von Hippel-Lindau综合征外科治疗

目的 探讨von Hippel-Lindau(VHL)综合征的外科治疗方法.方法 VHL综合征患者4例.例1,男,56岁.主诉乏力、心悸2 d.空腹血糖2.37 mmol/L.CT检查示左肾上、下极3个肿块,直径分别为8.0、7.0、4.0 cm.10年前行脑血管母细胞瘤切除术.例2,女,57岁.主诉左腰痛不适1个月.CT检查示左肾上腺、左肾、胰体肿物,直径分别为2.7、4.5、2.1 cm.例3,女,39岁.查体发现左肾上腺占位1个月.CT检查示左肾上腺3.0 cm×4.0 cm实性占位,增强后肿块明显强化.既往有小脑、脊髓血管母细胞瘤及双侧肾癌手术史.例4,女,41岁.B超发现双肾肿瘤1个月入院.CT检查示左肾、左肾上腺、右肾、胰腺肿物,直径分别为4.0、3.0、1.5、2.0 cm.1个月前行y刀治疗多发脑部肿瘤.结果 4例均手术治疗.例1行左肾根治性切除术,病理报告肾血管周细胞瘤,随访6个月右肾未见异常.例2行左肾、左肾上腺、胰体尾及脾切除,病理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺切除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘除术,避免肾上腺全切术.腹部多器官肿瘤一期手术安全可行. 理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺 除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘除术,避免肾上腺全切术.腹部多器官肿瘤一期手术安全可行. 理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿,随访3个月MRI检查发现脊髓肿瘤.例3行左肾上腺肿瘤摘除术,病理报告嗜铬细胞瘤,随访2年未见肿瘤复发.例4行左肾、左肾上腺 除,病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤,3周后剜除右肾肿瘤,病理报告透明细胞癌,随访1年肿瘤未复发.结论 VHL可并发多器官肿瘤,较大中枢神经系统肿瘤可手术切除,多发较小的实性肿瘤可放疗.直径<3cm肾癌可密切随访、择期处理;直径>3cm肾癌首选保留肾单位手术.肾上腺肿瘤首选腺瘤摘     

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。     

肾肌周细胞瘤1例报告并文献复习

目的:探讨肾肌周细胞瘤的临床病理特点及诊疗方法。方法:回顾性分析1例罕见肾肌周细胞瘤的临床表现、影像学及术后病理学资料,并结合相关文献,总结其临床表现、病理特征及诊疗方法。结果:肾肌周细胞瘤无典型临床表现,彩超和CT检查缺乏特异性。患者行后腹腔镜左肾部分切除术,病理检查报告为肌周细胞瘤。免疫组织化学检查显示SMA+,Vim+,CD34+。术后随访25个月未见肿瘤复发和转移。结论:肾肌周细胞瘤为极其罕见的良性肿瘤,不易复发和转移;诊断主要依靠病理检查;手术切除肿瘤是目前唯一有效的治疗方法。     

肾癌附睾转移一例报告

患者，男，50岁。左肾癌根治术后2年，发现左阴囊内肿块于2005年4月入院。患者3年前因左腰部酸痛伴全程肉眼血尿入院，B超示左肾实质性肿瘤，腹主动脉旁未见淋巴结肿大。CT示左肾巨大占位，直径约10cm。行左肾肿瘤根治性切除术。术后病理示：肾透明细胞癌，累及肾盂，未侵犯肾包膜，肾门处血管未见癌浸润，肾门及腹主动脉旁淋巴结未见转移，肾盂未见癌浸润。S-100蛋白、p21单克隆抗体、癌胚抗原（＋），增殖细胞核抗原60％（＋），p53单克隆抗体、波形蛋白（-）。术后未进行其他辅助治疗。     

VHL综合征双肾切除术后维持性血液透析并发上消化道出血一例

正患者,男,48岁,因"双肾切除后维持性血液透析一年,颅内多发血管母细胞瘤伽马刀术后3天"于2014年3月13日入院。患者先后于1997年行视网膜血管母细胞瘤切除术,2000年行脑血管母细胞瘤切除术,2008年因右肾肿瘤行右肾切除术,2011年发现左肾肿瘤,行保留肾单位左肾肿瘤切除术。2013年4月复查CT示:左肾占位,肾癌可能,左肾多发囊肿,胰腺多发囊肿。在外院诊断为"VHL综合征",     

肾盂原发性恶性纤维组织细胞瘤1例报告并文献复习

目的：探讨肾盂原发性恶性纤维组织细胞瘤（Malignant fibrous histiocytoma,MFH）的临床特点和诊治方法。方法：对1例MFH患者行根治性肾切除＋肾静脉癌栓切除＋周围淋巴结清扫术。结果：术后病理报道左肾盂恶性纤维组织细胞瘤伴肾静脉癌栓。结论：术后应终身定期随访．如复发则相应积极治疗,从而能使部分患者有机会长期存活。     

肾部分切除术后肾动脉假性动脉瘤一例并文献复习

目的 探讨肾部分切除术后少见但危重的肾动脉假性动脉瘤(RAP)的临床表现、诊断与处理方法。方法 报告2019年8月我科诊疗的1例腹腔镜左肾部分切除术后出现RAP患者的临床资料,并复习相关文献对RAP的临床特征、诊断与治疗进行分析总结。结果 患者男,77岁,因“发现左肾占位1个月”入院,术前诊断为“左肾占位:肾癌可能性大”。行腹腔镜下左肾部分切除术,术后病理结果示左肾透明细胞癌,包膜完整。术后第8天出现无痛性肉眼血尿,超声造影提示左肾RAP,介入下肾动脉造影再次确诊并同时行超选择性肾动脉栓塞,成功栓塞动脉后患者无再发血尿,顺利出院。结论 RAP是肾部分切除术后少见但严重的并发症,可疑患者应尽早行超声造影检查,超选择性肾动脉栓塞治疗安全有效。     

转移性肝血管外皮瘤一例报道

病人:女,70岁。因体检发现肝占位性病变5d就诊,体检未见异常。AFP、CA1 9 9、CEA正常。B型超声示:肝右前叶下段4 9.4mm×37.9mm低回声团块,边界可见,内回声不均,中心见液性区。CT示:肝脏平扫右前叶4 .7cm×3.8cm内分隔低密度影,边缘清晰,增强动脉期明显强化,呈粗分隔状,门脉期密度稍减为略高密度影,延迟期分隔呈等密度。诊断:肝右前叶占位,血管源性肿瘤可能。病人于1 979年行下肢血管外皮瘤切除术,病理示:良性血管外皮瘤。1 996年行左肺肿瘤切除术,病理示:转移性低度恶性血管外皮瘤。本次行右肝肿瘤切除术(V段) ,术中见:肝右前叶下段一…     

儿童巨大肾血管平滑肌脂肪瘤1例报告及文献复习

目的探讨儿童肾脏血管平滑肌脂肪瘤(angiomyolipoma,AML)的临床特征、诊断及治疗方法。方法通过回顾分析1例术前误诊为肾母细胞瘤的儿童巨大肾脏AML的临床、影像学及病理资料,结合相关文献复习,讨论其临床生物学行为、诊断及治疗方法。结果 CT及MRI检查提示左肾肾母细胞瘤,由于肿瘤体积巨大,行剖腹探查及左肾切除术,术后病理诊断为肾AML,随访6个月未见复发。结论对于小体积及儿童肾AML,仅仅依靠CT影像资料做诊断可能存在一定困难,体积巨大而肿瘤脂肪成分低的患者容易被误诊,大体积的儿童肾AML可以行手术治疗以避免不必要的化疗。     

巨大肾孤立性纤维性肿瘤1例报告

孤立性纤维性肿瘤是一种罕见的NAB2-STAT6融合基因相关的间质源性肿瘤, 发生于肾脏者极罕见。本文报道1例16岁男性患者, 因左侧腰背部疼痛3年余入院, 腹部CT/MRI检查可见左肾巨大占位性病变, 行腹腔镜根治性左肾切除术, 因肿瘤巨大, 术中中转开放手术。术后病理诊断为肾孤立性纤维性肿瘤。术后随访2个月, 患者症状消失, 未见肿瘤复发。     

Bilateral metastatic renal hemangiopericytoma ten years after primary intracranial lesion

We report a case of bilateral metastatic renal hemangiopericytoma. A 37-year-old Caucasian male presented in 1993 with intracranial hemangiopericytoma. Subsequent metastatic disease noted years later include bilateral renal hemangiopericytoma 10 years after initial presentation. To our knowledge, this is only the second reported case of bilateral metastatic renal hemangiopericytoma.     

Multiple extracranial metastases from a meningeal hemangiopericytoma and severe hypoglycemia: a case report

Extracranial multiple metastases from meningeal hemangiopericytoma have been reported only rarely. The authors describe the case of a 61-year-old woman, who was previously diagnosed as having primary meningeal hemangiopericytoma with its multiple metastases to the liver, lumbar spine, etc. and who suffered from a sudden attack of hypoglycemia. Considering the history of her present illness, this hypoglycemic shock was most likely brought on by the remarkable metastatic tumors to the liver. Recent literature pertinent to hypoglycemia suggests that insulin-like growth factor-II (IGF-II) produced by tumor is strongly suspected to be involved in the development of hypoglycemia.     

Metastatic hemangiopericytoma to the cauda equina: a case report.

BACKGROUND CONTEXT: Hemangiopericytoma is an aggressive tumor associated with high recurrence and metastasis. Metastases are usually delayed, long after diagnosis of the primary lesion. Metastatic hemangiopericytoma to the spinal cord is especially rare. PURPOSE: To report a rare clinical presentation of a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina from a cerebellar hemangiopericytoma. STUDY DESIGN: Case report with a review of the literature. METHODS: Clinical history, physical findings, and magnetic resonance imaging studies of a patient with a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina are reported. RESULTS: A case report is presented of a female with an intradural, intramedullary lesion at the L4-S1 level, presenting initially with progressive pain and motor weakness affecting the left lower extremity. She had a history of a cerebellar hemangiopericytoma, which had been treated with total resection and radiotherapy 4 years earlier. This patient developed urinary urgency and frequency. Pathological analysis revealed a hemangiopericytoma, which had a similar character to a cerebellar lesion. After radiotherapy, the tumor was mostly diminished and her symptoms totally resolved. CONCLUSIONS: Hemangiopericytomas have a strong tendency to both local recurrence and metastasis. Common metastatic sites are the skeletal system, lung, liver, and abdominal cavity. To the authors' knowledge, there have been no reports of spinal intradural, intramedullary metastasis of hemangiopericytoma.     

Intramedullary hemangiopericytoma of the spinal cord; case report and review of the literature

Hemangiopericytoma is a rare tumor deriving from capillaries and pericytes. Although it is a well established entity clinically and histologically, its occurrence in the central nervous system adhering to the meninges has been controversial. We described a case of a 39-year-old man with intramedullary hemangiopericytoma. To the best of our knowledge, no previous case of hemangiopericytoma in this location has been reported. We reviewed differential diagnosis of spinal hemangiopericytoma based on neuroradiological points, and clinical findings of the 14 primary cases reported in the literature. The nosological problem of hemangiopericytoma was also discussed.     

Hemangiopericytoma of the kidney: a case with preoperative ethanol embolization

We report a case of a giant renal hemangiopericytoma that was embolized preoperatively with ethanol. Ultrasound and computerized tomography showed multiple smooth-walled cysts within the tumor. The tumor itself was hypervascular and a vascular pattern specific for hemangiopericytoma was noted upon reinterpretation of the angiograms. The diagnostic and therapeutic aspects are discussed, and the literature is reviewed.     

Renal hemangiopericytoma discovered at a health screening: a case report

We report a case of renal hemangiopericytoma which was incidentally discovered by ultrasonography at a health screening. A 58-year-old man was admitted to our hospital for close examination of the renal tumor. Computed tomography revealed the left renal tumor, 60 x 50 mm in size, which was well enhanced with contrast medium. Magnetic resonance imaging revealed an isointensity mass (T1-weighted) and high-intensity mass (T2-weighted) at the left kidney. Radical nephrectomy was performed on suspicion of left renal cell carcinoma. Histopathological examination revealed renal hemangiopericytoma. The present case is the 7th in the Japanese literature.     

Hemangiopericytoma of the Greater Omentum

A 41-year-old Chinese woman was admitted to our hospital with epigastric pain. Computed tomography detected a heterogeneous enhancement tumor fed by the left gastroepiploic artery in the left lower quadrant and cholelithiasis. Excision of the tumor in the greater omentum and cholecystectomy were performed laparoscopically. Histological findings confirmed a diagnosis of hemangiopericytoma with low-grade malignancy. To our knowledge, hemangiopericytoma of the greater omentum is very rare, and only 12 cases were reported in English literature. We report a case of hemangiopericytoma arising in the greater omentum and review the literature.     

Case report of a newborn with a posterior thoracic midline congenital hemangiopericytoma of the back

The posterior thoracic midline location is an unusual site for a congenital hemangiopericytoma. The authors report such a case that caused near fatal exsanguination of a newborn after vaginal delivery. Magnetic resonance imaging (MRI) studies of the mass were completed after hemostasis. These studies showed a well-defined border between the tumor and underlying trapezius muscle. The mass was removed successfully surgically and presumed initially to be a teratoma. Pathological diagnosis of the tumor was hemangiopericytoma with low malignant potential. After a 9-day hospital course, the patient was discharged with recovering hepatic and renal function.     

Intracranial hemangiopericytoma with extracranial metastasis occurring after 22 years

A 65-year-old man developed pancreatic and pubic tumors 22 years after craniotomy for a left sphenoid ridge tumor. The histological finding of the primary sphenoid ridge tumor was meningotheliomatous meningioma. The histological appearance of the biopsy specimen of the pubic tumor was hemangiopericytoma. The recurrent intracranial tumor was treated by radiosurgery, and the metastatic tumors were treated by conventional irradiation. Intracranial tumors rarely metastasize outside the central nervous system, except for meningeal hemangiopericytomas. This case indicates that meningeal hemangiopericytoma may metastasize many years after the initial onset and requires long-term follow up.     

Two cases of metastatic renal tumor

Two cases of metastatic renal tumor are reported, one in a 78-year-old male who had undergone total gastrectomy for gastric carcinoma, and the other in a 45-year-old female who had undergone hysterectomy for cervical carcinoma of the uterus. The chief complaint was flank pain and nephrectomy was performed in both cases. Histopathological examination of the removed specimen revealed metastatic renal tumor the primary site of which was probably the stomach in the first case and cervix uteri in the second case. The literature on metastatic renal tumors in Japan is reviewed.