鼻腔鼻窦小圆细胞未分化恶性肿瘤一例并文献复习

目的 提高对鼻腔鼻窦小圆细胞未分化恶性肿瘤的认识。 方法 报道吉林大学第二医院收治的1例鼻腔鼻窦小圆细胞未分化恶性肿瘤患者的诊疗情况,并复习相关文献。 结果 该患者明确诊断为鼻腔鼻窦小圆细胞未分化恶性肿瘤,行局部根治性放疗后,肿瘤得到有效控制,放疗后2个月发生脑转移、胰腺转移,未行进一步治疗,1周后患者死亡。 结论 鼻腔鼻窦小圆细胞未分化恶性肿瘤在临床上极为少见,肿瘤恶性程度高,预后极差;免疫组织化学技术对其病理类型的鉴别具有重要意义;目前早诊断、早治疗仍为改善患者预后的关键。     

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...     

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。     

Establishment and Characterization of a Small Round Cell Sarcoma Cell Line, SCCH-196, with t(11;22)(q24;q12)

A cell line designated SCCH-196 was established from an extraskeletal small round cell sarcoma developed in a 16-year-old Japanese girl. The cells grew as a monolayer, and have been continuously propagated by serial subcultures during the past 26 months. Cells from the primary tumor and those from the SCCH-196 cell line at passage 10 both showed the same karyotype, 51, XX, + 8, + 20, + 21, t(11;22)(q24;q12), + i(1q), + i(1q). Histologically the primary tumor was difficult to classify as either Ewing's sarcoma (ES) or peripheral neuroepithelioma (NE). Neuron-specific enolase-positive cells in the primary tumor and the occurrence in the upper extremity were in favor of NE, while positive reaction of SCCH-196 cells to an ES-speciflc monoclonal antibody 5C11 suggested a diagnosis of ES. The SCCH-196 cell line may be useful for basic studies on differentiation of neuroectodermal tumors, and for future cloning of still unidentified genes which may be located at the breakpoints of the 11;22 translocation.     

What is an adequate margin for infiltrative soft-tissue sarcomas?

ObjectivesWhat constitutes an adequate margin of resection for infiltrative subtypes of soft-tissue sarcomas remains unclear. We aimed to determine the prognostic significance of the margin in millimetres for myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS).Methods305 patients diagnosed with either a high-grade, localised MFS (n = 98) or UPS (n = 207) were included. The relationship of closest margin in millimetres to viable tumour and oncological outcomes was analysed.ResultsThe overall local recurrence (LR) rate for all patients were 12%: 19% with positive margin and 10% with negative margin (p = 0.051). The LR rate was similar in patients with negative but <10 mm margin; 13%, 6%, 15%, 17% with 0.1–0.9 mm, 1.0–1.9 mm, 2.0–4.9 mm, and 5.0–9.9 mm margin, respectively. However, the LR rate decreased to 3% if the margin was ≥10 mm. By the R- or R+1-classification, the 10-year cumulative probability of LR was 9%, 15%, 48% for R0, R1, R2 resections, respectively, which was not sensitive enough to stratify the LR risk in patients with negative margins. However, the cumulative probability of LR was significantly stratified by metric distance; the 10-year cumulative LR probability was 3%, 14%, 25% with ≥10.0 mm, 0.1–9.9 mm, and 0 mm, respectively (p = 0.026). A trend towards improved local control by adjuvant radiotherapy was seen in patients with 0–9.9 mm margin (p = 0.078).ConclusionThe resection margin, when measured as a metric distance, correlates with a reduction in LR, and appears to be more significant on local control than radiotherapy. To minimise the risk of LR, a margin distance of at least 10 mm is advocated for MFS and UPS.