脾脏炎性假瘤1例

1病例资料患者女,46岁,因"左肩背部疼痛2 d"于2012年9月15日就诊,未诉腹部明显不适。患者无发热,无反酸、嗳气,无恶心、呕吐,无腹泻、黑便,否认外伤史及手术史。体格检查:腹平,触软,左上腹轻度压痛,无反跳痛,肝脾肋下未及,肝肾脾区无叩痛,肠鸣音正常。血常规:RBC 3.14×1012/L,Hb 87 g/L,PLT 213×109/L,Hbs-Ag(-)。超声检查示:脾脏体积     

肝脏滤泡树突细胞肉瘤

肝脏滤泡树突细胞肉瘤(follicular dendritic cell sarcoma, FDCS)是甚为罕见的肝脏恶性肿瘤.本文报告1例肝脏巨大FDCS,并结合相关文献资料,讨论其临床特点、诊断及治疗方法,为临床医师正确认识该肿瘤提供参考.     

脾脏炎性假瘤七例诊治报告

目的探讨脾脏炎性假瘤的临床诊断和治疗。方法回顾性分析2006年1月至2015年3月7例经手术和病理证实的脾脏炎性假瘤的临床资料以及结合文献探讨其CT表现。结果 7例病人中2例有外伤致脾脏包膜下血肿病史,病程较长,早期无自觉症状,其余病例均由体检发现脾脏占位,无特异性临床表现,均行脾脏切除术。术后定期随访均无并发症。结论脾脏炎性假瘤的治疗主要是通过手术,增强CT对脾脏炎性假瘤的诊断和鉴别诊断具有重要价值。     

阑尾滤泡树突状细胞肉瘤的临床与病理

目的 探讨阑尾滤泡树突状细胞肉瘤（follicular dendritic cell sarcoma,FDCS）的生物学行为特征,诊断要点和临床治疗.方法 对1例阑尾FDCS术后8个月腹腔内复发患者的手术标本进行病理形态学观察、免疫组化检测.结果 两次手术切除的肿瘤的组织病理学形态一致.免疫组化：CD21、CD35、S-100、Vimentin、CD68和FN呈阳性反应;CD117、CD34、SMA、Actin、NSE和CK呈阴性反应.病理学诊断：阑尾FDCS,腹腔内复发.结论 FDCS是一种非常罕见的肿瘤,大多发生于淋巴结,少数可见于淋巴结外.手术切除是治疗FDCS的主要方法.     

少见类型的肝脏良性肿瘤和瘤样病变

目的：总结少见类型的肝脏良性肿瘤和瘤样病变的诊治经验。方法：对1980年1月－2000年6月，收治的并经手术和病理证实的58例原发于肝脏的少见的良性肿瘤和瘤样病变的临床资料进行回顾性分析。结果：58例中炎性假瘤26例，局灶性增生性病变8例，肝细胞腺瘤7例，脂肪类肿瘤7例，囊腺瘤5例，肝结核3例及错构瘤和神经纤维瘤各1例。男性33例，女性25例，年龄20－66岁，平均42．7岁，82．8％的病人有临床症状，17．2％的病人无任何症状，93．1％为单发病灶，6．9％为多发病灶，手术方式：病灶局部除30例，肝段切除14例，肝叶切除9例，肝活检2例，肝动脉结扎2例，囊腔引流加肝动脉结扎1例，未发生严重并发症和手术死亡。结论：近年随着影像检查技术的普及，少见类型的肝脏良性肿瘤和瘤样病变的发生率有所增加，然而影像技术在诊断上缺乏特异性，在鉴别诊断上往往存在困难，故我们应熟悉上述病变的临床情况，影像特征和处理方法。     

肝脏炎性假瘤的临床病理要点

目的 探讨肝脏炎性假瘤的临床病理要点。方法 对我院从1994年6月至2000年4月病理证实为肝脏炎性假瘤的穿刺及手术切除的37例标本的临床病理要点进行分析和讨论。结果 肝脏炎性假瘤在临床上可以无症状，在这37例中无症状的有25例(67．6％)。病理诊断上也有本身特殊的表现。结论 肝脏炎性假瘤极为少见，临床上常与其它的肿瘤相混淆，确诊只能依靠病理检查，因此其临床病理要点对于临床及病理医生来说都具有重要价值。     

肝脏瘤样病变11例临床与病理分析

目的：探讨肝脏瘤样病变的临床与病理特征，指导临床诊治。方法：回顾性分析本院1991年5月至2000年月经手术和病理学证实的11例肝脏瘤样病变的临床，影像，手术及病理学资料。结果：概括本病具有以下特点，患者多为男性（9／11），发病年龄较轻，平均38．5岁，血清AFP一般正常，18％有肝炎感染证据。彩色超声和CT均提示肝脏占位病变，病理形态学呈良性改变，多数无肝硬化背景。结论：肝脏瘤样病变临床主要应用于肝脏恶性肿瘤相鉴别，如能在手术前获明确诊断可密切随访外，均应积极手术切除。     

肝脏血管周上皮样细胞肿瘤临床分析

目的 提高对罕见的肝脏原发性肿瘤——肝脏血管周上皮样细胞肿瘤(PEComa)的诊疗水平.方法 采用文献检索收集1989 ～ 2013年间报道的包括北京大学人民医院1例在内资料齐全的原发性肝脏PEComa患者41例,总结其病例资料,对原发性肝脏PEComa的临床特点、影像学资料、病理特点、治疗及预后进行回顾性分析.结果 肝脏PEComa无特异性临床表现,影像学特征不易识别,主要依靠术后病理确诊.手术是目前公认的主要治疗方法.41例患者中34例为良性,7例为恶性,恶性者预后较差.结论 原发性肝脏PEComa是一种肝脏罕见肿瘤,应提高对其认识和诊疗水平,术后应长期密切随访.     

树突细胞与肾肿瘤治疗研究进展

树突细胞作为目前功能最强的抗原提呈细胞其在抗肿瘤中的作用已成为热点，本文就DC在肾肿瘤中的应用进行综述，包括DC的概况、TIDC及免疫逃逸、体外DC的制备方法、DC在肾肿瘤中的临床应用以及DC引起的免疫耐受、自身免疫反应等，并对各种形式瘤苗的可行性进行探讨。     

Inflammatory myofibroblastic tumor of the liver

Inflammatory myofibroblastic tumor is a rare benign entity formerly known as inflammatory pseudotumor. Involvement of the liver is extremely rare. There are controversies about the optimal treatment of this benign entity. Newer reports suggest an association with autoimmune sclerosing pancreatitis and primary sclerosing cholangitis. We present a case of an 18-year-old patient with biliary obstruction from a perihilar mass of the liver requiring hepatic resection. Division of the hepatic bile duct resulted in drainage of yellow, thick, gelatinous material in the presence of benign margins and absence of cholangitis. Histological examination showed a mass with fibroblastic and myofibroblastic cells set in a loose myxoid matrix containing scattered lymphocytes, consistent with an inflammatory myofibroblastic tumor. One-year recovery was uneventful. This report discusses the presentation, diagnosis, and controversies in management of this disease.     

Inflammatory pseudotumor-like follicular dendritic cell tumor: a distinctive low-grade malignant intra-abdominal neoplasm with consistent Epstein-Barr virus association

Follicular dendritic cell (FDC) tumors are uncommon neoplasms that can involve lymph nodes or extranodal sites. They can exhibit a broad spectrum of histologic appearances and behavior, but the intra-abdominal ones usually pursue an aggressive course. The purpose of this study was to characterize a distinctive variant of FDC tumor morphologically mimicking inflammatory pseudotumor through analysis of the clinicopathologic features of 11 cases. The patients included 10 women and one man (age range, 19-61 years; median age, 40 years) who presented with abdominal discomfort or pain. Six patients had systemic symptoms such as marked weight loss, fever, or malaise. All tumors occurred in intra-abdominal sites: liver (n = 7), spleen (n = 3), and peripancreatic region (n = 1). Of the nine patients with follow-up data, six were alive and well, one developed recurrence at 9 years, and two had repeated recurrences over many years. Grossly, the tumors were usually solitary and fleshy, punctuated by areas of hemorrhage and necrosis. Histologically, in a background of abundant lymphocytes and plasma cells were dispersed spindle or ovoid cells with vesicular nuclei and distinct nucleoli. The degree of nuclear atypia was variable, and some nuclei could be grotesque or resemble Reed-Sternberg cells. Focally, spindle cell fascicles could be formed. The atypical cells were immunoreactive for FDC markers such as CD21/CD35, CD23, and CNA.42. In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was positive in all cases, remarkably highlighting the spindle cells and their atypia. EBV-latent membrane protein-1 was expressed commonly, albeit often focally and weakly. Therefore, inflammatory pseudotumor-like FDC tumor represents a distinctive variant of FDC tumor that differs from conventional FDC tumor in the following aspects: marked female predominance; selective localization in intra-abdominal sites, especially the liver and spleen; frequent presence of systemic symptoms; indolent behavior despite an intra-abdominal location; dispersed distribution of tumor cells and prominent lymphoplasmacytic infiltration; and consistent association with EBV.     

Inflammatory pseudotumor of the spleen: Report of a case

A case of a 45-year-old Japanese man with a splenic inflammatory pseudotumor is described. This benign lesion is rarely reported in the world literature. We preoperatively could not rule out the possibility of a malignant neoplasm, due to the fact that the tumor had grown in size after a 2-year observation. However, after performing a splenectomy, a histological examination of the mass revealed an inflammatory process. Inflammatory pseudotumors often pose diagnostic difficulties because the clinical and radiological findings tend to suggest a malignancy. The clinical and pathological features of such previously reported cases are also reviewed. Received: August 9, 1999 / Accepted: March 24, 2000     

肾炎症性肌纤维母细胞瘤临床病理分析

目的探讨肾炎症性肌纤维母细胞瘤的病理学特点、临床表现和预后。方法总结1例罕见的肾炎症性肌纤维母细胞瘤,结合文献对其临床表现、组织形态、免疫组化特点、治疗及预后等进行分析和探讨。结果患者女性,58岁,无明显诱因出现排酱油色尿,伴尿频、尿急、尿痛。CT提示＂右肾盂输尿管交界处占位性病变＂,行右侧肾切除术。肾肿物大小为9cm×7cm×6cm,圆形,灰白色,质地较硬,无包膜,与周围界限欠清。肿物切面为灰白色,实性,部分区域粘液变性、出血及坏死。镜下见肿瘤细胞呈梭形,肿瘤细胞呈束状或编织状排列,伴较多淋巴细胞和浆细胞浸润。免疫组化显示Vimentin及SMA呈阳性表达。病理诊断：肾炎症性肌纤维母细胞瘤。结论肾炎症性肌纤维母细胞瘤是非常少见的低度恶性肿瘤,确诊需依靠病理诊断,治疗多采用外科手术切除。     

Inflammatory Pseudotumor of the Spleen: Report of a Case

We report the case of an inflammatory pseudotumor of the spleen in an asymptomatic 55-year-old woman, whose lesion was accidentally found and clinically misdiagnosed to be lymphoma. An inflammatory pseudotumor of the spleen was histopathologically diagnosed following a splenectomy. This lesion is a benign, reactive, and inflammatory process and its etiopathogenesis still remains elusive. The preoperative diagnosis is difficult and the optimal management of the asymptomastic patient with the disease is unclear. This entity should be kept in mind in the differential diagnosis of splenic space-occupying lesions.     

睾丸大细胞钙化型支持细胞瘤临床病理观察

目的:探讨睾丸大细胞钙化型支持细胞瘤的临床及病理组织学特征。方法:运用组织学、免疫组化及组织化学技术对1例睾丸大细胞钙化型支持细胞瘤进行光镜观察及免疫标记,并结合相关文献对其临床表现、组织形态、免疫组化特点及治疗和预后等进行分析。结果:患者为青年男性,病理组织学表现为肿瘤细胞排列成巢状、梁状,细胞呈多角形,胞质嗜酸,核大空泡状,伴间质广泛钙化。免疫组化染色显示肿瘤细胞inh ib in(+)、S-100(+)和vim entin(+),PLAP(-)、SMA(-)、CK(-)、AFP(-)。组织化学染色阿辛蓝(+)、过碘酸雪夫氏反应(PAS)阴性。结论:大细胞钙化型支持细胞瘤是一种罕见的睾丸性索间质肿瘤;免疫组化有助于睾丸大细胞钙化型支持细胞瘤的诊断,鉴别诊断包括精原细胞瘤的管状型、睾丸间质细胞瘤(Leyd ig细胞瘤)、普通型及硬化型支持细胞瘤、雄激素不敏感综合征以及隐睾中的支持细胞结节等,手术切除预后良好。