颌骨内神经鞘瘤:1例报告及文献复习

神经鞘瘤是来源于神经鞘膜的肿瘤,多见于软组织,骨内神经鞘瘤并不常见。本文报告1例以下颌骨肿大为主要表现的骨内神经鞘瘤,通过该患者的临床表现、影像学检查、术中所见、病理诊断和术后随访情况。结合相关文献将该病的病因、临床表现、影像学表现、组织病理学特点、治疗方法及预后进行系统性的分析和总结,旨在为临床早期诊断、早期治疗该病时提供参考。     

舌恶性外周神经鞘膜瘤1例

恶性外周神经鞘膜瘤是一种罕见的神经源性恶性肿瘤，其临床症状和影像学表现不典型，诊断困难，恶性程度高，预后较差。恶性外周神经鞘膜瘤通常发生在躯干，约20%发生在头颈部，发生于口腔内者罕见。本文报道1例舌部恶性外周神经鞘膜瘤病例，并结合文献复习，对恶性外周神经鞘膜瘤的临床特点、诊断和治疗进行总结，为该疾病的诊治提供参考。     

下颌骨骨内神经鞘瘤:1例报告及文献复习

神经鞘瘤是来源于神经髓鞘的良性肿瘤,多见于头颈部软组织.骨内神经鞘瘤较为罕见,但以下颌骨相对常见.本文报告1例首发表现为下颌下肿块的下颌骨多发性骨内神经鞘瘤,详细描述其临床特征、影像学表现、手术所见、病理结果以及术后随访情况,并回顾国内外文献将其好发因素、临床表现、影像学表现、组织病理学表现以及治疗情况和预后作了系统性总结和分析.大多数骨内神经鞘瘤临床表现一般为进行性无症状性下颌骨膨胀,也可表现为颌面畸形,牙移位或拥挤.     

口腔颌面部恶性外周神经鞘膜瘤：1例报告及文献复习

恶性外周神经鞘膜瘤（MPNST）是一种较为少见的恶性肿瘤,头颈部尤为少见。本文报告1例口腔颌面部MPNST患者,并结合国内外相关文献,探讨其临床表现、组织病理学及影像学特点、治疗及预后。MPNST是一种恶性程度较高的恶性肿瘤．手术治疗复发率较高．局部复发和远处转移是导致死亡的主要原因。     

颌骨內神经鞘瘤11例临床分析

目的回顾分析11例颌骨内神经鞘瘤的临床资料,旨在提高对疾病的诊断和治疗水平。方法对1993年1月～2002年2月间经治的11例颌骨内神经鞘瘤患者,从临床、病理、免疫组化、影像学检查及治疗手段等方面进行回顾分析。8例良性者行彻底刮治加烧灼术;2例下颌骨恶性者行扩大切除加颈淋巴清扫术;1例上颌骨恶性伴神经纤维瘤病患者行上颌骨扩大切除加中厚皮片修复及颈淋巴清扫术,术后辅以放疗、化疗。结果5例位上颌骨(1例恶性),6例位下颌骨(2例恶性);其X线表现有一定特异性,术后除1例上颌骨恶性者于11个月时死于广泛多发性转移外,其他10例随访7～122月无复发。结论颌骨内神经鞘瘤临床上少见。CT、MRI及穿刺细胞学检查有助于该病的早期诊断;手术仍是最有效的治疗手段,预后一般良好;辅助放疗、化疗与否,尤其是伴发神经纤维瘤病患者无多大价值。     

颌骨内神经鞘瘤11例临床分析

目的 回顾分析11例颌骨内神经鞘瘤的临床资料，旨在提高对疾病的诊断和治疗水平。方法 对1993年1月--2002年2月间经治的11例颌骨内神经鞘瘤患者，从临床、病理、免疫组化、影像学检查及治疗手段等方面进行回顾分析。8例良性者行彻底刮治加烧灼术；2例下颌骨恶性者行扩大切除加颈淋巴清扫术；1例上颌骨恶性伴神经纤维瘤病患者行上颌骨扩大切除加中厚皮片修复及颈淋巴清扫术，术后辅以放疗、化疗。结果 5例位上颌骨(1例恶性)，6例位下颌骨(2例恶性)；其X线表现有一定特异性，术后除1例上颌骨恶性者于11个月时死于广泛多发性转移外，其他10例随访7—122月无复发。结论 颌骨内神经鞘瘤临床上少见。CT、MRI及穿刺细胞学检查有助于该病的早期诊断；手术仍是最有效的治疗手段，预后一般良好；辅助放疗、化疗与否，尤其是伴发神经纤维瘤病患者无多大价值。     

下颌骨恶性神经鞘膜瘤1例治疗体会

恶性神经鞘膜瘤（Matignant peripheral nerve sheathtumor．MPNST）来源于外周神经。神经鞘膜瘤或神经纤维瘤多发于四肢和躯干，头颈部较少见，颌骨内尤为罕见。我科曾收治1例下颌骨恶性神经鞘膜瘤患者，现报道如下。     

下颌骨恶性神经鞘膜瘤1例报告

恶性神经鞘膜瘤（malignant peripheral nerve sheath tumor,MPNST)来源于外周神经,神经鞘膜或神经纤维瘤,它多发于四肢和躯干,头颈部较少见,颌骨内尤为罕见。四川大学华西口腔医院曾收治1例下颌骨恶性神经鞘膜瘤患者,现报道如下。     

34例下颌骨中心性癌临床、病理及预后分析

目的： 回顾分析下颌骨中心性癌的临床、病理以及预后特点。方法： 纳入2010—2015年于我院口腔颌面-头颈肿瘤科接受手术治疗的下颌骨中心性癌患者的基本信息、临床治疗信息、影像学信息、病理学信息、随访记录,采用SPSS17.0软件包对数据进行统计学分析。结果： 最终纳入36例患者,其中34例随访信息完整。随访患者中,男22例,女12例;年龄31~60岁23例,>60岁11例;有既往吸烟史13例,有既往酗酒史4例。瘤体最大直径中位值为4 cm,瘤体最大直径≤4 cm 21例,瘤体最大直径>4 cm 13例。病理报告提示9例存在同期淋巴结转移,所有术区切缘均为阴性。28例接受术后放疗,9例在随访期间死于肿瘤局部复发或肿瘤远处转移。结论： 手术是治疗下颌骨中心性癌的主要措施。瘤体位于下颌骨体部以外,同期肿瘤淋巴结转移的患者具有较高的术后肿瘤局部复发几率。既往酗酒史对肿瘤术后远处转移有一定促进作用,瘤体最大直径>4 cm的患者,总体预后较差。     

下颌骨神经鞘瘤1例

神经鞘瘤在临床上以全身软组织多见,但骨内神经鞘瘤较罕见。本文报道发生于右下颌骨内的神经鞘瘤1例,并结合相关文献探讨下颌骨神经鞘瘤的临床特点、诊断、治疗及预后。     

Malignant peripheral nerve sheath tumor of mandible

Malignant peripheral nerve sheath tumor is a common tumor that rarely affects the head and neck region. The patient presented in this report is a teenage girl presented with a lesion in the right body of the mandible with severe disfigurement of the lower face. The lesion was first histopathologically diagnosed as embryonal rhabdomyosarcoma. After excision, however, the histopathology report proved the diagnosis of malignant peripheral nerve sheath tumor.     

Malignant peripheral nerve sheath tumor of the maxilla.

The term malignant peripheral nerve sheath tumor (MPNST) refers to most spindle cell sarcomas arising from nerve or neurofibroma or showing nerve sheath differentiation and has been recently adopted by WHO. Malignant tumors of the peripheral nerve sheath are most commonly seen in association with the neurofibroma or at the site of irradiation and may also occur de novo. Its diagnosis has been called "one of the most difficult and elusive diagnosis in soft tissues diseases". We present a rare case of MPNST in a 12-year-old girl, who presented with a swelling of left cheek region of 2 months duration. Intraorally, the swelling extended buccally and palatally from premolar to tuberosity region. Swelling was fixed and firm to hard in consistency. A clinical diagnosis of odontogenic sarcoma was made. It was surgically excised and the histological features were suggestive of malignant peripheral nerve sheath tumor of the maxilla.     

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.     

Surgical management of recurrent malignant schwannoma of the scalp

Localization of malignant schwannoma in the head and neck region is rare. Malignant schwannoma is a highly aggressive, slowly growing, encapsulated tumor of nerve sheath origin. The most common presentation of this tumor is a subcutaneous progressively enlarging mass with or without neurologic symptoms. These tumors arise from neurofibromas with or without von Recklinghausen disease, and most of the remains develop from peripheral nerve trunks. We report a case with recurrent malignant schwannoma of the scalp that is an uncommon presentation and discuss the clinicopathologic properties of this tumor.     

Reconstruction of an Extensive Midfacial Defect Using Additive Manufacturing Techniques

Malignant peripheral nerve sheath tumors are extremely rare tumors arising in peripheral nerves. Only 17 cases involving the trigeminal nerve have ever been reported. These tumors have a very poor prognosis and very high rates of recurrence and metastases. Their recommended treatment involves complete tumor resection followed by radiation. This can be problematic in the head and neck region. We present a clinical case involving a 33‐year‐old female patient presenting with a slow‐growing, exophytic mass of the anterior maxilla. Incisional biopsy and subsequent histological examination revealed a diagnosis of a malignant peripheral nerve sheath tumor. Surgical resection involved a complete maxillectomy, rhinectomy, and resection of the upper lip and aspects of the left and right cheeks. Reconstruction of the subsequent defect incorporated the placement of four zygomatic oncology implants to aid in retention of a facial prosthesis. These implants, however, were subsequently lost; and an anatomical model of the hard tissues was manufactured via 3D printing. This model was used to design and manufacture a titanium frame (customized implant) for the patient. The frame was then fixated and secured intraoperatively with 21 cortical screws. A maxillary denture and silicone facial prosthesis were also made to fit onto this frame. This is the first known case where additive manufacturing, via the use of rapid prototyping and 3D printing, was employed to manufacture a facial prosthesis.     

Schwannoma of the mental nerve: usefulness of preoperative imaging: a case report

Schwannoma of the head and neck region is relatively uncommon. We report a case of schwannoma arising from the left mental nerve. A 21-year-old male presented at our hospital with a painless swelling of the left cheek. Because the lesion had rapidly increased in size, a malignant tumor was suspected. Magnetic resonance imaging (MRI), computed tomography, and ultrasonography were performed. MRI and ultrasonography revealed that the tumor was connected to the mental nerve. Both MRI and ultrasonography were found to be useful in making a preoperative diagnosis. It was possible to determine prior to surgery that this was a peripheral nerve sheath tumor. The lesion was completely resected. The pathological diagnosis was Antoni type A schwannoma. There has been no sign of recurrence 1 year after surgery.     

An intraosseous malignant peripheral nerve sheath tumor in the mandible of a patient with neurofibromatosis type 1

Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas that originate in peripheral nerves or neurilemma cells. Here, we report an extremely rare case of an intraosseous MPNST in the mandible of a patient with neurofibromatosis type 1 (NF1). A 57-year-old woman with a history of NF1 was referred to our hospital because she had abnormal sensations at her left mandible. She was diagnosed with MPNST and underwent radical resection, although local recurrence and multiple metastases were detected during follow-up. Despite receiving palliative radiotherapy, the patient died at 13 months after the initial diagnosis.     

Unusual intramaxillary plexiform schwannoma

Background

Neoplasms of peripheral nerve in the head and neck region are of common occurrence, but origin in the oral and para-oral tissues is uncommon and they rarely occur centrally within the jaws. Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but its occurrence within the jaw bones is most unusual. Plexiform schwannoma is a unique variant of Schwann cell tumours having plexiform pattern. Literature revealed only one case of plexiform schwannoma of the jaw bones, i.e. involving the mandible.

Case report

In this report, we present the first documented case of intraosseous plexiform schwannoma of the maxilla, an extremely rare benign neurogenic tumour treated surgically.

Discussion

Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but to occur within the jaw bones is exceptional. Plexiform schwannoma is a rare variant of Schwann cell tumour having plexiform pattern of intraneural growth with multinodularity. Plexiform schwannoma is a benign neoplasm with no malignant potential, but recurrences are evident if excised incompletely. Plexiform schwannoma has similar clinical and histopathological features as that of plexiform neurofibroma which has high malignant potential; hence, it is imperative to correctly diagnose and differentiate this lesion as treatment modality of these two lesions differs.     

Primary malignant peripheral nerve sheath tumour in the temporalis muscle.

INTRODUCTION: Primary malignant neoplasms of nerve sheath origin are rare, highly aggressive tumours that account for no more than 5% of all soft tissue sarcomas. In the head and neck where the great majority of peripheral nerve neoplasms are benign, as in the rest of the body, malignant peripheral nerve sheath tumours (MPNSTs) are unusual lesions. PATIENT: A case of a large MPNST is reported, which originated in the left temporalis muscle of a 74-year-old man who did not suffer from von Recklinghausen's disease. It had been treated as an abscess for a long time. DISCUSSION: The clinicopathologic properties of this neoplasm have been widely discussed and the emphasis is on the need for its early identification since the tumour's non-specific symptoms may result in delayed diagnosis and treatment. CONCLUSION: To date, to our knowledge, no case of MPNST located in the temporalis muscle has been described. The rarity of the tumour along with its clinical appearance and the vague symptoms may result in delayed referral and thus the tumour being quite large when diagnosed.