反复多次清除术治疗后腹膜促结缔组织增生性小圆细胞瘤一例报告

1995年9月～2005年4月我院收治随访的1例腹膜后促结缔组织增生性小圆细胞瘤(desmoplastic small round cell tumor,DSRCT),术后多次复发,行单纯性反复探查清除术治疗,生存10年以上,回顾报道如下。     

盆腔促结缔组织增生性小圆细胞瘤2例报告并文献复习

促结缔组织增生性小圆细胞瘤(Desmoplastic small round cell tumor,DSRCT) 是一种来源不明的高度恶性小细胞肿瘤.1989年国外首次报道本病[1],临床上罕见,极易出现误诊.我科于2008年3月和5月收治2例,结合相关文献,对其临床及病理特征报告如下.     

腹腔内促结缔组织增生性小圆细胞瘤2例

促结缔组织增生性小圆细胞瘤（desmoplasticsmall round cell tumor,DSRCT）是一种罕见的、恶性程度极高的小细胞肿瘤,95%以上病例发生在腹、盆腔内,称之为腹腔内促结缔组织增生性小圆细胞瘤（intra-abdominal desmoplastic small round cell tu-mor,IDSRCT）,由于罕见、     

睾丸旁促结缔组织增生性小圆细胞肿瘤1例报告及文献复习

目的:探讨睾丸旁促结缔组织增生性小圆细胞肿瘤的临床、病理特征、诊断及治疗。方法:回顾分析1例睾丸旁促结缔组织增生性小圆细胞肿瘤的诊治及随访资料,并复习相关文献。患者,男,27岁,因阴囊内无痛性肿块4个月就诊。体检于左侧阴囊内可扪及囊性肿块,同侧睾丸不能扪及。行左侧睾丸根治性切除术,术后辅以化疗。结果:术中见左侧睾丸旁多发结节状隆起肿块,质偏硬;术后病理检查肿瘤细胞呈巢状或梁索状结构并埋没在增生的纤维结缔组织中;免疫组化显示瘤细胞具有上皮源性、间质性和神经源性等多向分化的特点。术后已随访3年无瘤生存,预后良好。结论:促结缔组织增生性小圆细胞肿瘤有特异的临床病理特征,好发于年轻男性,手术切除肿瘤联合化疗是治疗的主要方法,睾丸旁促结缔组织增生性小圆细胞肿瘤比腹腔型预后相对好。     

肝脏促结缔组织增生性小圆细胞瘤一例

患者男,44岁,以"体检超声发现肝脏占位性病变半个月余"于2006年11月11日入院.体格检查未见明显异常.实验室检查:甲胎蛋白(AFP):105.78 ng/ml,癌胚抗原(CEA):3.7μg/L,CA19-9:12.2 U/ml;HBsAg、HBeAb、HBcAb阳性,HCVAb阴性,血生化及血尿粪常规均无异常.腹部CT:肝Ⅳ、Ⅴ、Ⅷ段交界有-6 cm×5.5 cm大小富血供占位,肝癌可能性大.全麻下行肝肿瘤切除术(不规则性切除),术中未见腹腔有转移性病灶,完整切除肿瘤.     

腹膜后神经鞘瘤1例报告并文献复习

目的报告1例腹膜后神经鞘瘤,并复习相关文献,分析其临床诊治现状。方法通过对1例腹膜后神经鞘瘤的诊断、治疗方法及术后病理、随访的回顾,结合国内外有关文献报道,综合分析腹膜后神经鞘瘤的诊断及治疗状况。结果本例行手术治疗,术后病理证实为腹膜后神经鞘瘤,随访半年无复发。结论腹膜后神经鞘瘤诊断困难,影像学检查无特异性,确诊依靠病理检查,理想的治疗方法是通过外科手术完整切除肿瘤,其预后良好,早期复发率低。     

腹膜后促纤维增生性小圆细胞瘤七例报告

促纤维增生性小圆细胞瘤(DSRCT)是一种近年来才被认识的高度恶性肿瘤,临床罕见,可累及多个器官、组织.我们收治7例,分别为天津医科大学第二医院泌尿外科2例,北京军区总医院肿瘤科2例,江苏宜兴市人民医院妇产科1例,滨州医学院附属医院泌尿外科2例,现报告如下.     

腹膜后血管瘤1例报告并文献复习

目的 提高对腹膜后血管瘤的认识及诊疗水平.方法回顾性分析1例腹膜后肾上腺旁血管瘤患者的临床资料,结合文献复习并讨论其诊断和治疗特点.结果患者经MRI检查发现腹膜后肾上腺旁肿物并行手术治疗,术后患者恢复良好,病理结果证实为血管瘤,术后随访25个月未见病灶复发或扩散.结论 腹膜后肾上腺旁血管瘤在临床上罕见,是一种良性肿瘤,手术为其主要治疗方法,术前明确诊断有一定的难度,易与肾上腺肿瘤、副神经节瘤混淆,术中需注意血压波动,手术切除效果好.     

原发性腹膜后肿瘤的外科治疗(附85例报告)

目的探讨和总结原发性腹膜后肿瘤的手术治疗经验。方法回顾性分析1993年1月至2005年9月经手术治疗的85例原发性腹膜后肿瘤,分析总结其病理学类型、影像学检查、外科手术治疗等临床资料。结果良性肿瘤33例,恶性肿瘤51例,交界性肿瘤1例,均经手术治疗且病理证实。良性肿瘤完整切除31例(93.94%);恶性肿瘤完整切除39例(76.47%);交界性肿瘤行完整切除。手术中联合脏器切除共20例,其中良性2例,恶性18例。良性肿瘤术后复发再手术4例,均完整切除;恶性肿瘤术后复发再手术19例,完整切除14例,部分切除3例,探查活检2例。结论影像学检查对术前诊断和手术切除范围有重要意义。手术完整切除肿瘤是治疗的关键因素。再次手术仍是治疗复发性腹膜后肿瘤的有效手段。     

Intraabdominal desmoplastic small round cell tumor: Report of a case and literature review

Introduction

Desmoplastic small round cell tumor is a rare malignancy with poor prognosis that predominantly affects young males. Its etiopathogenesis is still unknown and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis.

Case presentation

We report a new case of a 29-year-old male who proceeded to our Emergency Department complaining about non-specific abdominal pain. Physical examination revealed no abnormalities except for a palpable mass in the lower abdomen and a diffuse abdominal pain. Computed Tomography scan showed enlarged paraortic and mesenteric lymphadenopathy, thickness of the small bowel wall and dispersed masses intraperitoneally. He underwent an exploratory laparotomy and the resultant biopsy revealed desmoplastic small round cell tumor.

Discussion

Diagnosis of desmoplastic small round cell tumor can easily be missed because it presents with few early warning symptoms and signs, while the routine blood tests are within normal limits.

Conclusion

A high degree of suspicion, a thorough physical examination, a full imaging check and an aggressive therapeutic approach are required in order to identify this disease and fight for a better quality of life for these patients. In addition we make a review of the literature in an effort to clarify the epidemiological, clinical and pathological aspects of this entity.     

Paratesticular desmoplastic small round cell tumor: case report

BACKGROUND: The desmoplastic small round cell tumor has recently been separated from other small round cell tumors because of its characteristic pathological and clinical features. They are usually intra-abdominal tumors affecting young people and have classically been associated with a bad prognosis. However, in recent years there have reports on desmoplastic small round cell tumors affecting other body regions, including the paratesticular area. CASE PRESENTATION: We report the case of a 23-year-old male, that consulted on a progressive enlargement of the right hemiscrotum in the last year and a half. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 2 to 3 cm mass distal to the tail of the epididymis, which was excised with a preoperative diagnosis of adenomatoid tumor. However, histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The extension study included a computed tomography scan and a plain chest radiograph, that showed no metastasis. The patient received chemoradiation therapy with methotrexate, dacarbacin, cyclophosphamide, actinomycin D and vincristin, but had to be changed to a vincristin, actinomycin D, cyclophosphamide and adriamicin scheme on severe toxicity. He completed five cycles of the chemotherapy with moderate toxicity. Today, 6 years after diagnosis the patient remains well and free of disease. CONCLUSIONS: Recent reviews on desmoplastic small round cell tumor affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of paratesticular tumors, mainly in children and adolescents.     

支气管肺癌的肿瘤血管与染色、病理学和预后相关性

目的探讨肺癌肿瘤血管与染色同病理学的相关性及其对介入治疗预后的影响。方法所有患者按年龄、性别、病灶大小和位置、病理学、肿瘤供血动脉、肿瘤血管、肿瘤染色、手术方式、治疗次数、首次发病时间、首次介入治疗时间、生存时间分组统计,并输入SPSS12.0软件进行系统分析。结果40例连续血管造影随访,治疗前肿瘤染色明显者18例,肿瘤染色一般者9例,肿瘤染色欠佳者13例;末次治疗后血管造影,肿瘤染色明显者9例,肿瘤染色一般者17例,肿瘤染色欠佳者14例;治疗前后肿瘤染色改变统计学处理(P<0.01),其中支气管动脉供血35例(35/40,87.5%),同时肺动脉参与供血者4例(10%),鳞癌和腺癌支气管动脉供血32例(80%)。病理学、介入方法和生存时间之间的相关分析显示有明显统计学意义。结论肺癌的肿瘤血管与肿瘤染色同组织病理学分型不相关,而与病理学分级呈负相关,即分化程度越低,肿瘤血管和染色越丰富。鳞癌和腺癌以支气管动脉供血为主。血管内介入治疗能明显减少肿瘤血管和肿瘤染色,提高支气管动脉内治疗效果。     

Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors

Desmoplastic small round cell tumors (DSRCTs) present a reciprocal chromosomal translocation, t(11;22)(p13;q12), that results in fusion of Ewing's sarcoma and Wilms' tumor (WT1) genes. The authors evaluated 15 DSRCTs and 71 other tumors often considered in the differential diagnosis for immunoreactivity using a polyclonal antibody directed against the WT1 part of the chimeric protein resulting from this translocation. WT1 immunostaining was performed on paraffin material using the WT(C-19) antibody after heat-antigen retrieval. All the DSRCTs (15 of 15, 100%) demonstrated strong WT1 nuclear immunoreactivity. Ten of 14 nephroblastomas (71%) disclosed WT1-positive nuclei in accordance with the staining reported by others, and rare and focal nuclear positivity was detected in two of 17 rhabdomyosarcomas. WT1 immunoreactivity was not observed in Ewing's sarcoma/primitive neuroectodermal tumors (zero of 21, 0%), neuroblastomas (zero of 17, 0%), or rhabdoid tumors of the kidney (zero of two, 0%). In nephroblastoma, differential diagnosis with DSRCT was not difficult: Clinical and morphologic data are not similar for these two entities. The current study validates WT1 immunoreactivity as a useful marker to separate DSRCT from other small round cell tumors.     

腹腔内纤维组织增生性小圆细胞肿瘤(1例报告及文献复习)

腹腔内纤维组织增生性小圆细胞肿瘤(intra-abdominal desmoplastic small round cell tumor．DSRCT)是由Gerald和Rosai于1989年首先报道的一种少见的恶性肿瘤，其特点为沿浆膜侵袭和播散性生长．组织学上由巢状排列的小圆细胞和明显增生的纤维组织间质构成．免疫组织化学染色表现为上皮、间叶和神经性标记物的复合表达。虽然本病较少见．但近年来对其研究却相当深入和广泛，从临床与治疗、病理学改变，     

不同病理学类型的腹膜后脂肪肉瘤患者的预后分析及临床评价

背景与目的 腹膜后脂肪肉瘤是一种罕见的软组织肉瘤,其发病率低,预后差,治疗手段有限,不同病理学类型之间患者的预后差异较大,缺少针对该类患者的大样本临床研究证据,临床个体化治疗方案制定困难。本研究旨在探讨不同病理学类型腹膜后脂肪肉瘤患者预后的影响因素,并分别探讨手术、放射、化疗在他们中的应用价值。方法 从SEER数据库提取1975—2016年期间经病理学确诊且有完整随访记录的腹膜后脂肪肉瘤患者资料,根据纳入和排除标准严格筛选病例,依据ICD-O-3分为高分化脂肪肉瘤、去分化脂肪肉瘤、黏液样/圆细胞脂肪肉瘤、多形性脂肪肉瘤、混合脂肪肉瘤5类,比较不同病理学类型患者间肿瘤特异生存（CSS）和总生存（OS）的差异,并分析患者CSS与OS的影响因素。结果 共纳入2 296例腹膜后脂肪肉瘤患者,其中,高分化脂肪肉瘤917例,去分化脂肪肉瘤847例,黏液样/圆细胞脂肪肉瘤302例,多形性脂肪肉瘤135例,混合脂肪肉瘤95例。中位随访42个月（IQR：13~90个月）。估算的5年OS分别为高分化脂肪肉瘤74.99%（95% CI=71.65%~78.01%）、去分化脂肪肉瘤42.83%（95% CI=38.87%~46.73%）、黏液样/圆细胞脂肪肉瘤50.13%（95% CI=47.02%~58.86%）、多形性脂肪肉瘤34.69%（95% CI=26.39%~43.11%）、混合脂肪肉瘤57.67%（95% CI=46.43%~67.37%）,Log-rank检验显示,组间差异有统计学意义（χ²=211.54,P<0.000 1）。估算的5年CSS分别为高分化脂肪肉瘤87.98%（95% CI=85.23%~90.25%）、去分化脂肪肉瘤57.78%（95% CI=50.46%~61.85%）、黏液样/圆细胞脂肪肉瘤64.99%（95% CI=58.64%~70.62%）、多形性脂肪肉瘤46.02%（95% CI=36.22%~55.25%）、混合脂肪肉瘤67.33%（95% CI=55.71%~76.53%）,Log-rank检验显示,组间差异有统计学意义（χ²=227.92,P<0.000 1）。不同病理学类型Cox多因素分析显示,年龄是各病理学类型患者预后的独立影响因素（均P<0.05）。在高分化组脂肪肉瘤患者中,根治性和非根治性手术均能提高OS（根治性：HR=0.42,95% CI=0.19~0.92,P=0.031;非根治性：HR=0.40,95% CI=0.18~0.88,P=0.023）和CSS（根治性：HR=0.32,95% CI=0.16~0.63,P=0.001;非根治性：HR=0.23,95% CI=0.12~0.44,P=0.001）;化疗会降低患者的OS（HR=2.29,95% CI=1.54~3.40,P<0.001）和CSS（HR=3.55,95% CI=2.16~5.83,P<0.001）。在去分化脂肪肉瘤患者中,根治性和非根治性手术均能提高OS（根治性：HR=0.25,95% CI=0.18~0.35,P<0.001;非根治性：HR=0.34,95% CI=0.24~0.47,P<0.001）和CSS（根治性：HR=0.22,95% CI=0.15~0.33,P<0.001;非根治性：HR=0.31,95% CI=0.21~0.45,P<0.001）;放疗能提高CSS（HR=0.75,95% CI=0.57~0.99,P=0.043）;化疗会降低OS（HR=1.33,95% CI=1.05~1.69,P=0.018）和CSS（HR=1.64,95% CI=1.24~2.18,P=0.001）。放疗能提高黏液样/圆细胞脂肪肉瘤患者的OS（HR=0.66,95% CI=0.47~0.92,P=0.015）和CSS（HR=0.54,95% CI=0.34~0.84,P=0.007）。结论 年龄是影响不同病理学类型腹膜后脂肪肉瘤患者预后的独立危险因素。腹膜后脂肪肉瘤患者的治疗,应以病理学分类为导向制定个体化治疗方案。手术切除是高分化和去分化脂肪肉瘤最佳适应证。对于手术方式的选择,应综合评价患者全身情况。在高分化脂肪肉瘤患者中R₀切除不是必须的,R₁切除或者局部治疗（包括微波、射频等）也能达到提高患者OS和CSS的作用。而对于去分化脂肪肉瘤,则应争取达到R₀切除。放疗是黏液样/圆形细胞脂肪肉瘤的最佳适应证,同时也是去分化脂肪肉瘤患者治疗手段的重要补充。在高分化和去分化病理学类型中,化疗会增加患者的死亡风险。     

Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare disease of children, adolescents, and young adults that begins and spreads on the peritoneal surfaces. Desmoplastic small round cell tumor usually presents with diffuse abdominal metastatic disease similar in gross appearance to carcinomatosis. To date, very aggressive treatment programs have yielded dismal outcomes. Here we present 2 cases of DSRCT that were treated with aggressive surgical excision followed by intraoperative continuous hyperthermic peritoneal perfusion using cisplatin. These are the first pediatric case reports of DSRCT being treated with continuous hyperthermic peritoneal perfusion, a procedure usually used in treatment of adult carcinomatosis.     

Retrovesical desmoplastic small round cell tumor in a patient with urinary frequency

A 21-year-old man with urological symptoms was found to have a large abdominal tumor in the retrovesical space between the bladder and the rectosigmoid colon. Radiologic findings revealed little evidence confirming the diagnosis. A transrectal biopsy failed to disclose the histopathologic origin of the tumor. An exploratory laparotomy with a complete surgical resection was impossible, so a wedge biopsy was performed. Combined histologic and immunohistochemical findings revealed the features of desmoplastic small round cell tumor (DSRCT). Despite subsequent multi-agent chemotherapy, the patient died as a result of the growing tumor and liver metastasis. There have been only two prior reports of this neoplasm in the urological literature.