肝脏炎性肌纤维母细胞瘤

目的探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料,并复习了国内外的文献,分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下:①常见于儿童和青少年;②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等;③物理检查可见肝脏包块;④病理特征:该瘤由分化的肌纤维母细胞性梭形细胞组成,常伴大量浆细胞和(或)淋巴细胞浸润;⑤外科切除或肝移植后,预后佳,无复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现,经手术切除后可治愈。     

肝脏炎性肌纤维母细胞瘤

目的 探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料，并复习了国内外的文献，分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下：①常见于儿童和青少年；②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等；③物理检查可见肝脏包块；①病理特征：该瘤由分化的肌纤维母细胞性俊形细胞组成．常伴大量浆细胞和（或）淋巴细胞浸润；⑤外科切除或肝移植后，预后佳，尤复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现，经手术切除后可治愈。     

Malignant hemangiopericytoma of the liver: Report of a case

A 30 year old female was admitted with right upper abdominal pain and fever. Ultrasonography and computed tomography revealed a large cystic mass in the right lobe of the liver, and aspiration bacteriology was negative. A right hepatic lobectomy was performed for a suspected cystadenocarcinoma, however, the tumor was histologically diagnosed as a hemangiopericytoma with prominent venous invasion. The patient died within a short time of multiple pulmonary metastases. Primary hepatic hemangiopericytoma is extremely rare, and according to our research, this is only the 4th case reported in the literature.     

Pancoast (Superior Sulcus) Tumors

Primary carcinomas arising in the apex of the lung (Pancoast tumors) have attracted attention because of the characteristic syndrome that is produced by local extension into the chest wall and the brachial plexus. This article reviews the history of the treatment of this disease, the natural history of untreated patients, and the diagnosis of Pancoast tumors. The published data on results, prognostic factors, and technical aspects of treatment with combined irradiation and operation are examined, as well as those pertaining to treatment with irradiation alone.     

Papillary glioneuronal tumor: case report and review of literature

Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles.In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.In our report we discuss the clinical, neuroradiological and surgical features. The final result is compared with literature data.     

Glypican-3基因和肿瘤的研究进展

GPC3是一种膜性（membranous）硫酸乙酰肝素糖蛋白（heparansulfate proteoglycan,HSPG）,通过磷脂酰肌醇锚定在细胞膜上。GPC3在细胞的生长发育中起着重要的调控作用,在不同的恶性肿瘤中表达差异很大,可能是肿瘤发生的重要原因,新近研究最多的是GPC3是否可以作为肝癌、黑色素瘤、睾丸生殖细胞诊断的一个标记物,GPC3与肿瘤的免疫治疗及GPC3与Wnt通路的关系。     

Suprasellar yolk-sac tumor in two sisters

The familial occurrence of intracranial tumors is rare except in patients with phacomatoses. The occurrence of suprasellar yolk-sac tumors in two sisters without phacomatoses is reported. The pertinent literature is reviewed, and the possible role of heredity in the pathogenesis of these tumors is discussed briefly.     

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目的　探讨胃肠道间质瘤的临床表现、诊断及治疗。方法　回顾性分析上海建工医院及复旦大学华山医院外科 2000年 9月至 2003年 12月间收治 35例胃肠道间质瘤病人的临床及病理资料。结果　35例均行手术治疗。肿瘤根治 4例,近端或远端胃大部切除、病变肠段切除、肿块切除或扩大切除 26例;因肿瘤广泛粘连,无法完整切除 5例。病理:CD117阳性 29例(82 .9% ),CD34阳性 31例 (88 .6% )。结论　胃肠道间质瘤在中老年人中好发,以便血、腹痛和腹部不适、腹部肿块表现为主。肿瘤的大小是胃肠道间质瘤良、恶性的重要临床指标。病理形态学检查是确诊间质瘤的唯一方法。完整的局部手术切除是最有效的治疗手段。对无法切除或有肿瘤残余及术后复发的病人可应用甲磺酸伊马替尼(格列卫)进行治疗。     

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目的探讨原发性腹膜后内胚窦瘤的治疗和预后方法回顾性分析1991～2002年收治的11例碍发性腹膜后内胚窦瘤．结果该组病例占同期收治恶性原发性腹膜后肿瘤的8.8％(1l／125)和内胚窦瘤的20.3％(1l／54)。术前B超、CT或MR1检查发现腹膜后肿瘤。术前血清甲胎蛋白升高5例全组手术切除全部或大部分肿瘤。术后用PVH(顺铂、长春新碱、博菜霉素)或BEP方案(博菜霉素、足叶乙甙、顺铂)化疗随访8个月至8年，生存10例，死亡1例。结论以顺铂为基础的联合化疗改善了内胚窦瘤的疗效，测定血清AFP确助内胚窦瘤的诊治，应选择恰当的手术方式并辅以联合化疗来救治病人     

胃肠道间质瘤46例临床诊治分析

目的探讨胃肠道间质瘤(G IST)的临床特点。方法对1998~2004年收治的46例G IST病人的临床表现、手术处理及病理结果进行回顾性分析。结果46例均行手术治疗。病理诊断良性13例(28.3%),潜在恶性10例(21.7%),恶性23例(50%)。随访时间6个月至6年,7例出现远处转移或局部复发,10例死亡(其中9例为恶性G IST)。结论G IST缺乏特征性临床表现,术前确诊率较低,其确诊依赖病理结果。目前G IST的治疗仍以手术切除为主,对复发或远处转移者积极再次手术可延长生存期。     

Gastrointestinal stromal tumor of the lesser omentum: report of a case

We describe herein an extremely unusual case of a gastrointestinal stromal tumor (GIST) of the lesser omentum. A 45-year-old man was admitted to our hospital with an intra-abdominal mass that was subsequently misdiagnosed as a submucosal tumor of the stomach. The tumor arose from the lesser omentum and was removed without difficulty. Histologically, the tumor was composed of spindle–shaped cells with an interlacing bundle pattern, and immunohistochemical examination showed that it was positive for myeloid stem cell antigen (CD34), but negative for HHF35 and S-100 protein. These findings were consistent with a GIST lacking myogenic features and neural attributes. The patient had an uneventful postoperative course, and was free of recurrence when last seen 11 months after his operation. Received: April 3, 2000 / Accepted: March 6, 2001     

Metachronous testicular tumor developing eight years after retroperitoneal extragonadal germ cell tumor

Abstract:   A 30 year-old man was admitted to our hospital with a chief complaint of left flank pain. Computed tomography revealed a retroperitoneal tumor. Levels of lactic dehydrogenase, human β-chorionic gonadotropin, α-fetoprotein, DUPAN-2 and carbohydrate antigen 19–9 were elevated. No abnormal findings were present according to palpation and ultrasonography of the testes. The patient was diagnosed as having a retroperitoneal extragonadal germ cell tumor (EGCT) considering the elevated markers. Resection of the tumor, two cycles of neoadjuvant and one cycle of adjuvant chemotherapy (cisplatin and etoposide) were performed. The surgical specimen showed total necrotic tissue. Eight years later, the patient noticed an enlargement of his left testicle. The tumor felt elastic and firm on his left testis. Neither distant metastasis nor lymph node metastasis was present according to computed tomography. Left high orchiectomy was performed and histology revealed seminoma. Twenty-three cases have been reported previously about metachronous testicular tumor developing after retroperitoneal EGCT. We report the 24th case.     

Myofibroblastic tumor causing severe neonatal distress. Successful surgical resection after embolization

This report describes a case of a term male 3.1 kg, normal delivery, 38 weeks of gestation with a record of hydramnios by prenatal sonography. He had fetal acute suffering and respiratory distress. The first radiographic study showed a mass filling the whole left thorax cage causing erosion of the inferior edge of the third rib. The mediastinum was displaced to the right. Computed tomography scan confirmed a homogeneous tumor that filled the left thorax and displaced the mediastinum to the right without invasion. Surgical biopsy informed of a highly vascularized mesenchymal tumor. The tumor was embolized with Ivalon microparticles obtaining a nearly avascular mass. Complete surgical excision was made, including the whole mass and costal segments. Microscopically, it was an inflammatory myofibroblastic tumor. It was composed mainly of spindle-shaped cells without malignant features. On immunohistochemistry, the tumor showed positive staining for vimentin, whereas antidesmin antibodies and S-100 protein were negative. The aim of this article is to present an extremely uncommon case of neonatal distress caused by an intrathoracic, extrapulmonary myofibroblastic tumor. Complete surgical resection was possible after embolization.     

Dumbbell neurogenic tumor of the mediastinum: A report of three cases undergoing single-staged complete romoval without thoracotomy

Of a total thirteen patients who underwent surgery for a neurogenic tumor in the posterior mediastinum 4 (30.8 per cent) presented with dumbbell type development of the tumor. Along with a comparatively greater incidence in the number of cases of dumbbell neurogenic tumors in the posterior mediastinum, resection has also recently become more popular, necessitating the establishment of a standard operative approach for this type of tumor. We successfully removed dumbbell neurogenic tumor from the posterior mediastinum in our 3 most recent casesvia a dorsal approach by virtue of a laminectomy and resection of a small portion of the neighbouring rib root without opening the parietal pleura at all. These three aptients were a 14 year old female, a 54 year old male and a 68 year old female, respectively, and the largest diameter in cm and level of origin of the tumors were 5.5 at Th 1 in case 1, 3.0 at Th 2 in case 2 and 3.7 at Th 11 in case 3. The operative approach described herein was easy to perform, felt secure and was less invasive and better tolerated by the patients than the thoractomy approach. Avoiding a thoractomy in such cases has many advantages to enumerate, but does not seem to have been clearly aimed at by others to date. We therefore propose our technique as a standard approach for dumbbell neurogenic tumors in the posterior mediastinum.     

Fetal and neonatal rhabdoid tumor

Purpose

Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis.

Methods

The author conducted a 40-year systematic review of the literature. Clinical presentation, pathology, management, and outcome of 72 fetuses and neonates with RTs are discussed.

Results

Seventy-two fetuses and neonates presented with RTs detected prenatally (n = 12) and during the neonatal period (n = 60). The review consisted of 3 main groups: extrarenal noncentral nervous system (CNS) RT, renal RT, and CNS RT. There were some group differences in survival: extrarenal non-CNS RT (3/33 or 9.1%), renal RT (2/27 or 7.4%), and CNS RT (2/12 or 16.7%). Metastatic RT was present at diagnosis in more than half the patients (41/72 or 57%) who had a survival of 2.3%. The overall survival was 9.7%. For statistical results, there was no significant difference in survival among the 3 groups by type of tumor (P = .692). χ² analysis for survival with and without metastases was not valid due to small sample size.

Conclusions

The review shows that extrarenal RT was more common than either renal RT or CNS RT groups that is different than that observed in older individuals. Concomitant brain tumors were found in almost a third of fetuses and neonates. The CNS involvement occurred more often in patients with renal RT than in those with extrarenal RT. Metastatic disease at diagnosis was noted in more than half of the patients. Higher stage and presence of a CNS tumor were significant determinants in survival.     

十二指肠间质瘤的临床特征及外科治疗

目的探讨十二指肠间质瘤的临床特征及外科治疗效果. 方法回顾性分析我科1990～2004年14例十二指肠间质瘤的临床资料. 结果病理类型:良性2例,潜在恶性4例,恶性8例.50.0%(7/14)发生在降部,其中恶性占57.1%(4/7).临床表现为右上腹肿物(6/14),右上腹隐痛不适(5/14)和乏力、体重下降(3/14),诊断手段为十二指肠低张造影(13/14)、B超(13/14)和CT(9/14).肿瘤距离十二指肠大乳头＜3 cm或位于降部、直径≥5 cm者宜行胰十二指肠切除术,肿瘤距离十二指肠大乳头＞3 cm及较小者宜行十二指肠节段性切除术. 结论十二指肠间质瘤恶性多见,临床表现无特异性,主要依靠十二指肠低张造影、B超、CT、EUS确诊,对无远处转移的肿瘤应积极争取根治性切除.     

阴茎原始神经外胚叶瘤1例并文献复习

目的:探讨原发于泌尿生殖系统的原始神经外胚叶瘤的临床表现、病理特点、治疗方法及预后。方法:分析本院2007年3月收治的1例原发于阴茎的原始神经外胚叶瘤患者的病例资料并复习相关文献。结果:该患者经过病理确诊为阴茎原始神经外胚叶瘤,已采取手术根治,拟进一步化疗。结论:病理及免疫组化有助于对原始神经外胚叶瘤的确诊。对于早期患者手术是首选的治疗方法,晚期患者可以辅助性地行联合化疗,目前原发于阴茎的原始神经外胚叶瘤非常罕见,对预后尚没有评估依据。