巢等亦有报道[1 ].1989年,GERALD和ROSAIL报道了第一例发生于腹腔内的促纤维组织增生性小细胞肿瘤,并于1991 年...     

横结肠促纤维组织增生性小圆细胞肿瘤1例

促纤维组织增生性小圆细胞肿瘤(Desmoplastic small round cell tumor,DSRCT)是一种近年来鲜有报道的极少见的高度恶性肿瘤。自1991年由Gerald报道第1例以来迄今为止全球报道仅数百例^[1],国内也仅见个例报道。本文将报道我院首例DSRCT,以期与临床医师分享对此病的认识。     

促纤维增生性小圆细胞肿瘤1例报道及文献复习

目的：探讨促纤维增生性小圆细胞肿瘤的诊断及鉴别诊断。方法：通过HE、免疫组化染色观察1例腹腔内促纤维增生性小圆细胞肿瘤,并复习文献。结果：肿瘤组织由小圆形瘤细胞巢及周围硬化性纤维间质所组成。免疫组化染色显示有上皮性、间叶性及神经性标记物共同表达。结论：促纤维增生性小圆细胞肿瘤是一种特殊的高度恶性肿瘤。     

结缔组织增生性小圆细胞肿瘤2例并文献复习

目的:分析结缔组织增生性小圆细胞肿瘤(DSRCT)的临床病理特征。方法:结合文献对2例相对少见的DSRCT进行临床、病理形态及免疫组化研究。结果:DSRCT由成团巢的小圆细胞和丰富的结缔组织间质组成。免疫组化:小圆形的肿瘤细胞vimentin( );desmin( );EMA( );CK( );NSE( )。结论:DSRCT为高度侵袭性肿瘤,主要累及腹腔或盆腔的腹膜,肿瘤由小圆细胞和丰富的结缔组织间质组成。     

食管小细胞未分化癌3例

食管小细胞未分化癌罕见,文献报道,到1993年为止,国内外文献总共报道仅百余例,其发生率占食管癌的0.05～7.60％,近年来有增多趋势。我院自1988年10月至1995年7月经内镜诊断食管癌212例,均经病理证实,其中     

Primary retroperitoneal perirenal CIC rearrangement sarcoma: A case report

Primary retroperitoneal perirenal CIC rearrangement sarcoma is rare. The current case report presents a 69-year-old male patient with this pathology, including the clinical features, pathomorphology and immunohistochemistry, and CIC gene rupture detected by fluorescence in situ hybridization (FISH). Furthermore, the relevant literature was reviewed. Histologically, the tumor was composed of diffuse nests of small- to medium-sized juvenile round blue cells with hyperchromatic nuclei, prominent nucleoli and occasional mitotic signs. The tumor involved adipose tissue with no obvious hemorrhagic necrotic foci. Immunohistochemistry indicated scattered expression of CD99 in tumor cells. FISH examination suggested that the CIC gene was fragmented and translocated.     

滤泡树突状细胞肉瘤伴嗜血细胞综合征1例并文献复习

滤泡树突状细胞肉瘤(follicular dendritic cell sarcoma,FDCS)是一种极为罕见的恶性肿瘤,有文献报道称树突状细胞性肿瘤和组织细胞性肿瘤共占淋巴结或软组织肿瘤的比例小于1%[1],FDCS主要起源在树突网状细胞,多以侵及颈部与腋窝的淋巴结为主要临床表现。噬血细胞综合征(hemophagocytic syndrome,HPS)又称嗜血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH),也是一种发病率极低的疾病,进展迅速,致死率高,其主要特征为嗜血细胞多器官浸润和炎性细胞因子过量生成导致的血细胞减少[2]。本文报道了1例滤泡树突状肉瘤合并嗜血细胞综合征的诊疗经过,复习国内外相关文献,并介绍FCDS合并HPS相关病因、临床表现、诊断、治疗、研究和进展。     

Follicular dendritic cell sarcoma of the stomach: case report and review of the literature

Follicular dendritic cell sarcoma (fdcs) is a rare entity, often presenting a diagnostic challenge for both the pathologist and the clinician. It accounts for only 0.4% of soft-tissue sarcomas, and its underlying causes are largely unknown. Most of these tumours occur in lymph nodes, and extranodal involvement is uncommon. In the gastrointestinal tract, fdcs is extremely rare. Here, we report a case of primary fdcs originating in the stomach. Upon review of the literature, we identified only 2 additional cases of fdcs presenting as a primary stomach tumour. Given the rarity of this tumour in gastrointestinal sites and the lack of consensus on treatment, evaluation of this entity must continue.     

Surgical treatment for small round cell sarcoma of the bone

Background. The role of surgical intervention for the treatment of small round cell sarcoma of bone, has become important. However, there have been few reports concerning the surgical safety margin for this disease. An appropriate surgical margin should be discussed in relation to the effects of preoperative treatment, local recurrence rate, and prognosis. Methods. Twenty-eight patients with small round cell sarcoma of the bone were studied. Nineteen patients were stage M0 (of whom 18 were operated) and 9 were stage M1 (of whom 4 were operated). Preoperative treatment was evaluated by histological examination of the surgical specimen. The surgical margin was evaluated as curative, wide, marginal, or intralesional, by methods accepted by the Japanese Orthopedic Association. Results. (1) Of the 18 operated patients in stage M0, 2 showed local recurrence because of an inappropriate surgical margin or poor response to preoperative treatment. (2) Patients who had a wide margin procedure and who responded to preoperative treatment did not show local recurrence. (3) Patients who had a curative procedure did not show local recurrence regardless of the effect of preoperative treatment. (4) Patients with surgical speciment histologically evaluated as showing over 90% necrosis showed no local recurrences and had favorable prognoses with wide or curative procedures. Conclusion. The effects of preoperative treatment and surgical margins were important prognostic factors for local control in patients with small round cell sarcoma of the bone. Received: July 2, 1998 / Accepted: August 17, 1999     

A case report of primary small cell carcinoma of the breast and review of the literature

Primary small cell carcinoma (SCC) of the breast, an exceedingly rare and aggressive tumor, is often characterized by rapid progression and poor prognosis. We report a case of primary SCC of the breast that was diagnosed through pathologic and immunohistochemical examinations. Computed tomography (CT) scans failed to reveal a non-mammary primary site. Due to the scant number of relevant case summaries, this type of tumor is proved to be a diagnostic and therapeutic challenge. Therefore, we also reviewed relevant literature to share expertise in diagnosis, clinicopathologic characteristics, treatment, and prognosis of this type of tumor. Future studies with more cases are required to define more appropriate treatment indications for this disease.     

肠系膜淋巴结滤泡树突细胞肉瘤1例报告并文献复习

目的探讨滤泡树突细胞肉瘤的临床病理特征、诊断和鉴别诊断。方法对1例肠系膜淋巴结滤泡树突细胞肉瘤进行光镜和免疫组化观察,结合文献进行讨论。结果镜下肿瘤由旋涡状、束状排列的胖梭形、卵圆形或多边形瘤细胞和大量混杂的淋巴细胞组成。免疫表型瘤细胞表达CD35和CD23,弱阳性表达CD68、S-100和EMA;淋巴细胞表达CD45RO和CD3。结论淋巴结滤泡树突状细胞肉瘤是一种罕见的免疫辅助细胞中度恶性肿瘤,其诊断依靠组织病理学和免疫组化,治疗以手术切除为主,必要时辅以化疗和(或)放疗。     

扁桃体上皮样滤泡树突细胞肉瘤临床病理学分析

目的:探讨扁桃体上皮样滤泡树突细胞肉瘤(follicular dendritic cell sarcoma,FDC sarcoma)的临床病理学特点、免疫表型、超微结构以及鉴别诊断.方法:对1例发生于扁桃体的上皮样FDC肉瘤进行组织学观察、免疫组化标记及电镜观察.结果:患者女性,55岁.咽部不适感2年,近期症状加重而就诊.术中见左扁桃体肿大伴有糜烂.临床考虑为恶性淋巴瘤.镜下见瘤细胞形态较一致,由大上皮样细胞组成,未见梭形瘤细胞.瘤细胞境界不清,丰富的嗜酸性胞质,空泡状核,核仁明显.瘤细胞间散在小淋巴细胞浸润.免疫组化标记显示瘤细胞弥漫性表达vimentin、CD21、CD23、CXCL13和D2 - 40.电镜观察于瘤细胞胞浆内可见粗面内质网和线粒体,少量由桥粒连接的细胞突,明显的椭圆形核及核内的大核仁.肿块切除后随访4个月无复发及转移.结论:扁桃体上皮样亚型的滤泡树突细胞肉瘤非常罕见.诊断时应与多种具有上皮样形态的肿瘤鉴别.     

儿童指突状树突细胞肉瘤合并骨髓增生异常综合征一例报告并文献复习

指突状树突细胞肉瘤(interdigitating dendritic cell sarcoma,IDCS)是一种罕见的树突状细胞肿瘤,目前全球仅百余例报道,常以无痛性淋巴结肿大起病,侵袭性较强、预后较差[1-2].骨髓增生异常综合征(myel-odysplastic syndromes,MDS)为起源于造血干、祖细胞...