肝胚胎性肉瘤的临床病例分析

  目的  分析肝脏未分化(胚胎性)肉瘤(UESL)的临床表现、影像特点以及治疗方案, 为诊断和治疗提供依据。  方法  报道广西医科大学附属肿瘤医院收治的2例UESL, 并检索国内近25年间文献报道的UESL, 共120例, 提取病例资料进行统计分析。  结果  UESL病例, 多表现为腹部包块, HBsAg和AFP阳性率低(17.72%和8.64%), 肿瘤平均直径为(13.82±4.90)em。72.34%的病例超声表现以实质性高回声为主, 91.78%的病例CT以囊性低密度为主要表现。单纯手术切除组的术后复发率高于手术联合化疗组(为72.97%和30.77%), 但两组的复发时间和生存曲线无明显差别。总体中位生存期为11(5.4~16.6)个月。  结论  UESL具有独特的影像学特征, 早期诊断及早期手术切除是治疗UESL的关键, 术后联合化疗有望降低术后复发风险, 延长生存时间。      

肺动脉肉瘤诊断及治疗进展

肺动脉肉瘤是一种极其罕见的肺血管系统恶性肿瘤,常起源于肺动脉内膜,其病因及发病机制不明。患者的临床表现、实验室及影像学检查常无明显特异性,故诊断难度大,常误诊为急慢性肺栓塞。CT肺动脉造影及MRI检查对肺动脉肉瘤的诊断有重要意义。当患者临床表现与影像学表现不符,溶栓治疗无效时,应高度警惕本病可能,其最终诊断需组织病理学检查。肺动脉内膜剥脱术、肺切除术等外科手术治疗可以改善患者的临床症状并延长患者的生存期,术后辅以放化疗可以增加存活率,但整体预后欠佳。     

原发中枢神经系统恶性淋巴瘤的诊断及治疗

目的：探讨原发中枢神经系统恶性淋巴瘤（PCNSL）的临床、影像学表现,治疗方案选择和预后。方法：对24例PCNSL的诊断、治疗过程、预后进行回顾性分析。结果：PCNSL的临床表现以颅内压增高、局灶占位性病变损伤症状为主,肿瘤可单发或多发;肿瘤影像学缺乏特异性,确诊依靠病理学诊断。结论：PCNSL恶性度高,预后不良,手术全切率低;对确诊病例采用个体化的手术方案结合术后放、化疗是治疗本病的关键,可显著延长患者生存时间。     

原发性中枢神经系统淋巴瘤六例

 目的　探讨原发性中枢神经系统淋巴瘤（PCNSL）的临床、影像学、病理学特点及其治疗和预后。方法　对1998年7月至2007年10月收治的6例PCNSL的临床资料进行回顾性分析。结果　患者常表现为颅内压增高的症状,多数被误诊为转移瘤或其他肿瘤;CT表现为等密度或稍高密度占位性病变,形态不规则,MRI表现为T1低或等信号,T2高信号,瘤周轻度水肿,两者增强扫描后肿物均匀强化;B细胞肿瘤5例（83.3 ％）。全组病例均行手术治疗,显微镜下全切除术4例,部分切除2例。术后化疗1例,放疗2例。生存期为5个月～3年7个月。结论　PCNSL临床表现及影像学检查缺乏特异性,术前诊断困难,极易误诊,确诊需病理学检查,术后需行化疗及放疗,该病预后差。     

原发中枢神经系统恶性淋巴瘤的诊断及治疗

目的:探讨原发中枢神经系统恶性淋巴瘤(PCNSL)的临床、影像学表现,治疗方案选择和预后. 方法: 对24例PCNSL的诊断、治疗过程、预后进行回顾性分析. 结果: PCNSL的临床表现以颅内压增高、局灶占位性病变损伤症状为主,肿瘤可单发或多发;肿瘤影像学缺乏特异性,确诊依靠病理学诊断. 结论: PCNSL恶性度高,预后不良,手术全切率低;对确诊病例采用个体化的手术方案结合术后放、化疗是治疗本病的关键,可显著延长患者生存时间.     

急性淋巴细胞白血病化疗后可逆性后部白质脑病综合征一例并文献复习

目的 探讨急性淋巴细胞白血病巩固化疗后可逆性后部白质脑病综合征(RPLS)的临床表现、诊治及其预后情况.方法 回顾分析1例急性淋巴细胞白血病患者的临床及影像学资料并结合文献进行复习.结果 该患者主要临床表现为腹胀、反复发热,伴乏力症状,经血常规及骨髓相关检查,诊断为早前B细胞性高危组急性淋巴细胞白血病,经诱导缓解化疗及巩固化疗后患者出现血压增高及神经系统症状,结合影像学检查诊断为RPLS,经积极治疗后完全恢复,影像学表现迅速改善.结论 RPLS可由多种病因产生,临床表现及影像学检查缺乏特异性,一般预后较好,早期作出正确诊治是关键.     

原发性颅内恶性淋巴瘤17例临床分析

总结原发性颅内恶性淋巴瘤的临床、影像学及病理学特征。回顾性分析17例原发性颅内恶性淋巴瘤的临床资料,并对其临床、影像学及病理学检查进行分析。原发性颅内恶性淋巴瘤是颅内少见的肿瘤,临床表现复杂,颅内高压是其主要表现之一(13/17),颅脑CT和MRI检查显示肿瘤位于额颞叶多见(12/17),主要发病起源于B细胞,综合治疗效果好,总体预后差。初步研究结果提示,原发性颅内恶性淋巴瘤的临床与影像学特征缺乏特异性表现,早期诊断困难,临床误诊率高,颅内活组织病理学检查是确诊本病的主要手段,综合治疗(手术放疗和化疗)可取得较好的疗效。     

原发性颅内恶性淋巴瘤17例临床分析

总结原发性颅内恶性淋巴瘤的临床、影像学及病理学特征。回顾性分析17例原发性颅内恶性淋巴瘤的临床资料，并对其临床、影像学及病理学检查进行分析。原发性颅内恶性淋巴瘤是颅内少见的肿瘤，临床表现复杂，颅内高压是其主要表现之一（13／17），颅脑CT和MRI检查显示肿瘤位于额颞叶多见（12／17），主要发病起源于B细胞，综合治疗效果好，总体预后差。初步研究结果提示，原发性颅内恶性淋巴瘤的临床与影像学特征缺乏特异性表现，早期诊断困难，临床误诊率高，颅内活组织病理学检查是确诊本病的主要手段，综合治疗（手术放疗和化疗）可取得较好的疗效。     

原发性中枢神经系统恶性淋巴瘤18例分析

目的：探讨原发性中枢神经系统恶性淋巴瘤（PCNSL）的临床、影像学与病理学特征。方法：对18例PCNSL的临床表现、实验室结果、影像学与病理学检查进行分析。结果：PCNSL临床表现复杂,颅内高压为其主要表现之一,脑脊液中蛋白定量增高,中构神经系统CT以及MRI检查显示肿瘤侵及额叶与颞叶多见,PCNSL主要起源于B细胞,综合治疗效果好。结论：PCNSL临床与影像学缺乏特异性表现,早期诊断困难,易被误诊,脑活组织病理学检查是确诊本病的主要手段,手术后综合放化疗可取得较好的效果。     

肾上腺淋巴管瘤的诊疗分析(附2例报告并文献复习)

目的:探讨肾上腺淋巴管瘤的临床特点、诊断、鉴别诊断、治疗及预后.方法:报告2例肾上腺淋巴管瘤病例资料.例1,男,54岁.因右腰肋部持续性钝痛2个月入院,行腹部超声检查时发现右侧肾上腺区囊性占位,CT扫描提示右侧肾上腺区可见一大小约6.0cm×3.8cm哑铃状囊性占位,内见分隔,囊壁可见点状钙化,增强扫描时肿物无明显强化.例2,女,43岁.因体检发现左侧肾上腺肿物1周入院,CT检查提示左侧肾上腺区可见一大小约3.2cm×1.8cm低密度占位病变,内部不均匀,增强扫描可见肿物轻度强化.结果:2例患者均行腹腔镜下肾上腺切除术.术后组织病理学检查结果为肾上腺淋巴管瘤.分别随访2年、16个月,均无复发.结论:肾上腺淋巴管瘤是一种极为罕见的肾上腺良性肿瘤,通常无明显临床表现.术前诊断主要依靠影像学检查,确诊需组织病理学检查.手术治疗为首选方法,预后较好.     

Undifferentiated embryonal sarcoma of the liver in adults

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL), a rare tumor that predominantly affects children, generally has been considered an aggressive neoplasm with an unfavorable prognosis. More recent reports have indicated that modern multimodal treatment and supportive care improve the survival of children with UESL. Data regarding the treatment and survival of adults have not been reviewed comprehensively, and only a few adult patients with UESL have been reported in the literature. METHODS: The authors analyzed demographics, treatment, and actuarial survival of all reported cases of UESL in patients aged >or=15 years (n = 67 patients). In addition, 1 case is presented of a patient with UESL who was treated successfully at the authors' institution. RESULTS: The median survival of all patients with UESL who were analyzed was 29 months. Patients who underwent complete tumor resection followed by adjuvant chemotherapy survived over a median follow-up of 28.5 months and had significantly better survival compared with patients who underwent surgical treatment alone. Patients who underwent an incomplete tumor resection had a tendency toward poorer outcomes. CONCLUSIONS: To the authors' knowledge, this is the first report to demonstrate a significant effect on survival for adjuvant chemotherapy after complete surgical resection of UESL in adults. The role of neoadjuvant chemotherapy was not evaluated in this study. In the case study presented herein, combined therapy with surgery and chemotherapy led to a complete, sustained remission that has lasted for >6 years to date.     

Undifferentiated sarcoma of the liver in childhood: a curable disease

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis. METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease. Radiotherapy was occasionally used (2 of 17 patients). RESULTS: Twelve patients are alive with follow-up ranging from 2.4 to 20 years. Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive. After initial chemotherapy tumor reduction was evident in six of nine evaluable cases. Overall three patients died of disease and one of a surgical complication. One child died in second complete remission for a non-disease-related cause. CONCLUSIONS: The current prognosis of UESL no longer should be regarded as poor. Modern multimodal treatment and supportive therapy have improved survival.     

Mesenchymal Hamartoma of the Liver in Children： a Report of Four Cases

OBJECTIVE Mesenchymal hamartoma of the liver (MHL) is a rare disease that makes up about 5%-8% of primitive hepatic tumors in children. The etiology of MHL has been the subject of considerable discussion. The possibility of malignant transformation of MHL is inclusive. To enhance the recognition of MHL in children, we have review the procedures in diagnosis and treatment and present 4 of our own cases. METHODS The clinical characteristics of MHL in 4 children including history, physical examination, lab examination, imaging data and pathology were analyzed and discussed and related to reports from the literature. RESULTS All the cases were diagnosed with B-ultrasonography and CT preoperatively, and were confirmed intra-operatively and histologically. Satisfactory results were reached postoperatively. CONCLUSION MHL is an uncommon benign lesion in children. The main presentation is abdominal painless mass. Diagnosis is aided with B-ultrasonography and CT preoperatively. Complete tumor removal is the best choice for MHL treatment resulting in a good outcome.     

原发性肝脏类癌的诊断一例报道

Primary hepatic carcinoid tumor (PHCT) is a extremely rare neoplasm, usually lacking specific symptoms. We present a histologically proved case and review the cases in the literature. The patient was an 65-year-old man with right upper abdominal pain and weight loss who underwent exploratory laparotomy for huge mass of the liver. Ultrasonography showed a well-demarked, cystic mass with irregular thick wall, whereas CT revealed a low-density tumor in nephrographic phase and peripheral enhanced areas in artery phase. He was diagnosed as PHCT by histological findings including positive staining of tumor cells for chromogranin A and synaptophysin, and no evidence of other primary source of tumor. Thus, a patient with this type of mass showed by imaging examination should be suspected of PHCT, for it is of great importance to clinical treatment.     

肾上腺皮质癌1例报道并文献回顾

目的:探讨肾上腺皮质癌的临床表现、诊断及治疗方法。方法:报道我科近期收治的1例肾上腺皮质癌患者的临床资料,并检索中国知网、PubMed等数据库,进行文献回顾,对该病的临床特征及诊断要点、治疗方法及最新的研究进展进行归纳总结。结果:本例患者以腹痛为首发临床表现,影像学提示肾上腺肿瘤,行腹腔镜切除,术后20天查PET/CT提示多发转移,给予依托泊苷+顺铂化疗4周期后进展,遂应用吉西他滨联合卡培他滨化疗1周期,出现脑转移后行放疗。结论:肾上腺皮质癌是一种罕见的泌尿系恶性肿瘤,且预后差。诊断应结合临床表现、肾上腺皮质激素水平、影像学检查及病理结果。治疗方面首推多学科会诊（MDT）,早期患者首选手术,术后需根据疾病分期、分级选择辅助治疗,复发转移的患者可给予化疗、放疗、米托坦、免疫治疗等。     

小儿腹部节细胞神经瘤的诊断和治疗

目的提高小儿腹腔节细胞神经瘤的诊治水平。方法回顾分析3例腹腔节细胞神经瘤的临床资料，结合文献对其发病情况、生物学特性、临床、病理诊断及手术原则进行讨论。结果术前经B超、CT检查，2例分别诊断神经母细胞瘤和腹膜后肿瘤；1例考虑节细胞神经瘤。3例均手术切除肿瘤，2例为腹膜后节细胞神经瘤，1例为肾上腺节细胞神经瘤。术后恢复顺利。随访6个月～8年，患儿健康，无肿瘤复发。结论小儿腹部节细胞神经瘤临床少见，平均发病年龄6岁左右，除腹部包块外，一般情况良好。B超、CT可了解肿瘤的部位及其与周围组织、脏器的关系，对于诊断和指导手术有帮助。确诊有赖病理。完全切除肿瘤，预后良好。     

儿童原发中枢神经系统间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤一例并文献复习

目的:探讨儿童原发中枢神经系统（CNS）间变性大细胞淋巴瘤（ALCL）的临床特征及预后。方法:回顾性分析福建医科大学附属协和医院收治的1例原发CNS间变性淋巴瘤激酶（ALK）阳性ALCL患儿的临床资料,并复习相关文献。结果:患儿以头痛、发热为主要症状就诊外院,颅脑磁共振成像提示右侧小脑肿块,术前无CNS以外淋巴瘤浸润证据,行小脑肿瘤切除术,术后病理明确诊断为ALK阳性ALCL,未及时化疗。术后第27天转入福建医科大学附属协和医院,化疗前评估肿瘤已扩散至骨髓、睾丸、椎骨等部位,外周血NPM-ALK融合基因阳性。2个疗程化疗后达完全缓解,但最终死于化疗相关并发症。结论:原发CNS的ALK阳性ALCL病例罕见,易误诊,病情进展快,总体预后不良;及时进行活组织病理检查以确诊,早期行以化疗为主的综合治疗可能改善患者预后。     

睾丸内胚窦瘤1例报告并文献复习

目的:探讨睾丸内胚窦瘤的生物学行为、临床病理特点、诊断治疗方法及其预后。方法:报告1例睾丸内胚窦瘤的诊断治疗及随访情况,并结合国内外文献进行分析。结果:本例患者肿瘤分期为Ⅰ期,行患侧睾丸肿瘤根治性切除术,术后给予PVB方案化疗3个疗程,效果良好,随访24个月,直至最后一次复查,患者AFP、HCG处于正常水平,相关影像学检查未见复发及远处转移。结论:睾丸内胚窦瘤临床罕见,恶性程度高,总体预后差,其确诊依靠病理,外科手术为主要治疗手段,运用手术加放、化疗的综合治疗措施可显著延长生存期。     

Hepatocellular carcinoma with multiple lung metastasis resulting in long-term disease-free survival by transcatheter arterial infusion chemotherapy of SMANCS

The patient was a 61-year-old female with alcoholic liver cirrhosis, who was admitted to our hospital due to elevation of AFP.During the evaluation, both abdominal ultrasound and enhanced abdominal CT revealed a hepatocellular carcinoma measuring 4 cm in the S6-7 region, complicated with an arteriovenous shunt.Additionally, the lung CT examination showed 20 isolated bilateral lung tumors, all of which were less than 1.4 cm in diameter. Following the diagnosis, we performed a transcatheter arterial infusion chemotherapy of SMANCS at 3 mg through the right heptic artery. Thereafter, the AFP level returned to normal. Additionally, the tumors previously observed in both liver and lung, and exhibited by both lung CT and enhanced abdominal MRI, had disappeared.The patient has been in clinical remission more than 10 years to date.