肾上腺节细胞神经瘤9例报告

目的：提高肾上腺节细胞神经瘤的诊治水平。方法：回顾性分析我院近10年来经病理检查证实的9例肾上腺节细胞神经瘤的临床资料，对其病理学特征、临床表现、诊断及治疗进行讨论。结果：9例均手术切除肿瘤，术后病理检查证实为肾上腺节细胞神经瘤，术后恢复顺利。2例血醛固酮增高者及1例血去甲肾上腺素增高者，术后1个月复查均恢复正常；4例有腰腹部疼痛等症状者，术后症状消失。随访14个月－6年，肿瘤无复发。结论：肾上腺节细胞神经瘤是一种罕见的良性肿瘤，B超和CT是诊断肾上腺节细胞神经瘤的主要方法，确诊有赖于病理检查；手术切除肿瘤是有效的治疗手段。     

胸部原始神经外胚层肿瘤(附10例报告)

目的 提高对胸部原始神经外胚层肿瘤（PNET）的认识，改进治疗效果。方法 回顾性分析1999年至2004年手术切除并经病理证实的10例胸部PNET治疗结果。结果 肿瘤位于胸腔内6例、胸壁2例，心包内和后纵隔各1例。胸内肿瘤完全摘除2例，大部分切除2例，肿瘤并肺叶切除2例。2例胸壁肿瘤合并受累肋骨切除胸壁重建，1例行肿瘤及心包部分切除，1例后纵隔哑铃形肿瘤由胸外科和神经外科同期切除。全组无手术死亡或住院死亡。病理标本免疫组化检查，10例CD99均为阳性，LEA均为阴性。术后4例接受化疗，4例放化疗，2例未行任何辅助治疗。术后随访6年，死亡7例，生存最长27个月，最短9个月，平均17个月。3例生存已超过12个月者仍在随访中。结论 胸部PNET恶性程度高，进展快，术前诊断率低。诊断与鉴别诊断需要病理组织学和免疫组化确定。彻底摘除肿瘤及受累组织是治疗的重要环节，术后放化疗有助延长生存期。提高生存率还需要寻找更有效的方法。     

无功能性肾上腺肿瘤的诊断与治疗(附39例报告)

目的：提高无功能性肾上腺肿瘤的诊治水平。方法：回顾性分析我院17年来39例无功能性肾上腺肿瘤患者的临床资料。结果：39例中,38例行肿瘤切除或剜除术,1例仅行活组织检查;30例病理检查证实为良性无功能性肾上腺肿瘤,其中节细胞神经瘤7例,平滑肌瘤1例,皮质腺瘤10例,髓脂瘤3例,神经鞘瘤1例．肾上腺囊肿8例。随访6个月～7年,临床症状消失,无肿瘤复发。9例病理检查证实为恶性无功能性肾上腺肿瘤,其中脂肪肉瘤1例,皮质腺癌4例,转移癌4例,术后2年内,8例死亡,1例无癌生存14个月。结论：无功能性肾上腺肿瘤临床少见,早期诊断困难,CT是首选检查方法,确诊依赖于病理检查。手术切除是良性肿瘤的有效手段,但对恶性肿瘤预后较差。     

儿童阴茎原始神经外胚层肿瘤/尤文氏瘤1例并文献复习

目的:探讨原发于阴茎的原始神经外胚层肿瘤/尤文氏瘤(PNET/Ewing's sarcoma)的临床表现、病理特点、治疗方法。方法:分析本院收治的1例5岁患儿原发于阴茎的PNET/EWS患者的病例资料进行文献复习。结果:病理表现为小圆细胞恶性肿瘤,免疫组化结果显示CD99(+),分子生物学检查:EWS荧光原位杂交可见易位,符合原始神经外胚层肿瘤/尤文氏肉瘤诊断,该患者经过病理确诊为阴茎PNET/EWS,经45周化疗和局部放疗,瘤灶缩小。目前停药3个月。结论:原发阴茎PNET/EWS极罕见,临床症状无特异性,以阴茎增大伴疼痛为主,免疫组化及分子生物学检查有助于对PNET/EWS的确诊。     

肾上腺原始神经外胚叶肿瘤1例报告并文献复习

目的探讨肾上腺原始神经外胚叶肿瘤的临床表现、病理特点、治疗方法及预后。方法分析1例右侧肾上腺原始神经外胚叶肿瘤患者的病例资料并复习相关文献。结果手术根治切除肾上腺肿瘤,病理诊断为肾上腺原始神经外胚叶肿瘤,术后采取辅助大剂量放疗,半年后右侧腹壁肿瘤转移,再次手术切除转移灶,术后2月复查B超见右肾上腺区肿瘤复发,患者1年后死亡。结论肾上腺原始神经外胚叶肿瘤临床罕见,病理及免疫组化是原始神经外胚叶肿瘤确诊的金标准。手术是早期患者首选的治疗方法,晚期患者可以辅助联合化疗,对预后尚没有评估依据。     

肾上腺神经内分泌癌的临床特点及诊断治疗

目的总结肾上腺神经内分泌癌的临床表现、诊治和预后特点。方法回顾性分析2011-10—2017-09间15例肾上腺神经内分泌癌患者的临床资料。结果本组15例患者均有明确的病理或活检结果。7例行腹腔镜下肾上腺根治性切除术。8例患者术前已发生不同程度的转移,其中3例行化疗,2例行介入栓塞治疗,1例行放疗,2例保守治疗。免疫组化检查示突触素(Syn)、神经元特异性烯醇化酶(NSE)、嗜铬粒素A(Cg A)、CD56和EMA的阳性表达比例分别为80%(12/15)、73.3%(11/15)、53.3%(8/15)、73.3%(11/15)、46.7%(7/15)。12例获随访1~50个月,中位随访时间11.5个月。2例肾上腺根治术后48个月、50个月无复发、转移,仍存活。1例根治术后11个月出现肝转移,行放疗,至今存活15个月。1例确诊时出现肝脏多发转移,行肾上腺介入栓塞治疗及肝转移射频消融治疗,目前存活19个月。其余8例均死亡,死亡患者的平均生存期为8.6个月。结论肾上腺神经内分泌癌是一种恶性程度很高的肿瘤,预后极差。病理为诊断的金标准,早期治疗首选肾上腺根治性切除术,晚期行放化疗及介入治疗,有助于延长患者的生存期。     

肾上腺节细胞神经瘤的临床诊治特点（附20例报告）

目的 总结肾上腺节细胞神经瘤的临床诊治特点.方法 对20例肾上腺节细胞神经瘤患者分别行后腹腔镜下肿瘤摘除术（18例）及行开放手术（2例）.结果 切除肿瘤大小3.6cm×2.7cm×1.8cm～9.2cm×5.5cm×4.2cm.所有患者术中血压均无明显波动,术后无并发症发生,恢复较好.其中12例随访1～36个月肿瘤无复发和转移,8例失访.结论 肾上腺节细胞神经瘤是一种较少见的良性肿瘤,确诊主要依靠病理学检查,主要治疗方法为外科手术,包括腹腔镜或开放手术,一般预后较好.     

肾上腺节细胞神经瘤的诊治体会（附6例报告）

目的提高肾上腺节细胞神经瘤的诊治水平。方法回顾性分析经病理证实的6例肾上腺节细胞神经瘤患者的临床资料。站果6例术后随访5个月至7年，均未见肿瘤局部复发或远处转移。结论B超、CT检查在肾上腺节细胞神经瘤的诊断中具有重要价值，确诊需要病理学检查。治疗采取肿瘤切除术，患者预后良好。     

肾上腺节细胞神经瘤2例报告并文献复习

目的:总结肾上腺节细胞神经瘤的诊治体会。方法:回顾性分析我院2010年1月~2013年12月收治的2例肾上腺节细胞神经瘤患者的临床资料:2例患者均行内分泌实验室检查、B超、MRI及18氟-脱氧葡萄糖(18F-FDG)断层和CT断层显像检查,并行后腹腔镜手术切除肿瘤。结果:2例患者术前均未明确诊断为肾上腺节细胞神经瘤,经后腹腔镜手术完整切除肿瘤。患者术中血压平稳,无波动。术后病理检查肿瘤包膜均完整,质地均匀、质韧,切面呈灰白色、鱼肉状改变,报告为肾上腺节细胞神经瘤。患者术后未见并发症发生,恢复较好。随访1年,无复发和转移。结论:肾上腺节细胞神经瘤的临床症状、体征并无特异性,术前实验室检查和影像学检查并不能对其确诊,但可以鉴别相关功能性肾上腺肿瘤,最终确诊需依靠病理学检查。     

肾上腺组合性嗜铬细胞瘤四例报告及文献复习

目的 探讨肾上腺组合性嗜铬细胞瘤(嗜铬细胞瘤-神经节瘤)的临床特点及治疗方法.方法病理确诊的肾上腺组合性嗜铬细胞瘤患者4例,均为男性,年龄37～62岁.临床表现:高血压3例,其中伴有阵发性心悸1例;血压正常1例.B超、CT检查提示肿瘤均为单发,直径分别2.5、3.8、6.3、15.0 cm.行131Ⅰ-间位碘代苄胍显像检查2例,均为阳性;99Tcm-奥曲肽显像检查2例,阳性1例.24 h尿儿茶酚胺检查,去甲肾上腺素(196.1±92.2)nmol/24 h,肾上腺素(26.6±8.9)nmol/24 h,多巴胺(1957.9±913.5)nmol/24 h,其中轻度升高2例,正常2例.术前α受体阻滞剂准备2～4周.结果 3例行后腹腔镜下肾上腺肿瘤切除手术,1例行开放手术切除.病理报告:肾上腺组合性嗜铬细胞瘤.4例随访15～38个月,平均28个月,肿瘤未见复发、转移;3例高血压患者中2例血压正常.结论 肾上腺组合性嗜铬细胞瘤临床罕见,临床表现与嗜铬细胞瘤相似,儿茶酚胺稍升高,无恶性倾向,手术效果良好.     

脊柱原始神经外胚叶肿瘤的诊断与治疗

目的 总结脊柱原始神经外胚叶肿瘤(primitive neuroectodermal tumor,PNET)的诊断和治疗经验.方法 回顾性分析1999年至2009年收治的PNET患者共13例,男8例,女5例.年龄4～43岁,平均(26.9±11.1)岁.肿瘤累及颈椎6例,腰椎5例,胸椎1例,骶椎1例.9例为手术后依据病...     

Intramedullary primitive neuroectodermal tumor presenting with rapidly-progressive cauda equina syndrome

A 39-year-old male presented with gait disturbances with rapid deterioration for 2 weeks. Neurological examination found paraparesis, sensory loss in the L1-S5 dermatomes, and vesicorectal dysfunction. Magnetic resonance (MR) imaging revealed a fusiform intramedullary tumor at T12-L1 levels with heterogeneous enhancement. The patient underwent microsurgical tumor resection. A myelotomy exposed a highly vascular tumor that was subtotally resected. Histological examination demonstrated hypercellular tumor accompanied by significant cell atypism and mitotic figures. Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100 protein, synaptophysin, and INI-1, consistent with primitive neuroectodermal tumor (PNET). Postoperatively, the patient underwent irradiation to the whole craniospinal axis. He experienced local recurrence 7 months after surgery. MR imaging performed at 10 months revealed holocord progression and intracranial dissemination. The patient died 13 months after the onset of the disease. PNET should be considered in the differential diagnosis of an intramedullary spinal cord tumor.     

腹膜后原始神经外胚层瘤／尤因肉瘤一例报告并文献复习

目的总结腹膜后原始神经外胚层瘤／尤因肉瘤（PNET／EWS）的临床及组织病理特点,探讨其诊疗方法及预后情况。方法分析1例罕见的腹膜后PNET／EWS的临床特征、组织病理学特点以及诊断、治疗方法,同时复习近年来的国内外相关文献。结果患者当地医院行左肾切除＋左腹膜后肿物部分切除,术后病理示PNET,并在当地医院行化疗。手术后4个月肿瘤复发,再次行手术切除并行化疗,1年后检查发现肿瘤再复发。结论腹膜后PNET／EWS是一种小圆形细胞组成的恶性肿瘤。诊断时应与其他腹腔和腹膜后软组织肿瘤相鉴别。治疗以手术切除为主,化疗效果不肯定。本病有侵袭性的临床过程,远处转移早,经常局部复发和区域淋巴结转移,肺、肝、骨和骨髓转移,预后较差。     

泌尿系统原发性原始神经外胚层瘤临床病理分析

目的 探讨泌尿系统原发性原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)的临床病理特征、免疫学表型、治疗方法及预后.方法 回顾性分析3例泌尿系统原发性PNET患者资料.3例均为男性,年龄分别为29、32和75岁.2例原发于肾脏,1例原发于膀胱.2例肾肿瘤大小分别为7.7 cm×6.2 cm和12.6 cm×9.4 cm,影像学检查提示肿瘤边界尚清,内部回声欠均匀.膀胱肿瘤大小为10.0 cm×10.0 cm,影像学检查提示膀胱壁不规则增厚,其内密度不均匀.2例肾肿瘤行肿瘤根治术,膀胱肿瘤行血块取出术及肿瘤活检术.结果 光镜下,瘤细胞为形态一致的小圆形或卵圆形,被纤维结缔组织分隔成实性片状或巢状,并形成假菊形团或Homer-Wright菊形团,核分裂象多见.免疫组化标记:3例肿瘤CD99、突触素和波形蛋白均为阳性.1例肾肿瘤Ki67阳性率＜5%,另1例80%阳性.3例病理诊断均为PNET.例1肾肿瘤患者未行化疗,于术后14个月复发死亡;例2肾肿瘤及例3膀胱肿瘤患者术后予以化疗,分别于术后4、6个月死亡.结论泌尿系统原发性PNET是一种少见的高度恶性软组织肿瘤,诊断主要依据病理形态学特征及免疫组化标记.目前治疗方法主要是手术加放、化疗.

Abstract：

Objective To explore the clinico-pathological features, immunophenotype, treatment and prognosis of urologic primary primitive neuroectodermal tumor (PNET). Methods The clinical data of 3 patients with urologic PNET were analyzed retrospectively. All patients were male, aged 29, 32 and 75 years respectively. Two of the lesions were located in the kidney, and the third was located in the bladder. The sizes of renal tumors were 7.7 cm×6.2 cm and 12.6 cm×9.4 cm respectively. Imaging examinations revealed a well-defined mass with inhomogeneous echo inside. The size of bladder tumor was 10.0 cm×10.0 cm. CT scan demonstrated irregular thickening of the bladder wall, and the density of the wall was inhomogeneous. In the 2 cases of renal PNET radical surgery was performed, while an emergency palliative surgery to remove a blood clot and biopsy were performed in the bladder PNET case. Results In light microscope, the tumors were characterized by uniform small round or oval cells and nest-like or dense sheet structures surrounded by sparse fibrovascular stroma. Homer-Wright rosettes or pseudorosettes were observed, as well as mitoses. Immunohistochemical study revealed that all cases showed positive staining for CD99, synaptophysin and vimentin. One of the renal tumor cells showed positive for CD56, and the other renal tumor and urocystic tumor cells were focally positive for chromogranin A. Additionally, in 1 of the cases of renal tumor there was a high positive rate of 80% for Ki67 staining while the other case showed less than 5%. All 3 cases were eventually diagnosed as PNET. The first renal tumor case was not treated with radiotherapy and chemotherapy postoperatively, and the patient died of recurrence 14 months after surgery. Both the second renal tumor case and the bladder tumor case underwent chemotherapy postoperatively, and they died 4 and 6 months after surgery respectively. Conclusions The urologic primary PNET is a very rare, highly malignant soft tissue tumor, and the diagnosis must be based on pathologic findings and immunohistochemical phenotypes. The multimodal treatment for urologic primary PNET consists of surgery, chemotherapy and radiotherapy.     

Case of primitive neuroectodermal tumor of the kidney

A 28-year-old woman presented with right flank pain. A large, firm, fixed mass was palpable in the right side of the abdomen. Computed tomography revealed a solid mass of the right kidney with extension into the renal vein and inferior vena cava. The patient underwent right radical nephrectomy with en bloc resection of the inferior vena cava containing tumor thrombus and right adrenalectomy. Histologically the tumor consisted of small tumor cells with rosette formation. Immunohistochemical staining was positive for CD99 and NSE. Analysis with polymerase chain reaction (PCR) demonstrated the EWS/FLI1 fusion products resulting from a chromosomal translocation. These findings were consistent with primary renal primitive neuroectodermal tumor (PNET). Two months after surgery, multiple lung, liver and lymph node metastases were found. The patient received 2 cycles of chemotherapy with cisplatin, ifosfamide, etoposide, resulting in a partial remission. She subsequently received 1 cycle chemotherapy with paclitaxel and carboplatin, resulting in no response. The metastatic lung and liver diseases progressed and she died 5 months after diagnosis.     

骨与软组织原始神经外胚层瘤的病理诊断与临床治疗

目的探讨原始神经外胚层瘤（ＰＮＥＴ）的病理学特点及其临床意义。方法报告本科１９８９年４月～１９９６年８月收治５例ＰＮＥＴ患者的经验。肿瘤发生部位为髂骨２例、腋窝软组织１例、股骨髁１例、颅骨１例。Ｘ线片显示骨组织呈溶骨性破坏,无骨膜反应及钙化征象。５例患者病理标本除常规石蜡切片和ＨＥ染色,均作了ＡＢＣ法免疫组化,其中２例还作了透射电镜观察。以光镜下具有Ｈｏｍｅｒ－Ｗｒｉｇｈｔ（Ｈ－Ｗ）菊形团的排列;免疫组化检查Ｏ１３阳性并至少含２种以上不同神经标记的表达,如ＮＳＥ、ＳＹ３８、ＣｇＡ等,或经电镜检查细胞内含有神经内分泌颗粒为ＰＮＥＴ诊断标准。结果５例均行术后化疗,３例同时行放疗。３例因肺转移分别于发病后２０、１３、５个月死亡;２例带瘤生存。结论（１）Ｏ１３阳性,光镜下Ｈ－Ｗ菊形团,免疫组化检查至少含２种以上不同神经标记的表达,如ＮＳＥ、ＳＹ３８、ＣｇＡ等,对于与其它骨和软组织的小圆细胞类肿瘤的鉴别具有一定意义;（２）临床上应注意ＰＮＥＴ与Ｅｗｉｎｇ瘤的区分和鉴别;（３）肿瘤远处转移是导致ＰＮＥＴ治疗失败的主要原因,更有效的治疗方法仍有待于探讨。     

Peripheral primitive neuroectodermal tumor -- PNET

The authors describe three cases of peripheral primitive neuroectodermal tumor. The tumor was found in soft tissues of the crus, shoulder girdle and perineum, and was also located paravertebrally and epidurally at the level of L1-L2 vertebrae. Radiological findings were not specific for this disease. The results of imaging methods (sonography, CT, MRI, DSA) were important for the assessment of tumor size, its boundary and invasion of the surrounding tissues, and for the evaluation of tumor response to therapy and detection of recurrent disease. The PNET diagnosis was based on immunohistochemical, biochemical and cytogenetic examinations. One patient died 5 months after the first clinical signs were manifested; the two patients surviving for 2 and 1 3/4 years after first sign manifestation, respectively, remained in the care of cancer specialists. Key words: skeletal Ewing's sarcoma, extra-skeletal Ewing's sarcoma, Ewing's sarcoma family of tumors, peripheral primitive neuroectodermal tumor.     

Sternal resection and chest wall reconstruction for primitive neuroectodermal tumor of the sternum

Primitive neuroectodermal tumor of the sternum is rare. A 59-year-old woman referred to our department with anterior chest pain and a tumor in the sternum. The patient was diagnosed as primitive neuroectodermal tumor of the sternum by core biopsy of the lesion. She received 2 cycles of preoperative chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide. She underwent a total sternectomy with resection of adjacent bilateral costal cartilages and sternal ends of the clavicles. The skeletal defect of chest wall was reconstructed by polypropylene mesh-resin sandwich. The myocutaneus defect was reconstructed by the pedicled latissimus dorsi myocutaneus flap and the bilateral breast flaps. The postoperative course was uneventful and adjuvant radiotherapy was started 6 weeks after the operation. She died of distant metastases 3 months after the operation, although this patient was free from local recurrence.