臂丛及周围神经鞘瘤手术治疗38例

[目的]探讨臂丛及周围神经鞘瘤的治疗要点和疗效。[方法]1999～2007年共收治经临床和病理证实的神经鞘瘤38例，其中副神经1例，颈丛皮神经2例，臂丛神经6例，膜丛神经2例，上肢神经17例，下肢神经10例。其中37例均在显微镜下获得肿瘤切除，术中均用神经电生理监测，1例将肿瘤切除后，同时行神经移植术。术前复杂部位均行MRI检查，术后所有肿瘤均经病理证实为神经鞘瘤。[结果]本组38例中，36例术后神经功能未受影响，随访6个月～7年，平均4．5年，无复发。1例尺神经鞘瘤切除后出现手内肌功能部分障碍，10d后恢复。L4神经鞘瘤行肿瘤切除神经移植术后，股四头肌肌力下降为2级减，神经移植后疗效正在随访中。[结论]（1）应用显微外科技术显微镜下操作，并采用术中神经电生理监测，可最大限度的切除肿瘤，减少复发，并保留正常神经功能。（2）对于颈部、臂从、膜丛等复杂部位的神经鞘瘤术前尽可能行MRI检查。（3）术后所有神经鞘瘤均应经病理证实。     

阴茎多发性神经鞘瘤1例并文献复习

目的 探讨阴茎多发性神经鞘瘤临床表现及诊治,并行文献复习.方法 报道1例阴茎多发神经鞘瘤.患者以阴茎根部多发结节收治,术前完善彩超、CT及勃起功能检查,拟诊为阴茎硬结症,行结节切除术.结果 结节与阴茎海绵体有明显分界,术后根据病理学特点及免疫组化诊断为阴茎神经鞘瘤,预后良好.结论 阴茎多发神经鞘瘤,临床罕见,主要依靠病...     

椎管内髓外硬膜下多发性肿瘤的诊断与手术治疗

目的探讨椎管内多发性肿瘤的诊断及治疗方法。方法本组男11例,女4例。年龄16～81岁,（51.0±21.2）岁。病程2～27月,（11.0±7.6）月。均无皮肤咖啡斑及体表神经纤维瘤,头颅MR未见异常。增强MR示椎管内髓外硬膜下肿瘤46个,其中≥0.5cm肿瘤35个,肿瘤累及颈段1例,累及胸段5例,累及腰骶段12例。手术方法：采用后正中入路椎板切除术切除肿瘤,〈1cm者切除半椎板,在显微镜下操作,力争保留重要的载瘤神经而将肿瘤自神经上剔除,如肿瘤与神经无法分离,尽可能游离神经根在切除肿瘤后行神经根吻合。术后常规应用抗生素及激素治疗,术后戴颈围领、围腰保护2个月,同时训练腰背部肌肉。结果15例手术肿瘤全切12例,其中1例行神经根吻合;未全切的3例中2例术前诊断为多发神经纤维瘤或神经鞘瘤,因部位散在且直径〈0.5cm未考虑手术切除,另1例术中见肿瘤与多根神经根粘连,病理为转移瘤,仅行部分切除减压术。手术共切除肿瘤33个。病理：神经鞘瘤8例,神经纤维瘤1例,室管膜瘤2例,黑色素瘤1例,腺癌转移1例,脊膜瘤2例。黑色素瘤患者于术后22个月死亡,转移瘤患者于术后1年死亡,余13例随访4～72个月,平均30.1月,肿瘤无复发增长,其中2例多发神经鞘瘤未手术全切除者分别随访12、43个月,肿瘤无明显增长。13例存活者术后KPS评分较术前均有改善（术前75.4±13.3,术后最后一次随访时97.7±6.0,t=-7.366,P=0.000）。结论椎管内髓外硬膜下多发肿瘤以良性居多,中老年多见,半椎板及次全椎板入路显微手术切除是有效的治疗方法,〈0.5cm的无症状肿瘤可严密随访。     

脊柱神经鞘瘤术后无病生存期的风险因素分析

目的 探讨脊柱神经鞘瘤的临床治疗方式,并讨论影响手术预后的风险因素。方法 回顾性分析本科自2016年1月-2017年9月行手术治疗的50例脊柱神经鞘瘤患者临床资料,随访观察其无病生存期,采用单因素及多因素分析探讨其术后无病生存期的影响因素,并采用生存曲线揭示相关因素对患者无病生存期的影响。结果 50例随访中,14例患者复发。患者平均无病生存时间为26.4个月(3～44个月,中位时间24个月)。单因素及多因素分析发现,术前Frankel分级和术后病理结果是其独立危险因素(P0.05)。结论 术前Frankel分级是影响脊柱神经鞘瘤患者肿瘤复发的独立危险因素;与良性脊柱神经鞘瘤相比,恶性脊柱神经鞘瘤患者术后复发率更高,预后更差。     

腹膜后神经鞘瘤1例报告并文献复习

目的报告1例腹膜后神经鞘瘤,并复习相关文献,分析其临床诊治现状。方法通过对1例腹膜后神经鞘瘤的诊断、治疗方法及术后病理、随访的回顾,结合国内外有关文献报道,综合分析腹膜后神经鞘瘤的诊断及治疗状况。结果本例行手术治疗,术后病理证实为腹膜后神经鞘瘤,随访半年无复发。结论腹膜后神经鞘瘤诊断困难,影像学检查无特异性,确诊依靠病理检查,理想的治疗方法是通过外科手术完整切除肿瘤,其预后良好,早期复发率低。     

椎旁巨大伴多发神经鞘瘤一例并文献复习周传坤 黄仕龙 刘丰 高书涛 李潇 方煌

【】 目的 探讨椎旁巨大伴多发神经鞘瘤的临床特点及手术治疗。 方法 结合文献复习,回顾分析1例胸腰段椎旁巨大伴多发神经鞘瘤的临床资料。患者,男性,47岁,因腰痛半年,加重伴左大腿前内侧酸胀麻木3天入院。术前腰椎MRI示：L1-2水平椎管外多发占位性病变,边界清楚,包膜完整。术前诊断：椎旁多发神经鞘瘤。全麻下行全肿瘤切除术+钉棒系统内固定术。 结果 病理检查：肿物大体观呈实性灰白色胶冻样,镜下见肿瘤细胞散在,呈梭形,束状排列,胞核呈卵圆形或波状,异型性少见(HE×400)。免疫表型示：S-100(+),VIM(+),CD34(散在+),EMA(-),GFAP(-),Ki-67 <1%。术后症状缓解,随访6个月未见复发。 结论 脊柱巨大伴多发神经鞘瘤较为少见,可通过影像学检查明确诊断,针对肿瘤特点选择合适的手术方式早期治疗效果良好。     

四肢周围神经鞘瘤40例临床回顾

目的：分析40例周围神经鞘瘤的临床特点，诊断和手术要点。方法：40例神经鞘瘤，其中臂丛神经5例，上肢神经21例，下肢神经14例，单发者38例，多发者2例（同一肢体2个肿瘤），8例肿块位于颈部及上臂近端者术前作MRI检查，证实5例的肿瘤起源于臂丛神经，2例为颈部肿瘤，1例为肺尖部肿瘤。33例行肿瘤完整切除；1例C8神经根肿瘤因邻近胸膜顶及锁骨下动静脉，很难分离，故只能行肿瘤包膜内切除，6例曾在其它医院误将肿瘤与神经干一同切除，行神经修复术。结果：33例术后神经功能正常，随访6个月，－28年未见复发。1例包膜内切除者术后出现手内肌功能部队障碍，出院时已有大部分恢复，仍在随访中，结论：神经鞘瘤能够完整切除者疗效满意。对起源于神经根的肿瘤可行包膜内切除。     

臂丛神经鞘膜瘤的手术治疗

目的 探讨臂丛神经鞘膜瘤的显微外科手术治疗。方法 1975年至今共收治臂丛神经鞘膜瘤17例，源自臂丛上干7例，中干8例，C5、6神经根1例，外侧束1例。14例行显微外科手术切除肿瘤，3例曾在外院误将肿瘤与神经干一并切除，行神经移植修复术。结果14例术后神经功能正常，随访6个月至10年，无1例复发。3例误将臂丛上干切除行神经缺损修复者，术后均有不同程度的功能恢复，效果良好。结论 臂丛神经鞘膜瘤在手术显微镜下逐层剥离包膜，完整切除瘤体，保护好神经干，均能获得较满意的疗效。     

上肢神经鞘瘤的显微手术治疗

目的 探讨上肢神经鞘瘤的显微手术摘除的治疗方法及效果。方法 对37例上肢神经鞘瘤患者行显微手术治疗以摘除神经鞘瘤。结果 本组37例神经鞘瘤均完整切除，术后平均随访14个月（3月～5年），尚未发现复发。结论 上肢神经鞘瘤采用显微手术摘除的方法可将肿瘤彻底摘除，神经主干损伤小，治疗满意。     

双镜联合治疗胃神经鞘瘤4例临床分析

目的:探讨腹腔镜联合胃镜治疗胃神经鞘瘤的临床应用。方法:回顾分析4例胃神经鞘瘤患者临床及诊疗资料。结果:4例患者术前均行胃镜及腹部CT检查(肿瘤直径均<3 cm),仅1例诊断为胃神经鞘瘤;联合腹腔镜及胃镜切除肿瘤,术中快速病理报告均诊为胃间质瘤;手术均顺利完成,手术平均时间(83±15)min;术中平均出血量(43±10)m L;无出血、狭窄等并发症;随访未出现复发及转移。术后病理诊断均为胃神经鞘瘤。结论:腹腔镜联合胃镜对直径<3 cm的胃神经鞘瘤是安全可行的,但术前诊断率仍有待提高。     

Recognizing schwannomatosis and distinguishing it from neurofibromatosis type 1 or 2

BACKGROUND DATA: Schwannomatosis has become a newly recognized classification of neurofibromatosis. Although the genetic loci are on chromosome 22, it lacks the classic bilateral vestibular schwannomas as seen in NF-2. We present the surgical treatment of 4 patients with schwannomatosis, including a brother and sister. METHOD: Case 1 presented with multiple progressively enlarging peripheral nerve sheath tumors. Case 4 presented with a trigeminal schwannoma and a vagal nerve schwannoma. Three of 4 patients had spinal intradural, extramedullary nerve sheath tumors. Surgery in all was multistaged and consisted of spinal laminectomies, site-specific explorations, and microsurgical tumor dissection and resection, with intraoperative neurophysiologic monitoring (including somatosensory-evoked and motor-evoked potentials, upper extremity electromyography and intraoperative nerve action potential monitoring, as appropriate). RESULTS: Intraoperatively the schwannomas had cystic and solid features and in all surgical cases the tumors arose from discrete fascicles of sensory nerve roots or sensory peripheral nerve branches. None of the patients experienced neurologic worsening as a result of their resections. Pathologic analysis of specimens from all cases demonstrated schwannoma. CONCLUSIONS: Not all patients with multiple schwannomas of cranial nerve, spinal nerve root, or peripheral nerve origin have NF-1 or NF-2. In schwannomatosis, these lesions are present in the absence of cutaneous stigmata, neurofibromas, vestibular schwannomas, or parenchymal brain tumors. Schwannomas in schwannomatosis can be large, cystic, and multiple. However, the predominant nerve involvement seems to be sensory and discrete fascicular in origin, facilitating microsurgical resection with minimal deficit.     

Retroperitoneal neural sheath tumors: Cleveland Clinic experience

Retroperitoneal neural sheath tumors are a rare clinical entity with a variable and nonspecific presentation, whose accurate preoperative diagnosis often can be difficult. Since July 1984, 9 retroperitoneal neural sheath tumors, including 3 benign schwannomas, 3 malignant schwannomas and 3 neurofibromas, were evaluated at our institution. Preoperative evaluation included a computerized tomography scan in all patients and magnetic resonance imaging in 4. Magnetic resonance imaging offered better resolution and anatomical definition in certain cases. Preoperative computerized tomography-guided needle biopsy, performed in 3 patients, yielded inaccurate or inconclusive results. The 6 patients with surgically resected benign schwannomas and neurofibromas had no local recurrences and all 6 had no evidence of disease (mean followup 17.3 and 14 months, respectively). Malignant tumors, especially when associated with von Recklinghausen's disease, offered a poor prognosis. Surgical considerations include complete tumor excision with free margins of resection and proper pathological evaluation to determine biological potential.     

Clinical features of multiple spinal schwannomas without vestibular schwannomas

BackgroundMultiple spinal cord tumors in a single patient are very rare and most often seen in cases of neurofibromatosis and associated disorders. Schwannomatosis, which is characterized by the development of multiple schwannomas without vestibular schwannomas, has been newly defined as a distinct form of neurofibromatosis. The purpose of the present study was to describe and review the clinical and radiological features and the management of patients with multiple spinal schwannomas without vestibular schwannomas.MethodsBetween 1986 and 2016, 19 patients with multiple spinal schwannomas without vestibular schwannoma were diagnosed and treated. Of the 19 patients, 13 were males, and 6 were females. The mean age at the first surgery for spinal schwannoma was 45.2 years old. The mean follow-up period was 123.4 months. The clinical features and radiological findings of the patients with multiple spinal schwannomas were retrospectively reviewed.ResultsAmong the 19 patients, there were more than 140 spinal schwannomas. The most common area of spinal schwannoma was the thoracolumbar-lumbar region. Initial symptoms and chief complaints caused by spinal schwannomas were primarily pain in the trunk or extremities in 17 (89.5%) of 19 patients. More than 60 spinal schwannomas were surgically resected. Multiple spinal surgeries were required in six patients. In all 19 patients, surgical treatment has provided successful relief of symptoms and neurological recovery.ConclusionsSurgical treatment was safe and effective in patients with multiple spinal schwannomas without vestibular schwannomas. After surgery, we recommend that all patients be followed with magnetic resonance imaging to monitor for asymptomatic tumors or detect new tumors early.     

Schwannomas in the colon and rectum: a clinicopathologic and immunohistochemical study of 20 cases.

Schwannomas of the colon and rectum are uncommon and incompletely characterized tumors, and only a small number of cases have been reported. This study was undertaken to determine the clinicopathologic profile of such tumors. A total of 20 colorectal schwannomas were identified and analyzed in a review of 600 mesenchymal tumors of the colon and rectum from the files of the Armed Forces Institute of Pathology. The schwannomas occurred equally in men (n = 9) and women (n = 11) in a wide age range (18-87 years; median age 65 years). The most common location was cecum (n = 7), followed by sigmoid and rectosigmoid (n = 6), transverse colon (n = 3), descending colon (n = 2), and rectum (n = 1); the location of one tumor had not been specified. The tumors commonly presented as polypoid intraluminal lesions, often with mucosal ulceration. Rectal bleeding, colonic obstruction, and abdominal pain were the most common presenting symptoms. The most common histologic variant (n = 15) was a spindle cell schwannoma with a trabecular pattern and vague or no Verocay bodies. These tumors ranged from 0.5 to 5.5 cm in diameter. A lymphoid cuff with germinal centers typically surrounded these tumors and focal nuclear atypia was often present, but mitotic activity never exceeded 5 per 50 HPF. All four epithelioid schwannomas occurred in the descending colon or sigmoid, three of them as small submucosal tumors. There was one plexiform schwannoma in the sigmoid composed of multiple nodules of prominently palisading schwann cells similar to those seen in conventional soft tissue schwannomas. All tumors studied were strongly positive for S-100 protein and also for low affinity nerve growth factor receptor (p75), collagen IV, and GFAP. Three tumors had CD34-positive cells, but all were negative for CD117 (KIT), neurofilament proteins, smooth muscle actin, and desmin. The percentage of MIB-1-positive cells was usually less than 1% and never higher than 3%. Colorectal schwannomas behaved in a benign fashion with no evidence of aggressive behavior or connection with neurofibromatosis 1 or 2, based on follow-up information on 18 patients.     

Retroperitoneal schwannoma

BACKGROUND: Retroperitoneal schwannomas are rare, benign tumors. The aim of this study is to present our surgical experience with 7 such tumors. METHODS: Between 1989 and 2004, 7 patients with pathologically proven retroperitoneal schwannomas were reviewed retrospectively. RESULTS: There were 6 male patients and 1 female patient, with a mean age of 43 years (range, 23 to 58 years). Two patients were symptomatic and presented with abdominal discomfort, and none of the patients suffered from von Recklinghausen's disease. All the patients underwent computed tomography scanning, which showed a heterogenous retroperitoneal mass, 4 of which were thought to arise from the adrenals. In 2 patients, calcification was seen in the tumors. All 7 of the patients had a preoperative diagnosis of a retroperitoneal tumor including 3 patients who were thought to have adrenal neoplasms (1 patient had a diagnosis of an adrenal neoplasm excluded on magnetic resonance imaging). Laparotomy and complete excision of tumors were performed in all the patients, and there was no morbidity or mortality. The schwannomas had a mean maximum diameter of 7.3 cm (range, 4 to 14 cm), and they were all benign. At a mean follow-up of 17 months (range, 3 to 48 months) postresection, all the patients remained free from recurrence. CONCLUSION: Retroperitoneal schwannomas are rare tumors that are difficult to diagnose preoperatively. Radiologic findings are usually nondiagnostic. The treatment of choice is complete surgical excision.     

Intrathoracic multiple schwannomas of a single intercostal nerve

Intrathoracic schwannomas are solitary in general. We report a rare case of intrathoracic multiple schwannomas arising from a single intercostal nerve. A 27-year-old man was admitted with multiple tumors, along with the fourth intercostal nerve in the posterior chest wall. Thoracoscopic surgical exploration found a small tumor at 2 cm distal to the main tumors, which had not been detected preoperatively. All these tumors were completely resected en bloc and were diagnosed as schwannomas of the fourth intercostal nerve.     

Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2.

Benign spinal nerve sheath tumors (neurofibromas and schwannomas) often occur on dorsal nerve roots sporadically or in neurofibromatosis types 1 and 2. These are histologically benign tumors, and distinction between them is frequently not made by clinicians. To determine if there is a correlation between the histological pattern of benign spinal nerve sheath tumors and the type of neurofibromatosis, the clinical and pathological features of these tumors (86 surgical specimens and five autopsies) in 68 patients were reviewed. The patients were classified into one of four categories: neurofibromatosis type 1, neurofibromatosis type 2, uncertain, or sporadic. The diagnostic criteria used for neurofibromatosis types 1 and 2 were established by the National Institutes of Health. Patients who did not fulfill criteria for either neurofibromatosis type 1 or 2 but who had multiple nervous system tumors or other stigmata of neurofibromatosis were designated "uncertain." Spinal nerve sheath tumors were considered sporadic in 42 cases (40 schwannomas and two neurofibromas). In the 14 patients with neurofibromatosis type 1, all spinal nerve sheath tumors were neurofibromas. In six of the seven patients with neurofibromatosis type 2, all spinal nerve sheath tumors were schwannomas. One patient with neurofibromatosis type 2 had a spinal nerve sheath schwannoma and a tumor with features of both tumor types. The authors conclude that spinal nerve sheath tumors in patients with neurofibromatosis type 1 are neurofibromas. In contrast, spinal nerve sheath tumors occurring in neurofibromatosis type 2 or sporadically are most frequently schwannomas. The distinct histological features of these tumors may reflect different pathogenetic mechanisms even though they arise at identical sites in neurofibromatosis types 1 and 2.     

Management of benign retroperitoneal schwannomas: a single-center experience

Background

We describe the presentation, diagnostic pathway, and management of 28 patients with benign retroperitoneal schwannomas.

Methods

A prospectively kept soft-tissue tumor database was reviewed to identify patients who presented to the Royal Marsden Hospital with retroperitoneal schwannomas.

Results

From 2001 to 2009, 28 patients presented with retroperitoneal schwannomas. In 13 patients, tumors were identified incidentally, 8 patients presented with pelvic or abdominal symptoms and 7 patients presented with a palpable mass. Seventeen occurred in the pelvis and 11 occurred in the abdominal retroperitoneal space. The median age was 47 years and 21 patients were women; the mean follow-up period was 39 months. Twenty patients underwent resection and 8 patients were managed conservatively with radiologic surveillance. An initial histologic diagnosis was accomplished in 19 of 28 patients. Complete resection was achieved in 17 of 20 patients and 3 patients with pelvic schwannomas underwent a subtotal resection. Resected tumor size ranged from 5 to 23 cm (median, 9 cm), and weighed between 64 and 2,300 g (median, 500 g). There was no surgical mortality. In the 3 subtotal resected tumors, no progression of residual disease or malignant transformation has been noted on follow-up imaging. All 8 patients in the surveillance group had a histologic diagnosis and typical radiologic findings.

Conclusions

An accurate preoperative diagnosis is important because the risks of complicated surgery in the retroperitoneum and pelvis for what is a benign lesion should be considered carefully. Cross-sectional imaging combined with a needle biopsy should in all cases provide the correct diagnosis. Management options include radiologic surveillance in asymptomatic patients or surgical resection in symptomatic patients.     

Management of patients with schwannomatosis: report of six cases and review of the literature

BACKGROUND: Schwannomatosis is a rare tumor syndrome characterized by the presence of multiple schwannomas without the stigmata of neurofibromatosis (NF) Type 1 or 2. To better understand the natural history and clinical management of the syndrome, a retrospective review was conducted of patients diagnosed with schwannomatosis over an 11-year period at the University of Pennsylvania Medical Center (UPMC). METHODS: Between 1990 and 2001, 131 patients underwent surgery for resection of spinal or peripheral nerve schwannomas in the Department of Neurosurgery at the University of Pennsylvania Medical Center. Among the 131 patients, there were 6 who had two or more pathologically proven schwannomas without radiographic or clinical evidence of vestibular schwannomas. The hospital charts, clinic notes, radiology films, operative reports, pathology slides, and reports from all 6 patients were retrospectively reviewed. RESULTS: The patient population consisted of 6 patients with a mean age of 48.7 (3 male: 3 female). All patients had enhanced brain magnetic resonance imaging (MRI) scans that were negative for vestibular schwannomas. Ophthalmological and general physical examinations did not reveal any findings suggestive of NF. There was no family history of NF or schwannomatosis. The locations of the schwannomas included intraspinal (multiple sites), paraspinal, brachial plexus, femoral nerve, sciatic nerve, calf, forearm, retroperitoneum, and middle cranial/infratemporal fossa region. The common presenting symptoms included paresthesias, palpable mass, pain, or weakness. All 6 patients underwent surgical resection of symptomatic lesions. CONCLUSIONS: For patients with schwannomatosis, surgery is indicated for symptomatic lesions, while asymptomatic tumors are followed conservatively. Because these patients are at increased risk for developing multiple schwannomas, we recommend regular surveillance and offer genetic counseling even though the pattern of inheritance is unknown.     

Gamma knife radiosurgery for trigeminal schwannomas

BACKGROUND: Trigeminal schwannomas are uncommon tumors that may be microsurgically removed in selected cases, albeit with significant risk for new neurological morbidity. We evaluated the role of stereotactic radiosurgery as an alternative for patients with newly diagnosed or residual trigeminal schwannomas. METHODS: The records of 23 patients who underwent radiosurgery for trigeminal schwannoma were reviewed. The most frequent presenting symptoms were facial numbness (11 patients), diplopia (6 patients), and facial pain (3 patients). One patient presented with partial complex seizures as the first symptom. Twelve patients had undergone one or more prior resections. Eleven underwent radiosurgery on the basis of imaging diagnosis only. The mean tumor volume was 4.5 mL (range 0.46-11.2 cc). Radiosurgery was performed using a median marginal dose of 15 Gy (range, 13-20 Gy). RESULTS: At a median imaging follow-up of 40 months (range, 12-146), 20 of 22 evaluable patients (91%) had tumor growth control (regression in 15 and no further tumor growth in 5). One 80-year-old patient died of unrelated cause 4 months after radiosurgery. Two patients with enlarged tumors were treated effectively with repeat radiosurgery. Twelve of 23 patients (52%) reported improvement and 9 (39%) had no change in their symptoms. Two patients noted new neurological complaints (transient facial weakness in 1 patient and worsening of the preradiosurgery facial numbness in another patient). CONCLUSIONS: Radiosurgery is an effective minimally invasive management option for patients with residual or newly diagnosed trigeminal schwannomas.