泌尿生殖系罕见癌肉瘤4例报告

目的：探讨泌尿生殖系癌肉瘤的诊断和治疗。方法：总结2例阴茎癌肉瘤、1例肾脏癌肉瘤、1例膀胱癌肉瘤患者的临床资料。4例均行手术切除。结果：术后病理和免疫组化证实为癌肉瘤，1例于术后2个月死于呼吸衰竭、1例失访、另2例现已分别生存6个月和10个月。结论：泌尿生殖系癌肉瘤临床罕见，术前诊断困难，免疫组化有助于诊断。恶性程度高，预后差，尽早行根治性切除是最佳治疗方法。     

直肠及肛周炎性肌纤维母细胞瘤三例

目的 探讨直肠及肛周炎性肌纤维母细胞瘤的临床特点及其疗效.方法 回顾性分析2005年1月至2011年6月术后病理证实为直肠或肛周炎性肌纤维母细胞瘤3例患者的临床资料.结果 炎性肌纤维母细胞瘤局部表现为浸润性生长,MRI或CT常表现为富血管实性肿块,影像学难以同直肠癌或肉瘤、血管瘤鉴别,术前肠镜活检或局部针吸活检常难以明确肿瘤性质,需手术切除后整体送检,并行免疫组化确诊.3例患者均行保肛手术,1例患者术后16个月出现复发,再次行根治性手术,随访至今.3例患者已分别存活67、55和35个月.结论 直肠及肛周炎性肌纤维母细胞瘤术前不易确诊,病理诊断主要依赖免疫组化.局部完整切除可以在保留肛门的同时获得肿瘤的根治,局部复发也常有再次手术机会.     

三例胆囊神经内分泌癌的病理特点及诊疗体会

目的 探讨胆囊神经内分泌癌（gallbladder-neuroendocrine carcinomas,GB-NECs）的病理特点及诊疗预后。方法 收集2015年5月至2019年5月期间湖南师范大学附属第一医院收治的3例GB-NECs,回顾性分析该3例患者的病史、实验室检查、影像学检查、治疗方案,探讨患者术后病理特点及其预后。结果 3例患者中男1例,女2例,年龄52～68岁,2例行根治性手术,1例仅行穿刺活检术,术后病理均证实为GB-NECs。术后病理及免疫组化：2例由腺癌和神经内分泌肿瘤（neuroendocrine tumours,NETs）两种成分组成,1例NETs呈弥漫片状生长。免疫组化：Syn、CgA联合阳性率100%,Ki-67阳性指数为75%～85%。3例GB-NECs均为G3级,且恶性程度较高。2例行根治性手术患者未行进一步辅助化疗,1例行穿刺活检术患者仅行进一步姑息化疗。3例患者生存时间分别为8个月、大于10个月、3个月。结论 GBNECs的确诊主要是通过术后病理及免疫组化。手术是主要的治疗手段,手术治疗能明显延长患者生存期。     

原发性肾输尿管小细胞神经内分泌癌的诊断与治疗(附10例报告)

目的:探讨原发性肾输尿管小细胞神经内分泌癌(NEC)的临床病理特点,提高该类疾病的诊治水平。方法:回顾分析四川大学华西医院2003~2012年诊治的10例原发性肾输尿管小细胞NEC的临床病理资料,总结该类疾病临床特点、免疫组化特点、鉴别诊断要点及临床预后。结果:10例患者病理诊断为完全的NEC7例(完全低分化6例,低分化和中分化混合1例),NEC混合高级别尿路上皮癌3例(肾盂2例,输尿管1例)。2例肿瘤局限在肾脏内,8例肿瘤侵犯肾周和输尿管外脂肪,6例淋巴结转移阳性,1例切缘阳性。免疫组化分析:CD56、嗜铬颗粒素A(CgA)、突触素、神经元烯醇化酶不同程度表达;10例患者上述4个指标中至少1个指标阳性。CD99(6/6),RCC(5/5),WT-1(5/5)等指标均为阴性。PCK和Ki-67指数不同程度表达。1例术后3个月失访;1例术后47个月无复发证据存活至今;1例术后8个月出现肝脏及肺转移;其余7例死亡,平均生存8个月(3~17个月)。结论:原发性肾输尿管NEC临床罕见,侵袭性强,确诊时多数伴有转移,同时合并尿路上皮癌常见。早期局限在肾内的肿瘤,根治性肾切除可取得较好效果;而合并转移的晚期肿瘤,尽管采取手术治疗和术后化疗,预后仍差。寻找包括分子靶向药物在内的新的治疗方法可能是今后治疗的方向。     

肾孤立性纤维瘤1例报告并文献复习

目的:探讨肾孤立性纤维瘤(SFT)的临床病理学特征。方法:对我院收治的1例肾SFT患者的临床资料进行分析,并复习相关文献。结果:本例患者因左侧腰痛1个月入院,术前诊断为左肾肿瘤,行根治性左肾切除术,术后病理诊断为肾SFT,术后随访1年,未见肿瘤复发转移。结论:肾SFT临床罕见,治疗方法以手术治疗为主,最终依靠病理学检查结合免疫组化染色确诊,大部分为良性肿瘤,但也有少部分肾SFT为恶性,术后需长期随访。     

肾细胞癌并发尿路移行细胞癌的诊断与治疗

目的 探讨肾细胞癌并发尿路移行细胞癌的临床特点和诊治方法。方法 回顾性分析5例肾细胞癌并发尿路移行细胞癌患者的临床资料。男4例，女1例。年龄42～75岁，平均62岁。间歇无痛全程肉眼血尿4例，间歇全程肉眼血尿伴右侧腰痛1例。B超、IVU及CT提示肾肿瘤并发尿路肿瘤4例，肾癌不除外合并同侧肾盂占位1例。结果 5例均行根治性手术，4例同时行不同部位肿瘤根治术，1例行分次手术。病理为肾癌并发膀胱癌3例，肾癌并发同侧输尿管癌1例，肾癌并发同侧肾盂癌1例。随访6～18个月，平均11个月。1例术后10个月膀胱肿瘤局部复发，再行经尿道膀胱肿瘤切除术；4例无瘤生存。结论 肾细胞癌并发尿路移行细胞癌临床少见，对肾癌患者行泌尿系超声、IVU和术中肾脏剖开检查有助于正确诊断。根治性手术宜同时切除肾癌侧输尿管，以避免残余输尿管发生肿瘤。     

原发性前列腺滑膜肉瘤的诊断和治疗（附1例报告及文献复习）

目的：探讨前列腺滑膜肉瘤的影像学特点、临床病理和诊治方法。方法：对1例原发性前列腺滑膜肉瘤的临床诊治资料进行回顾性分析。该例患者行超声、CT、MRI检查。手术后病理证实（免疫组化）。结果：该患者行前列腺肿瘤根治性切除术。病理：镜下所见异型性细胞片状分布,免疫组化：CK34（＋）,CD99（＋）,Ki67（＋）,MYOD1（＋／-）,Actin（＋）,CD117（＋）,dog-1（＋／-）,Vimentin（＋）。诊断原发性前列腺滑膜肉瘤随访6个月,患者存活。结论：原发性前列腺滑模肉瘤临床罕见,确诊依赖病理及免疫组化检查。应与梭形细胞肿瘤、恶性神经鞘瘤、横纹肌肉瘤、间质肉瘤鉴别。治疗以手术为主,需根据肿瘤分级分期决定手术方案及术后是否需行辅助治疗。     

睾丸腺瘤样瘤2例并文献复习

目的提高对睾丸腺瘤样瘤的诊疗认识。方法回顾性分析2例经病理证实的睾丸腺瘤样瘤的临床及病理学资料,并结合文献分析和讨论。结果 2例患者入院诊断均为右睾丸肿物。病例1行右睾丸根治性切除术,术后病理结合免疫组化诊断为睾丸腺瘤样瘤,患者术后6个月复查无明显异常。病例2行右睾丸肿块切除术,结合术后病理学特征也诊断为睾丸腺瘤样瘤,术后恢复良好。结论睾丸腺瘤样瘤较少见,临床确诊主要依靠病理及免疫组化检测,预后良好。手术切除是其选择性治疗方法 。     

胃肠道间质瘤的诊治分析(附21例报告)

目的 探讨胃肠道间质瘤（GISTs）的临床表现、病理特点及诊断、治疗和预后。方法 回顾性分析2002年1月至2004年3月期间收治的21例GISTs患者的临床和病理资料。结果 主要临床表现为腹部不适或疼痛、消化道出血、腹部包块和贫血。术前无一例确诊。均接受手术治疗．术中均未见淋巴结肿大转移。肿瘤直径〈5cm者有9例，5～10cm者有9例，〉10cm者3例。常规病理检查见梭形细胞，最终依靠免疫组化染色确诊。良性5例，低度恶性14例，恶性2例；免疫组化检测CD117阳性18例，CD34阳性13例,而CD117阳性和（或）CD34阳性20例。随访3～30个月,平均11．5个月，随访率为100％，1例于术后13个月死亡，1例术后7个月出现肝转移．其余均无瘤生存。结论 GISTs术前确诊非常困难，病理检查时发现非上皮性的梭形细胞肿瘤应作常规免疫组化染色以确诊．手术切除为主要治疗方法。     

肾上皮样血管平滑肌脂肪瘤2例报告并文献复习

目的提高对肾上皮样血管平滑肌脂肪瘤的认识,探讨其诊治方法。方法报告2例经本院收治的肾上皮样血管平滑肌脂肪瘤患者的临床资料,回顾性分析其临床特征及影像学表现。2例患者有1例有腹痛症状,1例无临床症状。术前超声检查均提示混合回声占位,占位可见血流信号。CT检查呈现不均匀强化。术前均诊断为肾脏恶性肿瘤。分析2例患者临床资料并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果 2例患者均接受根治性肾切除手术治疗。术后病理提示肾上皮样血管平滑肌脂肪瘤,肿瘤均侵及脉管且有瘤栓形成。免疫组化染色HMB-45及Melan-A均阳性。2例患者分别获随访3个月及18个月,未见肿瘤复发。结论肾上皮样血管平滑肌脂肪瘤临床少见。其生物学行为可呈现恶性表现。确诊需要依靠病理结合免疫组化。手术是主要的治疗方法,在对肿瘤恶性行为进行判断时应该综合考虑病例临床特征、病理特点以及分子生物学指标。应该对患者进行长期密切随访。     

骨血管源性恶性肿瘤的临床分析

目的 回顾性分析临床上少见的血管源性恶性骨肿瘤的诊断、治疗和临床疗效.方法 从1998年10月至2008年11月我科诊治7例骨血管源性恶性肿瘤患者,包括5例血管内皮瘤和2例血管肉瘤.男4例,女3例;年龄19～76岁,平均42.6岁.1例为多中心病变,6例为单中心.1例病变位于椎体,其余6例位于四肢骨.所有患者均以局部疼痛就诊,病变影像学表现均为溶骨性破坏.其中2例血管肉瘤伴有软组织包块.1例脊柱病变接受边缘切除和术后放疗,5例接受广泛切除重建或截肢术,未行放化疗,另外1例放弃治疗.所有患者均经病理学诊断确诊,平均随访40个月(1～89个月).结果 股骨上段血管肉瘤患者半盆截肢后于术后17个月死于肺和内脏多发转移.脊柱血管内皮瘤患者术后3年死于其他原因.胫骨上端血管内皮瘤患者术后6年出现局部软组织复发,予以再次完整切除.保守治疗的患者带瘤生存,其他3例患者均无瘤生存.结论 骨的血管源性恶性肿瘤临床少见,主要根据病理学检查确诊,同一解剖部位的多中心病灶对诊断有一定特异性.手术广泛切除为治疗四肢病变的主要手段,椎体病变瘤内刮除后可辅以术后放疗.血管肉瘤恶性程度高、预后差.     

膀胱副神经节瘤的诊断和治疗

目的：探讨膀胱副神经节瘤的临床特点及诊治疗效。方法：对5例膀胱副神经节瘤患者的临床资料进行回顾性分析。结果：5例患者,男2例,女3例,平均46（36～77）岁。经尿道膀胱肿瘤电切（TURBT）2例,开放膀胱部分切除术3例。5例患者随访1～7年,1例术后3年肿瘤复发行TURBT,术后病理诊断为移行细胞癌1～2级。1例术后6年复发,伴有左肾重度积水,左肾无功能,行根治性膀胱切除加左肾输尿管切除加右输尿管皮肤造口。3例患者未见肿瘤复发及转移。结论：膀胱副神经节瘤临床少见,其临床表现有一定特点,对于排尿时伴有相应副神经节瘤临床表现的患者应除外膀胱副神经节瘤的可能,治疗方法主要以手术为主。     

原发性肝肉瘤的诊断与治疗

目的探讨原发性肝肉瘤的临床特点及其诊治。方法回顾性分析7例原发性肝肉瘤的临床资料。结果7例均因腹痛就诊而发现肝脏肿瘤。完整切除6例,姑息性切除1例。术前肝动脉栓塞1例,术后化疗1例;因肿瘤复发经历4次肿瘤切除手术1例。存活3例,生存时间最长已达9年;死亡3例,分别于术后2月,2月,22月死亡;失访1例。病理诊断恶性间叶瘤(未分化肉瘤)3例,恶性纤维组织细胞瘤,血管肉瘤,平滑肌肉瘤,脂肪肉瘤各1例。4例行免疫组织化学或组织化学检测确诊。结论原发性肝肉瘤术前诊断非常困难,确诊尚需依赖术后病理或穿刺细胞学检查甚至免疫组织化学检查。采用以手术治疗为主的综合治疗,是延长患者生存期的主要手段。     

Peripheral primitive neuroectodermal tumor of the jugular foramen: case report

OBJECTIVE AND IMPORTANCE: Peripheral primitive neuroectodermal tumor (pPNET) is a rare type of tumor, most commonly found in the limbs of children and young adults. The authors report an extremely rare case of pPNET located at the jugular foramen whose clinical course demonstrated rapid progression. CLINICAL PRESENTATION: A 23-year-old man presented with a 2-month history of progressive hoarseness and dysphagia. The neuroradiological appearance of the lesion was a jugular foramen tumor. INTERVENTION: The patient underwent a partial resection of the tumor through a far lateral suboccipital craniotomy. After surgery, the patient experienced an unexpected deterioration in consciousness. Magnetic resonance images on postoperative Day 18 revealed rapid and large expansion of the residual tumor into the posterior fossa. No adjuvant therapy was administered, and the patient died 6 weeks after diagnosis. CONCLUSION: The pathological diagnosis of the surgical specimen was pPNET, according to the findings of hematoxylin and eosin and immunohistochemical stainings. To the best of our knowledge, this is the first reported case of pPNET at the jugular foramen.     

十二指肠间质瘤16例临床分析

目的 探讨十二指肠间质瘤的诊断和治疗.方法 回顾性分析了16例十二指肠间质瘤治疗的临床资料.结果 十二指肠间质瘤发病部位主要集中在降部和水平部;出血是最常见的临床症状,其次为上腹部疼痛不适,内镜和CT为最常用的辅助检查手段,16例中行胰十二指肠切除2例,十二指肠节段切除9例,肿瘤局部切除5例,术后随访6～42个月,1例复发并出现肝脏转移存活22个月,其余均未发现复发.结论 根据十二指肠间质瘤大小和确切位置应采取不同的手术方式,大部分患者可通过十二指肠节段切除或局部切除达到根治目的,对于病理分级呈高度危险患者术后应给予伊马替尼辅助治疗.     

Solitary Fibrous Tumor of the Pleura: An Analysis of 13 Cases

BACKGROUND: Solitary fibrous tumor of the pleura is a rare soft-tissue tumor. In search of appropriate diagnosis and treatment methods, we present our experience with 13 patients. METHODS: The medical charts of 13 patients treated during the last 5 years were reviewed, as well as pathological records, including immunohistochemical stains. Follow-up data were obtained. In addition, a literature review with regard to treatment and clinical outcome was performed. RESULTS: Our series consisted of four men and nine women with a mean age of 47 years. Two were diagnosed before operation with ultrasonography-guided core needle biopsy. All patients underwent primary surgical treatment, and four of them were resected by video-assisted thoracic surgery. Seven tumors were malignant and the other six were benign. Immunohistochemical staining showed nestin was positive in three malignant solitary fibrous tumors of pleura (3/7), which were negative for CD34. Except for one, all patients were followed-up for 3 to 35 (mean, 14.5) months. Among them, one patient experienced a recurrence and one patient died of brain metastasis. CONCLUSION: Ultrasonography-guided core needle biopsy combined with immunohistochemical analysis might be a safe and rapid method to provide a confirmatory diagnosis before resection. For smaller, pedunculated tumors, video-assisted thoracic surgery could be a powerful and useful approach. We speculate that CD34-negative and nestin-positive might be a malignant marker for solitary fibrous tumor of pleura.     

腹膜后局限性Castleman病误诊嗜铬细胞瘤4例并文献复习

目的探讨腹膜后局限性Castleman病的临床特征及外科治疗方法,结合复习相关文献,以提高腹膜后Castleman病的诊治水平。方法回顾性分析4例术前诊断嗜铬细胞瘤、经手术病理证实为腹膜后Castleman病患者的临床、病理、影像学特点,评价手术治疗效果。结果 4例患者均行手术治疗,切除腹膜后肿瘤,术后病理均为Castleman病。随访12～114个月,均未见肿瘤复发。结论腹膜后Castleman病临床症状不典型,实验室检查通常无异常结果,容易误诊,确诊需靠病理检查,手术切除疗效好。     

Primary Pulmonary Artery Sarcoma: Surgical Management and Differential Diagnosis with Pulmonary Embolism and Pulmonary Valve Stenosis

Abstract   Background and aim of the study: Primary pulmonary artery sarcomas are rare and lethal tumors that are usually diagnosed during surgery or autopsy. We present six cases of primary pulmonary artery sarcomas and discuss clinical features, differential diagnosis, surgical treatment, and outcome of the tumors. Methods: Between January 1994 and July 2008, six patients were identified with the disease during operation. Three patients were initially diagnosed with pulmonary valve stenosis, and two patients had a presumptive diagnosis of chronic pulmonary embolism. Two patients had simple or partial tumor resection. Four patients had radical tumor resection and homograft reconstruction of the pulmonary arteries. Results: Histological examinations showed five malignant mesenchymomas and one fibrosarcoma. One patient died of refractory pulmonary hypertension during operation. Two patients died 4 months postoperatively because of brain metastases. Two patients were alive for 3 and 9 months, respectively after the operation with recurrent tumor. One patient is alive even 2 years after resection with no signs of recurrence or metastasis. Conclusions: Because of similar clinical features, pulmonary artery sarcomas are often confused with other pulmonary vascular obstructive diseases. Computed tomography scanning and gadolinium-enhanced magnetic resonance imaging could be useful methods for differential diagnosis. The prognosis is very poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis. Early diagnosis and radical surgical resection presents the only opportunity for a potential cure.