Castleman病9例报道

目的总结Castleman病的临床特征及诊治经验。方法回顾性分析2010年1月至2014年12月期间我院收治的9例Castleman病患者的临床资料。结果本组患者发病部位以颈部多见(4例),病理分型以透明血管型多见(5例),5例局灶型Castleman病患者经手术治疗取得良好效果;3例多中心型Castleman病患者经环磷酰胺+多柔比星+长春新碱+泼尼松(CHOP)化疗后病情得到好转,1例中心型Castleman病患者因基础疾病未化疗而复发。结论 Castleman病是一种罕见的淋巴系统增生性疾病,无特异性表现,诊断依赖于病理组织学,手术是治疗局灶型Castleman病的首选方法,CHOP化疗方案是治疗中心型Castleman病的一种有效方案。     

结肠癌合并腹部局灶性Castleman病1例误诊分析

Castleman病(Castleman's disease,CD)又称血管滤泡性淋巴组织增生病或巨大淋巴结增生,是一种少见的病因未明的反应性淋巴结病。结肠癌合并腹部局灶性Castleman病目前尚未见报道,极易误诊。我科收治1例误诊的结肠癌合并腹部局灶型CD。报告如下。     

腹膜后局限性Castleman病20例临床诊治及预后分析

目的 探讨腹膜后局限性Castleman病的临床特征、诊治策略及影响预后的因素,提高伴副肿瘤性天疱疮(PNP)的Castleman病的诊治水平.方法 回顾性分析1993年1月至2009年5月在北京大学第一医院普通外科接受手术治疗的20例腹膜后Castleman病患者的临床资料,并将13例腹膜后Castleman病伴PNP患者的临床资料与同期7例单纯腹膜后Castleman病患者进行比较,分析两者在发病特点、部位、实验室检查、手术策略、病理特征及外科治疗效果的不同.结果 (1)本组腹膜后Castleman病多见于中青年(中位年龄36岁),发病部位多位于肾旁(14/20,70%)及髂窝(4/20,20%),左侧腹膜后多见,病理分型以透明血管型为主,腹膜后Castleman病合并PNP组的性别、年龄、肿瘤发病部位、大小及病理分型与单纯腹膜后Castleman病组无明显区别(P>0.05);(2)本组腹膜后Castleman病合并PNP患者较易合并闭塞性细支气管炎(8/13),有血清肿瘤标志物癌胚抗原或CA242升高现象(3/8);(3)本组腹膜后Castleman病常有包膜,与邻近脏器边界清晰,手术较为容易,但合并PNP的腹膜后Castleman病的生物学行为有恶性倾向及伴发子灶特征,有侵及邻近血管及术后局部复发可能;(4)Kaplan-Meier及Log-Rank生存分析显示,腹膜后Castleman病合并PNP患者5年生存率为42.8%,平均生存时间30个月,明显低于单纯腹膜后Castleman病组(P<0.05),是否合并闭塞性细支气管炎以及肿瘤能否根治切除是影响腹膜后Castleman病患者预后的重要因素.结论 腹膜后Castleman病伴PNP具有独特的临床特征,早期诊断和切除肿瘤、及时终止致病抗体的产生,是成功治愈的关键.     

65例巨大淋巴结增生症临床病理分析

目的:探讨巨大淋巴结增生症(Castleman病)的临床病理学特征、诊断及鉴别诊断和治疗方法。方法:对65例巨大淋巴结增生症患者的临床资料进行回顾性分析,包括光镜观察、免疫组化标记、临床病理分析及治疗、预后等。结果:巨大淋巴结增生症免疫标记物CD20、CD21、kappa、lamda、CD38、CD138阳性。63例为局限型,其中2例恶变,1例恶变为弥漫大B细胞性淋巴瘤,1例恶变为血管内皮细胞肉瘤,均经过化疗缓解。2例为多中心型,均经过CHOP治疗缓解。结论:巨大淋巴结增生症是一种交界性淋巴组织增生性疾病,多中心型常为恶性结局;手术切除是本病的主要治疗手段,预后良好,多中心型及恶变者需行积极的全身化疗。     

腹膜后Castleman病一例

腹膜后Castleman病(局灶型,浆细胞型)临床罕见,术前诊断困难.我们手术治疗1例,术后恢复顺利,现报道如下.     

腹部Castleman病的临床诊断和治疗

目的 总结腹部Castleman病的诊断和治疗经验.方法 回顾性分析1985年6月至2009年3月北京协和医院收治的17例腹部Castleman病患者的临床资料.11例患者无任何症状,2例表现为腹部包块,2例表现为口腔溃疡、皮疹,1例表现为浮肿、气短,1例表现为上腹部隐痛、恶心、呕吐.2例行X线检查,17例行超声检查,13例行CT检查.结果 4例术前经CT检查诊断为Castleman病,其余13例均未明确诊断.14例局灶型患者经手术完整切除肿瘤,3例多中心型患者行部分切除或活组织检查.所有患者经病理检查确诊,其中14例透明血管型患者的免疫组织化学检查结果为CD3、CD20、CD21、CD34阳性;3例浆细胞型患者的免疫组织化学检查结果为CD3、CD68、PCNA阳性.17例患者中1例失访,16例随访3～12个月无转移和复发.结论 腹部Castleman病临床表现无特异性,术前诊断困难.局灶型患者应尽早行手术完整切除,预后良好;多中心型难以完整切除,可于术后进行化疗,预后较好.     

腹膜后单中心型Castleman病12例诊疗分析

目的 分析腹膜后Castleman病的临床特征,总结诊疗经验。方法 回顾性分析2008—2018年间南京医科大学第一附属医院收治的12例腹膜后单中心型Castleman病的临床资料。结果 所有患者行开放或腹腔镜腹膜后(外)肿物切除术,其中2例加行术后辅助化疗。术后病理证实Castleman病:透明血管型11例,浆细胞型1例。随访时间1～102个月,中位随访时间46个月。12例患者随访均未提示复发,4例患者术后复查CT示腹主动脉周围淋巴结减小。结论 腹膜后Castleman病术前难以明确诊断,需术后病理证实。手术疗效好,放疗亦可作为一种替代疗法,但化疗在单中心型Castleman病治疗中的应用还需要更多的探讨。     

腹膜后局灶性Castleman病2例报告并文献复习

目的：复习Castleman病的原因,提高Castleman病的诊治水平。方法：回顾性分析收治2例腹膜后局灶性Castleman病患者的临床病例资料,结合复习相关文献。结果：2例腹膜后局灶性Castleman病患者均无明显临床症状及体征,为B超检查偶然发现;呈单个淋巴结增生,表现为腹膜后占位性病变;均行肿物切除术,病理确诊为Castleman病,术后随访无复发。结论：腹膜后局灶性Castleman病主要表现为单一部位的淋巴结肿大,多无临床症状,也无实验室异常检测结果及特征性影像学表现。术前不易明确诊断,最终的确诊依靠病理诊断。该病手术切除疗效好,术后可长期生存。     

15例Castleman病的临床特点及病理学分析

目的探讨Castleman病的病理学、临床表现和治疗特点。方法回顾分析确诊为Castleman病的15例患者的临床资料,病理组织采用HE切片光镜观察。结果局灶型9例,多中心型6例。结论 Castleman病的诊断依赖于病理组织学形态,临床误诊率高,其特征性表现为淋巴组织增生性的病理组织学改变,治疗以综合治疗为主。     

Castleman病2例临床探讨及文献复习

目的 提高对Castleman病的认识。方法分析2例Castleman病的临床资料并复习有关文献。结果 1例为局灶性透明血管型，主要表现为浅表淋巴结肿大，经手术切除及肾上腺糖皮质激素治疗预后好。另l例为多中心性混合型，主要表现为贫血、蛋白尿、血沉增快、多克隆丙种球蛋白血症和低白蛋白血症，经综合治疗好转。结论 Castleman病为淋巴组织增生性疾病，临床表现多样，早期诊断困难；临床上和病理上需与多种疾病鉴别，病理的鉴别尤其重要。     

儿童C1q肾病的临床病理特点及治疗

目的 探讨儿童C1q肾病的临床、病理特点及治疗方法。 方法 回顾性分析本院8年来经肾活检确诊的23例C1q肾病患儿临床、病理和预后资料。 结果 C1q肾病占同期肾活检的原发性肾小球疾病的4.78%。23例患儿中,男15例,女8例;年龄10个月~12岁5个月,平均发病年龄（5.0±3.4）岁;肾病综合征（NS） 18例（2例伴镜下血尿）,肾病水平蛋白尿4例（1例伴镜下血尿）,单纯镜下血尿1例。1例NS起病前曾服用2周中药,发病时同时并发急性肾功能不全。3例患儿有肾脏病家族史,其中2例（肾病水平蛋白尿）为姐弟,父亲亦有蛋白尿,基因检测证实为家族性Denys-Drash综合征并发C1q肾病。1例患儿（NS）姐姐亦有大量蛋白尿（未行肾活检）。所有患儿起病时血压均正常,补体正常,抗核抗体、抗dsDNA抗体、抗Sm抗体及乙肝两对半均阴性。18例NS中13例激素耐药（72.2%）,4例激素依赖,1例激素敏感。光镜下,13例为微小病变（MCD）（其中1例伴间质性肾炎）;6例为系膜增生性肾小球肾炎（MsPGN）;4例为局灶节段性肾小球硬化（FSGS）。另9例患儿伴有不同程度的小管萎缩和间质纤维化。免疫荧光下,所有患儿均见系膜区弥漫性C1q≥2+沉积,其中伴IgG沉积18例,IgM沉积18例,IgA沉积8例,C3沉积11例,6例患儿呈“满堂亮”表现。除4例患儿电镜下未见肾小球外,其余19例中4例系膜区见电子致密物沉积。12例激素耐药（包括2例肾病水平蛋白尿者）及3例激素依赖患儿在激素治疗基础上加用静脉CTX冲击;3例激素耐药者加用环孢素A（CsA）口服;1例激素依赖患儿给予足量激素重新诱导;1例单纯镜下血尿患儿及2例Denys-Drash综合征并发C1q肾病患儿仅给予血管紧张素转换酶抑制剂（ACEI）治疗。其中1例患儿CTX冲击满疗程无效后换用CsA治疗;1例患儿CTX冲击满疗程无效后换用FK506治疗。23例患儿中,1例失访,1例治疗时间<3个月未纳入随访对象,2例Denys-Drash综合征目前不能通过药物治疗好转未纳入疗效统计,余19例中,15例完全缓解（78.9%）,2例部分缓解（10.5%）,2例无效（10.5%）。NS患儿总缓解比例94.4%（17/18）,肾病水平蛋白尿患儿总缓解比例50.0%（2/4）。病理为MCD者总缓解比例100.0%,MsPGN者缓解比例83.4%,FSGS缓解比例50.0%。随访末所有患儿血压、肾功能均正常,自身抗体均阴性,补体水平均正常。 结论 C1q肾病罕见,临床以NS或肾病水平蛋白尿为主,且往往激素耐药或激素依赖;病理以MCD为主,也可表现为MsPGN或FSGS。加用其他免疫抑制剂治疗后,MCD和MsPGN者多可获缓解,但FSGS预后欠佳。     

Intravenous pulse cyclophosphamide—is it effective in children with steroid-resistant nephrotic syndrome?

Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a challenge to pediatric nephrologists. Recently, intravenous cyclophosphamide (IV-CPM) infusion was shown to be effective, safe, and economical for the treatment of SRNS, particularly minimal change disease (MCD), as it results in more sustained remissions, longer periods without proteinuria, and fewer significant side effects at a lower cumulative dose. A prospective study was conducted to evaluate IV-CPM infusions in the management of children with SRNS secondary to MCD or IgM nephropathy. Five patients with SRNS (4 IgM nephropathy and 1 MCD) received six monthly IV-CPM infusions at a dose of 500 mg/m². No patient achieved complete or sustained remission. Three patients attained partial remission, which was not sustained for more than 1 month post therapy. One patient progressed rapidly to end-stage renal disease during treatment. Side effects included vomiting in four patients and alopecia in one patient. Conclusion: IV-CPM pulse therapy at a dose of 500 mg/m² is unsuccessful in obtaining complete or sustained remission in children with SRNS secondary to IGM nephropathy or MCD. Further randomized controlled studies with higher doses are required.     

Surgical outcome of superficial and deep Castleman disease

BACKGROUND: Castleman disease is a rare lymphoproliferative disease of low malignant potential occurring in two forms, unicentric and multicentric. Surgery, chemotherapy, immunotherapy and radiation therapy have all been used to manage the disease. In this study, we evaluate whether the site of the lesions, that is, superficial or deep, influences the surgical outcome. METHODS: We retrospectively reviewed the records of 20 patients operated on for Castleman disease from 1994 to 2003, of whom 11 patients had superficial disease and 9 had deep lesions. The end-points of this study were survival and recurrence. RESULTS: Of the 20 patients, 19 had unicentric (cervical in 8, mediastinal in 5, retroperitoneal in 2, axillary in 2, hepatic in 1, and mesenteric in 1) and 1 had multicentric Castleman disease. Among 19 patients who had complete resection (18 with unicentric and 1 with multicentric disease), there has been no evidence of recurrence. CONCLUSION: Whether Castleman disease is superficial or deep has no effect on surgical outcome as long as resection is complete.     

肾上腺区域和肾周Castleman病5例报道

目的分析5例肾周和肾上腺区域Castleman病(Castleman disease,CD)的临床特点,提高对CD诊治的认识。方法结合文献复习,回顾分析本院2006年5月至2014年6月5例肾周和肾上腺区域CD的临床表现、影像学特点、治疗和预后。结果本组5例,男3例,女2例,中位年龄54岁(33～58岁)。CD病灶位于肾上腺区域3例,肾周2例。其中体检发现2例,因腰腹痛就诊2例,因发热和肌痛就诊1例。肿块最大径2.8～9.6cm,平均5.3cm。术前诊断为肾上腺良性肿瘤1例,肾上腺或肾恶性肿瘤2例,淋巴瘤1例,肾周Castleman病1例。影像学表现多无特异性,超声检查CD病灶多表现为界限清晰的低回声肿,较大的肿瘤中心部位可见点状钙化;CT平扫为均质肿块,可伴有微小星点状钙化,增强扫描呈明显强化的均质富血供肿块;MRI检查肿块呈T_1WI呈低或等信号,T_2WI呈均质等或高信号,增强扫描可明显强化。体积较大的肿块在CT和MRI动态增强扫描时,表现为从周边强化开始,逐渐向中间弥散的均质肿块。实验室检查除1例有发热等全身症状者C反应蛋白和血沉升高外,其余4例均未见异常。5例患者中,1例行右肾上腺及右肾切除,2例行腹腔镜左肾上腺及后腹膜淋巴结切除,1例行右肾根治+腹膜后淋巴结切除,1例行左肾门旁肿块切除。术后病理均为Castleman病,其中透明血管型4例,术后中位随访时间59.5个月(6～103个月)均未复发;浆细胞型1例,术后54个月腹膜后复发并骨转移。根据病理、临床特点及影像学临床最终诊断为局灶型Castleman病(LCD)4例,多中心型Castleman病(MCD)1例。结论手术切除是肾周和肾上腺区域LCD的首选治疗,MCD手术效果不佳。因病灶部位的特殊性,以及临床和影像学表现的非特异性,该区域的CD易误诊为肾和肾上腺恶性肿瘤,导致不必要的脏器切除。对于该区域的均质、血供丰富,特别是伴有微小点状钙化、从周边开始弥散增强的肿块,均应怀疑CD。术中活检再次评估,或可避免切除范围的扩大。     

The role of surgery in advanced testicular cancer

As from 1976 65 patients with advanced testicular cancer (non-seminomatous) were treated with intensive chemotherapy. In 28 cases chemotherapy induced a complete remission; in six cases the tumor masses did not disappear, with persisting high level of markers. In 31 cases the markers normalized but abnormalities persisted on palpation and/or on X-rays and CT-scans. In these cases operations were performed. Biopsies were taken (11/31) or, if possible, residual lesions were completely excised (20/31) without attempts to do radical retroperitoneal dissections or aggressive or dangerous debulking. Only in a minority of cases (five patients) vital tumor was found; in six cases mature teratoma was found; in all other cases only necrosis or scar tissue was found. The findings in these staging operations indicated the further treatment. Patients with vital tumor continued with intensive chemotherapy. The others received a consolidation course or no further treatment. The actuarial four-year survival rate of these operated re-staged patients exceeds 90%. All three patients with vital tumor, where complete excision was possible, are still alive in complete remission, and one of two patients where vital tumor was only biopsied, is still in complete remission of long duration after continuation of chemotherapy.     

Treatment of metastatic germ cell tumors with etoposide and cisplatin as first line chemotherapy

PURPOSE: The efficacy and toxicity of two-drug therapy (etoposide and cisplatin, EP) in patients with metastatic germ cell tumors were investigated. PATIENTS AND METHODS: Between December 1996 and November 1999, 18 patients with metastatic germ cell tumors (6 seminomas and 12 non-seminomas, Stage II 8, Stage IIIA 2, Stage IIIB 6, Stage IIIC 2) were treated by 3-5 cycles of induction chemotherapy regimen (EP). Etoposide and cisplatin were administrated in doses of 100 mg/m2 and 20 mg/m2, respectively, on days 1 to 5 and then repeated from day 21. After tumor markers obtained normal levels, one or two additional cycles of EP were continued. Patients showing evidence of residual tumor mass underwent debulking surgery as early as possible. RESULTS: At the end of EP therapy, 4 (22%) of the 18 patients achieved complete remission and 14 patients (78%) showed partial remission. Seven patients of partial remission were treated by excision of residual abnormalities: 6 had pathologically necrotic debris in the resected specimen and 1 had teratoma, and these 7 patients all achieved complete remission. Four other patients achieving partial remission were followed without surgical excision and have had no evidence of disease progression. Remaining three patients achieving partial remission received salvage chemotherapy with or without adjunctive surgery, resulted in complete remission in 2 patients and partial remission in 1 patient. EP demonstrated to have less treatment-related toxicity compared with that of EBP. Follow up studies ranging from 12 to 47 months (median, 29.6) showed that one patient experienced a relapse from complete remission at 13 months and was salvaged by chemotherapy and surgery. Finally, thirteen patients (72%) who achieved complete remission are alive and disease-free and 5 patients (28%) showing partial remission are alive with negative tumor markers and no evidence of relapse. CONCLUSION: These results suggests that EP is an efficacious and less toxic first line regimen for good-prognosis patients with metastatic germ cell tumors.     

Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma

Multicentric Castleman disease (MCD), a lymphoproliferative disorder and Kaposi sarcoma (KS), a vascular tumor, both occur at a higher frequency among patients with human immunodeficiency virus (HIV) infection. Human herpes virus 8 (HHV8), with an ability to infect and persist in B-lymphoid cells and endothelial cells, is causally associated with both MCD and KS. The coexistence of these HHV8-associated diseases in the same tissue samples has hitherto not been investigated. In this report, we compile the histologic and immunohistochemical findings in 24 lymph node (LN) and 5 spleen samples from 26 patients documented to have HIV-associated MCD. In addition to MCD, 15 of 24 LN samples (63%) showed evidence of coexisting KS. The involvement by KS was typically "microscopic" and involved the LN capsule, trabeculae, or hilum. Examination of 5 spleens involved by MCD did not show any evidence of KS. These were compared with LN biopsies from HIV patients with neither granulomatous diseases, metastatic carcinomas nor lymphoproliferative disorders. Among 20 LN biopsies from 19 individuals without MCD, 5 LNs showed involvement by KS (25%); an association significantly lower than LNs with MCD (Pearson chi 2: 6.2, 2-sided significance: 0.013). Coexistence of MCD and KS in the same tissue sample is a common phenomenon and we hypothesise that the association is due to lytic HHV8 infection of B-lymphoid cells exposing susceptible endothelial cells at vulnerable subsites within the LNs to extremely high levels of HHV8 resulting in formation of KS tumorlets in MCD-LNs.     

Cisplatin, cyclophosphamide and doxorubicin chemotherapy for unresectable urothelial tumors: the M.D. Anderson experience

We reviewed retrospectively 97 patients treated with cisplatin, cyclophosphamide and doxorubicin chemotherapy at our hospital to evaluate predictive variables for response to chemotherapy and long-term survival free of disease. Histological subtype influenced response: 70 per cent of the patients with pure transitional cell carcinoma achieved an objective response (partial remission 31 per cent and complete remission 39 per cent), whereas 45 per cent of those with mixed tumors achieved response (partial remission 20 per cent and complete remission 25 per cent). Patients with nodal metastases only had an equal over-all response rate to those with visceral metastasis (64 versus 62 per cent) but patients with nodal metastases had a higher complete remission rate (45 versus 20 per cent). A total of 35 patients (36 per cent) achieved a complete response. In 17 of the 35 patients the duration of response was less than 100 weeks and 18 (51 per cent) have survived longer than 100 weeks. Of the 17 patients with a survival free of disease of less than 100 weeks 16 died of recurrent urothelial tumors and 1 died of a second primary tumor. Among the patients with a survival free of disease of longer than 100 weeks only 2 have had recurrent urothelial tumors; 72 per cent of the patients in this category remain free of disease. Patients with pure transitional cell carcinoma were represented in equal frequency among patients achieving a durable complete remission and those with a complete remission of less than 100 weeks. These data demonstrate the ability of cisplatin, cyclophosphamide and doxorubicin combination chemotherapy to achieve a complete remission and long-term survival free of disease among select patients with unresectable urothelial tumors.     

Prognostic factors in advanced head and neck cancer patients undergoing multimodality therapy

A retrospective analysis was performed to investigate potential prognostic factors for complete remission to neoadjuvant chemotherapy and overall survival in patients with previously untreated stage III and stage IV head and neck cancer. Eighty consecutive patients were treated in one of two studies investigating three or four courses of neoadjuvant chemotherapy. Before local therapy and surgery and/or radiotherapy, 29% attained a complete remission. No strong significant and independent predictor of complete remission was identified. Only nodal stage (N) was found moderately associated with complete remission (p = 0.06). Node-negative patients had higher remission rates. Less important predictors were tumor stage (T) and site of disease; nasopharyngeal patients had superior remission rates (56%). With a median followup of 45 months and estimated 3-year survival rate of 38% (median 23.7 months), individual factors predictive of survival included pretherapy weight loss, performance status, alcohol use, pretherapy serum albumin level, site of disease, and N stage. In multivariate testing weight loss was identified as the strongest independent predictor of survival (p less than 0.0001) and surpassed other health status measures, such as performance status and serum albumin level. In addition, N stage (p = 0.019) and alcohol use (p = 0.017) were found to be predictive. A cross-classification by N stage and weight loss revealed risk groups with distinctly different prognoses, which may be useful for design and analysis in future trials.