Intraosseous neurilemmoma of the proximal ulna

INTRODUCTION

Neurilemmoma is a benign nerve sheath neoplasm commonly located in the soft tissue. Intraosseous neurilemmoma is rare, constituting less than 1% of primary bone tumors.

PRESENTATION OF CASE

A 21 year-old woman was presented with left elbow pain of 1-month duration. Plain radiographs showed a well-defined, lytic and expansile lesion of the proximal ulna. Computed tomography revealed cortical destruction and soft tissue extension. Because the tissue of origin for the tumor was uncertain, an open biopsy was performed. The specimens demonstrated a benign spindle cell tumor suggestive of a neurilemmoma, similar to a soft tissue neurilemmoma. The diagnosis of intraosseous neurilemmoma was established. Marginal excision of the soft tissue component and curettage of the lesion in the bone were performed. After 3.5 years of follow up, there is no clinical or radiographic finding to suggest any recurrence.

DISCUSSION

The major site of intraosseous neurilemmoma is the mandible. Occurrence in the long bone is particularly rare. Only two cases of intraosseous neurilemmoma involving the bones around the elbow have been reported to our knowledge; these cases arose in the distal humerus. We describe the first case of intraosseous neurilemmoma of the proximal ulna of the left elbow. The recommended treatment is conservative resection and bone grafting, as malignant change is extremely rare.

CONCLUSION

Although very rare, intraosseous neurilemmoma should be taken under consideration in the differential diagnosis of painful, radiographically benign-appearing osseous tumor around the elbow.     

Solitary cellular schwannoma (neurilemmoma) showing malignant changes: evaluation through magnetic resonance imaging (M.R.I.), surgical intervention, and histopathology

Schwannoma (neurilemmoma) are common benign tumors arising from the peripheral nerve sheath. Malignant transformation is uncommon. A unique case showing such a transformation is reported highlighting the roles of magnetic resonance imaging, surgical intervention, and histopathology. The case was thoroughly investigated by learning the details of the sequence of events leading to the current status. The evaluation was made through magnetic resonance imaging. In addition, computed tomography and conventional radiography were used to locate any foci of calcification. Subsequently, the tumor's gross and microscopic morphology was defined by surgical intervention and histopathology. Malignant schwannoma of the left leg occupying the entire calf is extremely uncommon. Only ten cases have been reported thus far, including the current one from the Indian subcontinent. Malignant transformation in a schwannoma differs significantly from malignant nerve sheath tumors (erroneously called malignant schwannomas). An endeavour has been made to differentiate malignant transformation in schwannoma from other malignant peripheral nerve sheath tumors. An innovation in this direction is magnetic resonance imaging. This investigate procedure is imperative in such situations, along with surgery and histopathology, which may also help in classifying the condition.     

颈1～2哑铃形神经鞘瘤的手术治疗

目的探讨颈1～2哑铃形神经鞘瘤的手术治疗及其效果。方法 2005年至2011年共收治颈1～2哑铃形神经鞘瘤患者16例,均采用颈后中线或倒钩形切口手术治疗,先从囊内分块切除肿瘤,再分离肿瘤包膜和神经根,最后探查硬脊膜下肿瘤。结果 16例肿瘤均全切除,术后症状均有不同程度改善,无新发神经功能障碍。随访3个月至5年未见肿瘤复发。结论颈1～2哑铃形神经鞘瘤可采用颈后中线或倒钩形入路手术,该方法简单易行,效果良好。     

Immunohistochemical study of GLUT-1 in oral peripheral nerve sheath tumors

Aim:  To investigate the immunoexpression and diagnostic applicability of human erythrocyte-type glucose transporter protein (GLUT-1) in oral peripheral nerve sheath tumors.
Material and methods:  Specimens diagnosed as oral peripheral nerve sheath tumors archived in the Oral Pathology Service of Universidade Federal de Minas Gerais from 1966 to 2006 were evaluated. Thirty-four lesions were included: 15 traumatic neuromas, 11 neurofibromas, four neurilemmomas, and four malignant peripheral nerve sheath tumors (MPNST). One case of neurofibroma was associated with neurofibromatosis type I. Immunohistochemistry for S-100 and GLUT-1 was performed. S-100 was immunopositive in all lesions.
Results:  Benign lesions were immunopositive for GLUT-1 except in two (18.2%) cases of neurofibromas. In the traumatic neuroma, the perineuriums were immunopositive for GLUT-1. In the neurofibroma, the immunoreactivity was heterogeneous. Immunopositivity was observed at levels of 54.5% in the periphery of the lesion, 9.1% in the center, and 18.2% in both. The neurilemmoma demonstrated immunopositivity in the capsule. One case (25%) of MPNST presented GLUT-1 positive stain in occasional cells distributed homogeneously in all the tumor area.
Conclusion:  GLUT-1 is a useful marker for perineurial cells and should be included in the oral peripheral nerve sheath tumors immunophenotyping thus aiding in the correct diagnosis of these lesions.     

Expression of the CXCL12/SDF-1 Chemokine Receptor CXCR7 in Human Brain Tumours

Purpose: Receptor 7 (CXCR7) has recently been characterized as a novel receptor for CXCL12/SDF-1(stromal cell derived factor-1). Given the demonstrated importance of CXCL12/SDF-1 in angiogenesis and tumourmetastasis, we hypothesized that CXCR7 may also play a role in tumour pathogenesis. Located in the limitedspace of the intracranial cavity, any brain tumours can be inherently serious and life-threatening. However, theexpression of CXCR7 in pituitary adenoma, neurilemmoma or hemangioblastoma remains to be elucidated.Therefore, we aimed to determine the potential contribution of CXCR7 in the development of brain tumours.Methods: In this study we examined and quantified the mRNA expression of CXCR7 in four different humanbrain tumours - 27 patients with neurilemmoma (8 patients), pituitary adenoma (7 patients), hemangioblastoma(6 patients), or meningioma (6 patients) undergoing surgical resection in the West China Hospital of SichuanUniversity. There were 15 females and 12 males aged from 28 to 70 years old. Total RNA was isolated and mRNAwas measured by quantitative real-time RT-PCR. One-way analysis of variance (ANOVA) was performed usingSPSS 11.0 statistical software to compare the mRNA levels of CXCR7 among four groups. Results: We foundthat CXCR7 mRNA was detected in all tumour samples. Quantitative results showed that the levels of CXCR7mRNA in brain tissues from patients with neurilemmoma or meningioma were significantly higher than thosewith pituitary adenoma or hemangioblastoma. Conclusions: The results suggest that the CXCR7 may play arole in progression, metastasis and angiogenesis of brain tumours.     

腮腺内面神经鞘瘤6例临床分析

目的 分析探讨腮腺内面神经鞘瘤的临床特点和治疗要点。方法 回顾分析1992—2010年常州市第一人民医院收治的6例面神经鞘瘤患者的临床特点、影像学表现、诊断及治疗。结果 6例患者术前2例确诊。术后5例均有不同程度的面瘫症状。随访有2例为面神经功能Ⅲ级和Ⅳ级,其余患者面神经功能均恢复。所有病例随访未发现肿瘤复发。结论 腮腺区面神经鞘瘤是来源于神经鞘膜的神经源性良性肿瘤,临床罕见,易误诊、误治。保留面神经结构和功能极其重要。     

肾上腺神经鞘瘤的临床特点与诊治(附4例报道)

目的探讨肾上腺神经鞘瘤的临床、影像学及病理学特点,以指导临床诊断和治疗.方法回顾性分析4例肾上腺神经鞘瘤患者的临床资料,结合文献报道的40例肾上腺神经鞘瘤的临床资料进行分析,研究该疾病的临床特点.结果本组4例,均在体检时发现肾上腺占位,实验室检查无异常.手术切除后病理证实为神经鞘瘤.结论肾上腺神经鞘瘤较为少见,且缺乏特异性的临床表现.治疗选择为手术切除.     

神经母细胞瘤与腰椎神经鞘瘤误诊并文献分析

目的:探讨神经母细胞瘤与腰椎神经鞘瘤容易出现误诊的原因,提高诊治的针对性及有效性。方法:对二例误诊为腰椎间盘突出症的肿瘤患者以往手法等诊疗过程进行反思并结合文献进行回顾性分析。结果:多以神经根痛为首发症状,继而出现感觉、运动及植物神经功能障碍;腰穿显示椎管梗阻,脊髓腔造影表现不典型,应仔细询问病史,认真查体,结合临床症状与影像学检查,可有效降低误诊率,反之是引起肿瘤常见的误诊的原因。结论:神经母细胞瘤临床少见,脊椎神经鞘瘤临床特征无特异性,明确诊断依赖于脊髓造影及MRI检查,确诊需病理检查,因误诊、漏诊率高,早期诊断是改善预后的前提。手术可完整切除肿瘤及其宿主神经,早期手术为二种肿瘤有效的治疗方法。     

PBM 血液管理在骶骨神经鞘瘤切除术中的应用

目的 探讨骶骨神经鞘瘤切除术术中血液管理的方案。方法 回顾性分析 2012 年 9 月至 2016 年 12 月首都医科 大学附属北京世纪坛医院 12 例骶骨神经鞘瘤的血液管理方案,探讨骶骨神经鞘瘤手术血液管理方案在术前准备、术中血 压监测、血液丢失及液体输入管理和血管活性药物使用等方面的经验。结果 12 例骶骨神经鞘瘤均行囊内刮除,1 例患者 切除了患者S1 神经根,出现足下垂神经功能障碍,其余11 例患者均成功从瘤体将神经游离,保留神经,1 例患者术后 出现脑脊液漏,经过换药及对症治疗治愈,随访平均13 个月,神经鞘瘤无复发。术中出血量（1,500±4,670）ml,平均 异体输血使用量（1,567±247）ml。通过实施血液管理医疗策略,术中循环维持稳定,所有患者均在术后l 小时内停用血 管活性药物并拔除气管导管,送返病房。结论 血液管理方案包括升红细胞药物及贮存式自身输血是切除骶骨神经鞘瘤减 少出血的有效手段。     

Giant Schwannoma of the First Metatarsal: A Rare Entity

Schwannomas of osseous origin are rare, and schwannomas of the short tubular bones are even rarer. These benign-looking tumors are difficult to diagnose using imaging alone. However, histopathologic evaluation of a biopsy specimen can establish the diagnosis by identifying Antoni type A and B zones. Curettage and bone grafting will probably be adequate for treatment because malignant changes are unlikely. Large lesions can require en bloc excision and reconstruction. We describe what appears to be only the second case of a schwannoma in the first metatarsal of the foot in a 48-year-old woman. The lesion was poorly contained, with obvious breaks in the cortical shell. The diagnosis was confirmed by pathologic analysis. The lesion was successfully treated with en bloc resection and reconstruction with a nonvascularized fibular graft.