肱三头肌恶性周围神经鞘瘤1例

恶性周围神经鞘瘸(malignant peripheral nerve sheath tumor,MPNST)是临床上一种较少见的软组织恶性肿瘤,占全身软组织恶性肿瘤的5%～10%,原发于肱三头肌的MPNST极罕见,Miura等[1]曾于1986年报道过1例.我科于2007年2月收治了复发性肱三头肌恶性周围神经鞘瘤1例.现报告如下.     

臂丛神经鞘瘤的治疗体会

神经鞘瘤是常见的周围神经肿瘤,但恶性神经鞘瘤较少见。有时病理切片可见神经鞘瘤束状区内有少量核分裂相及异形核细胞,容易被误诊为恶性变,应仔细鉴别。恶性神经鞘瘤来自小神经者,肿瘤与神经关系不明确。来自大神经,神经干常呈浸润性生长的圆形肿瘤包绕。恶性神经鞘瘤累及一段神经时,常难以确定肿瘤切缘,通常应在累及神经段的上、下切缘做冰冻切片检查浸润程度。对低度恶性的表浅肿瘤广泛切除可达治愈。高度恶性的肿瘤如累及较大神经干时,则可牺牲此神经。有作者尚建议若肿瘤深在,累及神经主干时需截肢手术。术中怀疑恶性神经鞘瘤应做冰冻切片。     

周围神经鞘瘤的显微外科治疗

目的：探讨周围神经鞘瘤显微手术治疗的效果。方法回顾性分析54例经显微手术切除神经鞘瘤的疗效。结果本组54例均取得良好效果。经过1～2年的随访,5例恶性神经鞘瘤无复发;良性神经鞘瘤,1例术后出现皮肤感觉麻木,3例出现运动功能障碍,6个月后基本恢复。结论周围神经鞘瘤经显微手术治疗可最大限度地降低神经的损伤,可最大程度地完整切除肿瘤,对于恶性神经鞘瘤,扩大切除可降低复发。     

臂丛恶性神经鞘瘤的诊断和治疗

臂丛恶性神经鞘瘤在周围神经中较少见。1996—2000年。我院共收治6例，对其中4例行手术治疗，2例未手术。臂丛恶性神经鞘瘤死亡率高，预后差。     

阴囊神经鞘瘤1例报道并文献复习

目的 探讨阴囊神经鞘瘤的疾病特点,提高对阴囊神经鞘瘤的临床诊治水平.方法 对1例阴囊神经鞘瘤病例进行报道,并结合文献报道的7例资料进行分析总结.结果 8例中,良性神经鞘瘤4例,平均直径6.5 cm,平均病程4.4年,年龄34～48岁,平均年龄38.4岁,超声检查提示肿瘤与周围组织界限清楚;1例合并阴茎癌良性神经鞘瘤,患者年龄78岁;3例恶性神经鞘瘤平均直径12.6 cm,平均病程2.2年,平均年龄75岁,超声检查提示肿瘤边界不清,内部回声不均.结论 阴囊神经鞘瘤极为罕见,目前确诊依赖于术后病理检查.对于阴囊肿瘤应尽早手术切除,手术是目前治疗的有效方法,以避免恶性肿瘤的诊治延误.     

臂丛恶性神经鞘瘤一例报告

神经鞘瘤为周围神经常见肿瘤之一,但位于臂丛的恶性神经鞘瘤则罕见。１９９４年～１９９７年,我科共诊治１１３例臂丛神经疾患,其中仅有１例为左侧恶性臂丛神经鞘瘤。患者男性,３９岁。因左前臂进行性麻木,左上肢部分肌肉萎缩８个月而入院。入院前曾在门诊诊治,因当...     

颈部神经鞘瘤12例临床分析

颈部神经鞘瘤１２例临床分析中国医科大学第一临床学院（沈阳，１１０００１）刘庆华单吉贤王舒宝陈峻青神经鞘瘤是一种神经源性肿瘤，起源于周围神经的雪旺细胞（Ｓｃｈｗａｎ细胞）故又称雪旺细胞瘤。颈部神经鞘瘤临床上缺乏典型的临床表现，极易误诊。现将１２例颈部神...     

甲状腺周围神经鞘瘤2例报告

<正>神经鞘瘤是一种起源于周围神经鞘内施万细胞的神经源性肿瘤,多为良性,可发生于全身各个部位^[1],该病在头颈部发病率为25%～45%^[1-2]。甲状腺周围神经鞘瘤非常罕见^[3],临床缺乏典型症状,术前极难与甲状腺腺瘤等甲状腺常见病变相鉴别。近期,吉林大学中日联谊医院收治了2例该病例。现报告如下。     

人工神经鞘管预防周围神经鞘瘤术后刺激性神经痛的临床研究

目的 探讨人工神经鞘管对周围神经鞘瘤切除术后刺激性神经痛的预防作用. 方法 将30例神经鞘瘤患者随机分为人工神经鞘管组和对照组,每组各15例.两组均按照常规方法行肿瘤切除术,人工神经鞘管组行肿瘤切除后神经局部用人工神经鞘管包裹,对照组仅行肿瘤切除术.两组分别于术后3个时间点采用视觉模拟量表(VAS)对疼痛进行量化评分并计算刺激性神经痛发生率,运用统计学方法对数据进行分析. 结果 对照组术后3个时间点疼痛评分分别为7.5、5.1和2.8,疼痛发生率分别为80％、67％和40％;人工神经鞘管组3个时间点疼痛评分分别为3.2、1.5和0,疼痛发生率分别为33％、13％和0.经统计学分析,两组各时间段平均疼痛评分及刺激性神经痛发生率的差异均有统计学意义(P＜0.05). 结论 人工神经鞘管能够显著降低神经鞘瘤切除术后刺激性神经痛的疼痛程度及发生率,是临床预防刺激性神经痛实用、有效的方法.     

神经鞘瘤

神经鞘瘤是周围神经中常见的良性肿瘤,一般很少恶变。本文综述了神经鞘瘤的病理、分型、临床表现、诊断和治疗。     

A resected case of malignant peripheral nerve sheath tumor (MPNST) necessary to distinguish from Askin tumor

We have treated a patient with malignant peripheral nerve sheath tumor (MPNST) for 7 years by interdisciplinary therapy. The diagnosis and treatment are reported with the clinical course so far. A 20-year-old man underwent surgical resection of a tumor on the left side of the chest in 1990, and a diagnosis of Askin tumor was made. In 1993, the disease recurred in the left axilla and was surgically resected. The surgical specimen was investigated histologically, and a diagnosis of MPNST was made instead of Askin tumor. Recently, a tumor began to grow in the left pleural cavity and was surgical resected. The tumor tissue contained both cells with oval and long spindle nuclei, which were intermingled with each other. Immunohistochemically, the tumor cells were positive for vimentin and some of them were positive for S100 protein. Because electron microscopy showed cytoplasmic processes on the tumor cell and basal lamina-like structures, a diagnosis of recurrent MPNST was made. Because this lesion resembled Askin tumor in its clinical manifestations and histological characteristics, differential diagnosis from Askin tumor was necessary. When a malignant soft tissue tumor arising from the mediastinum or chest walls is detected, immunohistochemical and electron microscopic studies should be performed. The diagnosis should be based on the overall findings including the location and clinical data.     

Intrasellar malignant peripheral nerve sheath tumor (MPNST)

Summary Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.     

Would a Massive Intra-abdominal Malignant Peripheral Nerve Sheath Tumor with Growth into the Inguinal Canal and Scrotum Preclude Surgical Option? A Case Report and Review of Literature

Malignant peripheral nerve sheath tumors (MPNST) are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest accounting for less than 10 % of all soft tissue sarcomas. They arise from major or minor peripheral nerve fibers or their sheaths. The World Health Organization coined the term MPNST for tumors of neurogenic origin with similar biological behavior replacing all the previous heterogeneous and, often, confusing nomenclature including malignant schwannoma, malignant neurilemmoma, and neurofibrosarcoma. The retroperitoneum and the lower extremities are the most common sites, but MPNST may arise anywhere in the body. Its location in the retroperitoneum in a patient without neurofibromatosis is an exceedingly rare occurrence. Imaging is routinely performed to assess the extent of the disease and to plan surgical resection. Surgical resection is the first line of therapy, ideally with total removal of the tumor. Owing to a high risk of recurrence with incomplete resection, postoperative irradiation and chemotherapy are necessary; however, they are often used as adjuvant therapy even if the tumor is completely resected.     

Malignant peripheral nerve sheath tumor of the esophagus

BACKGROUND: Sarcomas of the esophagus are rare representing 0.1-1.5% of all esophageal tumors. We report a case of malignant peripheral nerve sheath tumor (MPNST) of the esophagus in a 60-year-old woman. METHODS: The diagnosis was made preoperatively on endoscopic biopsy and confirmed after tumor resection by immunohistochemistry as well as electron microscopy. The patient underwent abdominal-thoracicen bloc esophagectomy with mediastinal lymphadenectomy and intrathoracic esophagogastrostomy. RESULTS: Our therapeutic concept for the first case of a high-grade MPNST (malignant schwannoma) of the esophagus resulted in a recurrence-free interval of 4 years. CONCLUSION: The therapy of choice was abdominal-thoracic en bloc esophagectomy with tumor-free resection margins and esophageal reconstruction with the stomach. After R0-resection we found no indication for adjuvant radio- and/or chemotherapy.     

Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report

Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) made up of both malignant schwannoma cells and malignant rhabdomyoblasts.A 26-years-old male patient was admitted with an asymptomatic gluteal mass. Magnetic resonance imaging showed heterogeneous soft tissue mass and he underwent open biopsy. Malignant peripheral nerve sheath tumor was diagnosed. He was given adjuvant chemotherapy following the removal of the tumor with hip disarticulation. The tumor was diagnosed as “malignant triton tumor” based on pathological examination including immunohistochemical studies. There were no signs of metastasis but recurrence was observed at 9 months follow up.MTT is usually associated with Neurofibromatosis 1 and located in head, neck region. In this case sporadic involvement of gluteal region and aggressive behavior of the lesion despite radical surgery was demonstrated.     

Malignant triton tumor of the nasal cavity.

BACKGROUND: Malignant triton tumor is usually an aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. However, malignant triton tumor of the nasal cavity is very rare. METHODS: A case report of a 38-year-old woman with polypoid mass, which bled easily in the right nasal cavity, and nasal obstruction is presented with a review of the literature pertaining to this unusual case. RESULTS: The malignant triton tumor was treated with wide surgical excision followed by radiation therapy. Histopathological diagnosis of the malignant triton tumor was made on the surgical specimen. The patient's postoperative course was unremarkable, and follow-up at 5 years reveals no evidence of disease. CONCLUSIONS: A malignant triton tumor in the nasal cavity is a rare disease. Head and neck surgeons should recognize the possibility of malignant triton tumor occurring in the nasal cavity.     

膀胱非上皮性肿瘤的诊治

目的 总结膀胱非上皮性肿瘤的诊治经验。方法 对1953～2002年收治的28例膀胱非上皮性肿瘤患者的诊治情况进行总结、分析。结果 膀胱非上皮性肿瘤的主要临床表现为血尿、盆腔肿块、尿频、排尿困难等症状。主要辅助检查为B超、CT、膀胱镜检查及镜下活检。本组28例中,经术后病理检查,恶性肿瘤17例(占61．7％),有7种病理类型,分别为膀胱横纹肌肉瘤、膀胱小细胞癌、膀胱平滑肌肉瘤、膀胱恶性淋巴瘤、膀胱恶性纤维组织细胞瘤、膀胱脂肪肉瘤、膀胱黑色素瘤;良性11例(占39．3％),有4种病理类型,分别为膀胱海绵状血管瘤、膀胱壁纤维瘤、膀胱平滑肌瘤、膀胱嗜铬细胞瘤。11例良性肿瘤均完整切除或电灼、电切。17例恶性肿瘤中,膀胱部分切除术7例、膀胱全切除术9例、无法切除1例,有7例恶性肿瘤因复发多次行手术切除。17例恶性肿瘤患者均获随访,3年存活率47．0％(8／17)。结论 膀胱非上皮性肿瘤临床少见,病理类型复杂,恶性居多且预后较差,良性肿瘤预后较好。术前诊断率低,膀胱镜下深部活检可提高诊断率。手术是该病的主要治疗方法。良性肿瘤应完整切除,恶性肿瘤应争取广泛切除,结合其病理特点辅助放化疗可能提高疗效。     

Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.

A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.     

Dumbbell-shaped nonpsammomatous malignant melanotic schwannoma of the cervical spinal root

Background contextMelanotic schwannoma is a very rare tumor of Schwann cell origin, which can develop in various locations, similar to conventional schwannoma. This tumor has a malignant potential and therefore careful therapy is required.PurposeTo describe a case of melanotic schwannoma with a histopathologically and clinically malignant behavior.Study designCase report.MethodsA 64-year-old man presented with sensory changes in his arm and gait disturbance. Magnetic resonance imaging revealed a dumbbell-shaped tumor at the left C7 spinal root, which was hyperintense on T1-weighted images and generally hypointense on T2-weighted images in comparison with conventional schwannoma; however, the peripheral zone was relatively hyperintense, and the central zone was hypointense like a target sign.ResultsThe tumor was partially resected and diagnosed to be nonpsammomatous malignant melanotic schwannoma. The patient experienced local recurrence and metastases to the bone and lung and finally developed quadriplegia. Radiation therapy failed to palliate the symptoms.ConclusionsSome melanotic schwannomas present with an aggressive behavior, which thus leads to poor prognosis. We should therefore be familiar with its characteristic clinical imaging and pathologic findings to provide a correct diagnosis and appropriate treatment for such patients.     

Superficial Malignant Schwannoma of the Scalp

BACKGROUND: Malignant schwannoma is a rare sarcoma that is located mostly on trunk and extremities; the head and neck are unusual sites for its development. Almost half of these tumors arise from neurofibromas with or without von Recklinghousen's disease, and most of the remainder develop de novo from peripheral nerve trunks. Development in the superficial soft tissues is extremely rare. OBJECTIVE: To present a case of superficial malignant schwannoma of the scalp, which is an uncommon presentation. METHODS: An 80-year-old male patient presented with a painless ulceration on his scalp. The clinical and radiologic properties of the tumor were consistent with a skin tumor. RESULTS: The lesion was excised, and reconstruction was achieved by transposition scalp flap. Malignant schwannoma was given as diagnosis by histopathologic examination with immunohistochemistry. CONCLUSION: Malignant schwannomas may also occur in the skin. Clinically, these superficial forms closely resemble skin tumors, especially malignant melanoma. The treatment of choice in these tumors is wide resection combined with radiotherapy.