Giant neurogenic tumors of mediastinum: report of two cases and literature review

Neurogenic tumors are commonly found in the mediastinum, especially in the posterior mediastinum or in the chest wall, neurogenic tumors may reach large size before becoming symptomatic. If the neurogenic tumor occupied more than half size of the chest wall accompanied by mediastinal shift, tracheal compression, or superior vena reflux disorder, it may be called giant intrathoracic neurogenic tumors. Giant intrathoracic neurogenic tumors are relatively rare. Most of intrathoracic neurogenic tumors were benign or low-grade malignant tumors in nature. Complete surgical excision should be the rule for these patients. We report two cases of giant neurogenic tumors, and study the clinical manifestations, diagnostic methods, surgical management, and prognosis in the light of the most important published data.

Comprehensive clinicopathologic characteristics of intraabdominal neurogenic tumors:Single institution experience

BACKGROUND Neurogenic tumors are rare but represent an important consideration in the differential diagnosis of abdominal mesenchymal tumors.Reports on their incidence,pathological features and clinical characteristics are scarce.AIM To advance the overall knowledge on the histologic,immunohistochemical,clinical and radiologic characteristics of neurogenic tumors through this case series.METHODS An established database of a nationwide tertiary referral center,covering a 15-year period(2005 and 2020),was retrospectively re-evaluated.Diagnoses of neurogenic tumor cases were confirmed by two experts following review of the macroscopic,histological and immunohistochemical records along with findings from analysis of archived tissue sections for each included patient.Tissue microarrays were constructed for cases lacking necessary immunohistochemical studies.Clinical data and follow-up information were collected from the hospital records and the patients themselves,when available.RESULTS The study included 19 cases of intraabdominal neurogenic tumors,representing 12 women and 7 men, between 18 and 86 years of age (median: 51 years). Finalconfirmed diagnoses were 12 schwannomas, 2 diffuse submucosal neurofibromatoses,2 ganglioneuromas, 2 malignant peripheral sheath nerve tumors,and 1 mucosal Schwann cell hamartoma. Sizes of the tumors were variable, with amedian diameter of 4 cm;the two largest (> 10 cm) were schwannomas. Themajority of cases were asymptomatic at presentation, but the most frequentsymptom was abdominal pain. Gastrointestinal tract lesions were detected withendoscopy and extra-luminal lesions were detected with cross-sectional imaging.All cases were S100-positive and CD117-negative;most cases were negative fordesmin, epithelial membrane antigen, smooth muscle actin and CD34. In all but 5cases, the Ki67 proliferation index was ≤ 1%.CONCLUSION Re-evaluation of 19 cases of abdominal neurogenic tumors demonstrated considerablevariability in clinicopathologic characteristics depending on location,dimension and histological features.

DIAGNOSIS AND TREATMENT OF MEDIASTINAL NEUROGENIC TUMORS--REPORT OF 110 CASES

Object.To study the clinical manifestations,diagnostic methods,surgical management ,and prognosis of various neurogenic mediastinal tumors.Method.One hundred and ten cases of neurogenic tumors of mediastinum were reviewed and retro-spectively analyzed.Results.Operative or hospitalization death happened in2cases,complications occurred in8cases,mostly were Horner’s syndrome or recurrent nerve paralysis.During the follow?up,there were2recur-rences in102benign tumors,and4cases of neurofibrosarcoma or malignant neurilemmoma died within3years postoperatively.Conclusion.Most mediastinal neurogenic tumors are benign.It is enough to establish diagnosis by chest X?ray and CT scan.There are different features in the clinical manifestations,diagnosing methods,and surgical management for the dumbbell tumors.Minimal invasive surgery and video?assisted thora-coscopy surgery(VATS)have a special value in treatment of the selected neurogenic mediastinal tumors.Benign neurogenic tumors rarely recur after complete resection,whereas malignant neurogenic tumors have poor prognosis.

Classification of submucosal tumors in the gastrointestinal tract

This review is part two of three, which will present an update on the classification of gastrointestinal submucosal tumors. Part one treats of the diagnosis and part three of the therapeutic methods regarding gastrointestinal submucosal tumors. In the past there has been some confusion as to the classification of gastrointestinal submucosal tumors. Changes in classifications have emerged due to recent advances in mainly immunohistochemistry and electron microscopy. The aim of this paper is to update the reader on the current classification. Literature searches were performed to find information related to classification of gastrointestinal submucosal tumors. Based on these searches the twelve most frequent submucosal tumor types were chosen for description of their classification. The factors that indicate whether tumors are benign or malignant are mainly size and number of mitotic counts. Gastrointestinal stromal tumors are defined mainly by their CD117 positivity. In the future, there should be no more confusion between gastrointestinal stromal tumors and other types of submucosal tumors.

Tension pneumocephalus following endoscopic resection of a mediastinal thoracic spinal tumor:A case report

BACKGROUND Pneumocephalus is a rare complication presenting in the postoperative period of a thoracoscopic operation.We report a case in which tension pneumocephalus occurred after thoracoscopic resection as well as the subsequent approach of surgical management.CASE SUMMARY A 66-year-old man who received thoracoscopic resection to remove an intrathoracic,posterior mediastinal,dumbbell-shaped,pathology-proven neurogenic tumor.The patient then reported experiencing progressively severe headaches,especially when in an upright position.A brain computed tomography scan at a local hospital disclosed extensive pneumocephalus.Revision surgery for resection of the pseudomeningocele and repair of the cerebrospinal fluid leakage was thus arranged for the patient.During the operation,we traced the cerebrospinal fluid leakage and found that it might have derived from incomplete endoscopic clipping around the tumor stump near the dural sac at the T3 level.After that,we wrapped and sealed all the possible origins of the leakage with autologous fat,tissue glue,gelfoam,and duraseal layer by layer.The patient recovered well,and the computed tomography images showed resolution of the pneumocephalus.CONCLUSION This report and literature review indicated that the risk of developing a tension pneumocephalus cannot be ignored and should be monitored carefully after thoracoscopic tumor resection.

Peripheral primitive neuroectodermal tumor of the posterior mediastinum: A case report

Peripheral primitive neuroectodermal tumor(pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed small round-cell tumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.

中耳神经源性肿瘤8例的诊治体会

目的 探讨中耳神经源性肿瘤的临床特征、诊治方法及预后。方法 回顾性分析2010年1月—2023年3月收治的8例经病理确诊为中耳神经源性肿瘤患者的临床资料。8例患者首发症状中以耳流脓为主4例,听力下降3例,面瘫1例。5例肿物突入外耳道,3例鼓膜紧张部穿孔。纯音测听示8例患者均伴有不同程度的听力下降,其中混合性聋4例、传导性聋3例、极重度感音神经性聋1例。结果 所有患者均完善中耳CT及MRI,提示肿瘤累及中鼓室或鼓岬表面5例,累及面神经水平段3例,其中1例侵入中颅窝。8例患者均行乳突改良根治术,术后病理证实鼓室体副神经节瘤4例、面神经纤维瘤2例、面神经鞘膜瘤1例、中耳神经内分泌腺瘤1例;2例患者术前行活检分别诊断为中耳胆脂瘤和慢性化脓性中耳炎,术前病理误诊率为25%(2/8)。术后随访1个月至8年,目前尚无复发病例。结论 中耳神经源性肿瘤发病率较低且临床表现各异,存在一定的误诊率,术前完善CT和增强MRI检查以及术后病理及免疫组化有助于明确诊断。手术是治疗该病的有效手段。

基于计划行为理论的靶向性康复训练在椎管内肿瘤神经源性膀胱患者中的应用效果

目的探究基于计划行为理论的靶向性康复训练对椎管内肿瘤神经源性膀胱(NB)患者膀胱功能恢复的效果。方法选取2019年8月至2022年7月郑州大学附属肿瘤医院接收的椎管内肿瘤NB患者86例,按照随机数字表法分为观察组(43例)和对照组(43例)。对照组接受常规康复训练,观察组接受基于计划行为理论的靶向性康复训练。比较两组排尿情况、尿动力学指标变化情况及生活质量评分,评估膀胱功能恢复情况及并发症发生情况。结果训练前,两组排尿情况、尿动力学指标、SF-36量表评分差异无统计学意义(P>0.05)。训练后,观察组最大排尿量多于对照组,日均尿失禁次数、残余尿量少于对照组(P<0.05);观察组最大尿道压力、最大尿流速率及膀胱排尿压力高于对照组(P<0.05);观察组SF-36量表评分高于对照组(P<0.05)。观察组并发症发生率低于对照组(P<0.05)。结论基于计划行为理论的靶向性康复训练可促进椎管内肿瘤NB患者膀胱功能及排尿反射恢复,降低并发症发生率,提高患者生活质量。

原发性腹膜后神经源性肿瘤93例分析

目的 探讨原发性腹膜后神经源性肿瘤的临床病理特征及外科治疗原则 ,以提高手术切除率及安全性。方法 回顾性分析 13年间经手术和病理证实的原发性腹膜后神经源性肿瘤患者的临床资料。结果 神经源性肿瘤占同期腹膜后肿瘤的 2 0 .6% (93 /4 5 2 ) ,其中神经鞘瘤 2 4例(2 5 .8% )、恶性神经鞘瘤 19例 (2 0 .4% )、副节瘤 14例 (15 .1% )、神经纤维瘤 16例 (17.2 % )、其他少见肿瘤 2 0例 (2 1.5 % )。术前CT和MRI检查的诊断率分别为 65 .6%和 73 .9%。手术切除率为97.9% (91/93 ) ,2例行探查活检。良、恶性肿瘤术后的 3 ,5年生存率分别为 95 .9% ,80 .1%和42 .4% ,10 .5 %。良性肿瘤术后复发 8例 ,复发率为 13 .1% ;恶性肿瘤复发 14例 ,复发率为 43 .8%。结论 CT和MRI对本病诊断有辅助意义 ;治疗首选手术切除。良性肿瘤预后良好 。

胸腔镜手术治疗后纵隔良性神经源性肿瘤40例报告

目的探讨胸腔镜在后纵隔神经源性肿瘤治疗中的价值。方法1994年5月～2006年5月,经胸腔镜手术切除40例后纵隔良性神经源性肿瘤,全麻双腔气管插管,套管切口根据肿瘤位置而定,如肿瘤过大,切除困难,可辅助小切口。结果34例经胸腔镜顺利完成肿瘤切除,6例附加6cm小切口完成手术。神经鞘瘤20例,神经纤维瘤14例,神经节细胞瘤6例。肿瘤直径平均4.7cm(2～12cm)。术后平均胸腔闭式引流2.2d(1～7d)。术后平均住院4.8d(3～13d)。轻微并发症2例,无手术死亡。随访9个月～12年,其中<3年16例,3～5年12例,>5年7例,失访5例。局部复发1例,接受二次手术治疗。结论胸腔镜切除后纵隔良性神经源性肿瘤安全、可靠、创伤小,可作为该疾病的首选治疗方法。

胃间质瘤临床分析

目的 探讨胃间质瘤的诊治方法。方法 回顾分析 14例胃间质瘤的临床表现、病理特点、诊断及治疗情况。结果 14例中 ,7例位于胃体底部 ,7例位于胃窦部 ;主要的临床症状为腹部疼痛 ;均经胃镜、胃肠钡餐、CT及B超等检查发现。 14例均经手术切除 ,病理组织学显示良性 8例(5 7.1% ) ,不确定型 5例 (3 5 .7% ) ,恶性 1例 (7.1% ) ;术后复发 3例 ,死亡 1例。结论 胃间质瘤的治疗以手术为主 ,术后应长期密切随访 。

哑铃型纵隔神经源性肿瘤的外科治疗

目的 探讨哑铃型纵隔神经源性肿瘤的临床特征及外科治疗原则 ,以提高手术疗效。方法 分析 7例哑铃型纵隔神经源性肿瘤的临床特征和影像学特点 ,所有病人均经手术治疗和病理证实。结果 肿瘤全部切除 5例 ,次全切除 2例。治愈 5例 ,复发 2例。结论 一期分别切除哑铃型纵隔神经源性肿瘤的胸腔内部分和椎管内部分。防止椎管内出血、脊髓损伤。关胸时勿使椎间孔切开处与胸腔相通 ,严密缝合硬脊膜 ,防止脑脊液漏。

生大黄对重症颅脑损伤并发NPE患者血清TNF-α、IL-6水平的影响

目的观察生大黄对重型颅脑损伤并发神经源性肺水肿(NPE)患者血清肿瘤坏死因子α(TNF-α)、白细胞介素6(IL-6)水平的影响。方法将38例颅脑损伤并发NPE患者随机分为治疗组20例和对照组18例,两组均予常规综合治疗,治疗组另予生大黄粉鼻饲3 g/次,3次/d,疗程为7 d。分别于治疗前、治疗后1、4、7 d,检测患者血清中的TNF-α、IL-6。结果治疗后第20天,治疗组死亡5例,对照组死亡7例,两组比较,P<0.05。治疗后第4、7天,治疗组血清TNF-α、IL-6水平明显低于对照组(P均<0.05)。结论生大黄粉鼻饲可降低重型颅脑损伤并发NPE患者血清中的TNF-α、IL-6水平。

四肢软组织神经源性肿瘤的MRI诊断

目的 探讨四肢软组织神经源性肿瘤的MRI诊断价值。方法 回顾性分析 13例由病理证实的四肢软组织神经源性肿瘤的MRI表现。结果 神经鞘瘤 8例 ,神经纤维瘤 2例 ,恶性神经鞘瘤 3例。神经源性肿瘤多表现为卵圆形或梭形 ( 9/13 ) ,边界清楚 ( 13 /13 ) ,与周围神经关系密切 ( 10 /13 ) ,T1WI为均匀等信号 ( 8/13 ) ,T2WI为不均匀高信号 ( 13 /13 ) ,Gd DTPA增强后不均匀明显强化 ( 13 /13 )。结论 结合肿瘤的形状、信号及与周围神经毗邻等特点 ,提示软组织神经源性肿瘤 ,而肿瘤大小、形状、信号均匀度、有无囊变 ,以及强化特点等有助于良、恶性的鉴别。

胃肠道间质瘤的诊断和治疗:附32例报告

目的探讨胃肠道间质瘤的诊断和治疗方法。方法回顾性分析近5年间收治的3 2例胃肠道间质瘤的临床和病理资料。结果肿瘤多位于胃部,最多见的临床表现为腹部隐痛不适。手术前8 1.3%(2 6/3 2)的病例有不同程度的误诊。均行外科手术切除。其中因复发而2次手术者1 0例,3次及以上手术者8例。随访时间6个月至5年,死亡8例。结论胃肠道间质瘤容易误诊,其确诊主要依靠病理组织学检查及免疫组化染色。治疗以外科手术切除为主。复发的病例亦可反复手术而取得较为满意的效果。

腹腔镜胃基质瘤切除术:附32例报告

目的探讨腹腔镜胃基质瘤切除术的临床应用价值.方法回顾性总结32例采用腹腔镜方法治疗胃基质肿瘤患者的临床资料.肿瘤直径1.5～5.5(平均2.6)cm.手术方式主要为腹腔镜下胃肿瘤切除术、腹腔镜辅助和手助腹腔镜下胃肿瘤切除术.结果32例均手术均顺利完成.平均手术时间75 min,术中平均出血50mL.术后疼痛轻微,术后排便、排气时间平均为34h,术后平均住院天数为7.5 d.术后病理证实25例为良性基质瘤,7例为低度恶性基质瘤.随访8～30个月未见复发.结论腹腔镜下胃基质瘤切除术是一种安全、有效的微创手术方法,值得临床推广应用.

软组织神经源性肿瘤的MRI诊断

目的：评价ＭＲＩ对软组织神经源性肿瘤的诊断价值。材料与方法：回顾分析１３例软组织神经源性肿瘤的ＭＲＩ表现，其中良性神经源性肿瘤１０例，恶性３例，均经手术病理证实。结果：大部分肿瘤表现为卵圆形或梭形（ｎ＝１１），边界清晰（ｎ＝１１），与邻近神经血管束关系密切（ｎ＝１２）。Ｔ１ＷＩ上均匀或轻度不均匀（ｎ＝９），均为等于或稍高于肌肉信号。Ｔ２ＷＩ上为中等或明显不均匀（ｎ＝１０）高信号，仅１例神经纤维瘤于Ｔ２ＷＩ上表现为“靶征”。ＧｄＤＴＰＡ增强后为不均匀（ｎ＝１０）中等或明显强化。结论：结合肿瘤的形态和ＭＲ信号特点，可提示软组织神经源性肿瘤的诊断。而良、恶性神经源性肿瘤的ＭＲＩ表现无特异性。

骶骨神经源性肿瘤的X线平片及CT分析(附6例报告)

目的：通过分析骶骨神经源性肿瘤的Ｘ线和ＣＴ表现，提高其诊断水平。材料与方法：６例骶骨神经源性肿瘤，其中神经鞘瘤４例，恶性神经鞘瘤１例，神经纤维瘤１例。对其平片及ＣＴ表现进行分析。结果：平片表现：骶骨神经源性肿瘤多位于上部骶椎，偏侧性生长，骶孔或骶骨内压性扩大破坏或变形及多囊状膨胀溶骨性骨质破坏；边缘骨质硬化。ＣＴ表现：膨胀的溶骨破坏区被软组织肿块充填，骨壁变薄，肿块无钙化。肿瘤破坏骨壁或骶孔侵入软组织及邻近骨质时应考虑为恶性。结论：ＣＴ能提供平片不能显示的更多信息，疑有骶骨肿瘤时宜做ＣＴ检查。骶骨神经源性肿瘤要与骶骨脊索瘤、巨细胞瘤鉴别。

颈部外周神经源性肿瘤的影像学诊断

目的 探讨颈部外周神经源性肿瘤的影像学表现及其诊断价值。资料与方法 33例颈部外周神经源性肿瘤 ,包括神经鞘瘤 2 8例 ,恶性神经鞘瘤 2例 ,神经纤维瘤 3例。 2 6例行CT扫描 ,4例行MRI扫描 ,3例同时行MRI和CT扫描。结果 颈部外周神经源性肿瘤因发生在特定的解剖部位 ,易于辨认 ,依据肿瘤引起的骨质缺损或附近间隙、肌肉、血管等移位方向 ,还可推测出它来自于哪一组神经。神经鞘瘤病理上有不同成分组成 ,故在影像学上多呈不均匀低密度 (低信号 )肿块 ,增强后不均匀现象更明显。根据这一现象可作出定性诊断。结论 CT和MRI扫描是诊断该肿瘤的有效方法。

经鼻内镜下摘除蝶鞍占位性病变(附7例分析)

目的 探讨经鼻内镜下蝶鞍手术方法的适应证、应用价值及应用前景，并探索性提出经鼻－经鼻中隔－经筛－经蝶入路摘除蝶鞍巨大肿块。方法 蝶鞍占位性病变病人共７例：垂体微腺瘤５例、巨大垂体腺瘤１例、鞍区炎性坏死性肉芽肿１例。６例采用经鼻－蝶入路；１例采用经鼻－鼻中隔－筛－蝶入路。结果 术前症状在术后均有不同程度的改善（除１例术前有一眼失明者外），无１例出现严重并发症，６例蝶鞍占位获得全切；１例获得次全切。结论 经鼻内镜下摘除蝶鞍占位性病变具有很好的推广应用前景。内镜下经鼻－鼻中隔－筛－蝶入路为蝶鞍巨大肿块的切除提供了一条较佳的手术途径。