peripheral nerve sheath tumors

  • 文章类型: Journal Article
    本研究报告了一名24岁健康女性中附于下直肌(IR)肌腱的孤立性神经纤维瘤,并回顾了有关临床表现的相关文献。诊断,以及这种罕见肿瘤的治疗。患者接受了成功的肿瘤手术切除,导致相关症状(左下眼睑突出和发红)的解决。病理检查根据特征性组织病理学和免疫组织化学标记证实了神经纤维瘤的诊断。该病例报告强调了孤立性神经纤维瘤以及眼眶和眼附件的原发性肿瘤的罕见性。我们还讨论了源自眼眶和眼附件的孤立性神经纤维瘤的背景。通过手术切除成功处理此病例凸显了准确诊断和量身定制治疗策略的重要性。据我们所知,这是首次报道的仅局限于IR肌腱的孤立性神经纤维瘤.
    The present study reports a case of solitary neurofibroma attached to the Inferior Rectus (IR) muscle tendon in a 24-year-old healthy woman and reviews the relevant literature regarding the clinical presentation, diagnosis, and management of this uncommon tumor. The patient underwent successful surgical resection of the tumor, leading to the resolution of associated symptoms (left lower eyelid protrusion and redness). Pathological examination confirmed the diagnosis of neurofibroma based on characteristic histopathological and immunohistochemical markers. This case report underscores the rarity of solitary neurofibromas and primary neoplasms of orbit and ocular adnexa. We also discuss the background of solitary neurofibromas originating from orbit and ocular adnexa. The successful management of this case through surgical resection highlights the importance of accurate diagnosis and tailored treatment strategies. To the best of our knowledge, this is the first reported solitary neurofibroma confined solely to the IR tendon.
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  • 文章类型: Case Reports
    恶性triton肿瘤(MTT)是恶性周围神经鞘瘤(MPNST)的亚型,由周围神经或神经纤维瘤的Schwan细胞发展而来,并显示横纹肌母细胞分化。是一种罕见的软组织肿瘤,预后较差。
    我们报告了一名46岁男性患者的右肩恶性Triton肿瘤(MTT)病例,该患者于2018年6月在侯赛因国王医疗中心皇家康复中心的肌肉骨骼肿瘤诊所就诊。
    患者主诉8个月的进行性右肩疼痛和肩后外侧区肿胀。在这种情况下,准确的诊断至关重要,包括X线和磁共振成像(MRI)在内的研究表明,软组织肿瘤累及右肩区域,从而对侵袭性软组织肿瘤进行了鉴别诊断,并制定了开放切开活检的计划,以组织病理学报告为一例。恶性Triton肿瘤是一种非常罕见且侵袭性的肉瘤,起源于周围神经鞘,因为它是恶性周围神经鞘肿瘤的亚型,此后对整个肿瘤进行了辅助切除,并进行了安全化疗。
    选择的治疗方法是广泛的肿瘤切除,然后进行化疗和/或放疗,以提高5年生存率。
    UNASSIGNED: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis.
    UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
    UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
    UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
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  • 文章类型: Journal Article
    颈部混合神经鞘瘤/神经纤维瘤并不常见,分类为周围神经鞘瘤(PNSTs)。PNST从软组织发展而来,其中神经鞘瘤,神经鞘瘤,和神经纤维瘤是最常见的。混合PNST由1种以上的PNST组成,如混合神经鞘瘤/神经纤维瘤。由于对该主题的研究有限,这些肿瘤的确切流行病学和发病机理仍在很大程度上未知。这种肿瘤可以扩散到软组织上,尽管大多数病例报道涉及皮下层或真皮。一些研究表明,混合神经鞘瘤/神经纤维瘤可能与神经纤维瘤病有关。我们介绍了一例51岁的女性患者,通过组织病理学和免疫组织化学诊断为颈部混合神经鞘瘤/神经纤维瘤。患者被转诊到神经内科进行神经纤维瘤病筛查,报告阴性结果。12个月后,患者没有肿瘤复发的证据.
    Neck hybrid schwannoma/neurofibromas are uncommon and classified as peripheral nerve sheath tumors (PNSTs). PNSTs develop from soft tissues, of which schwannoma, perineurioma, and neurofibroma are the most common. Hybrid PNSTs consist of more than 1 type of PNST, such as hybrid schwannoma/neurofibromas. The exact epidemiology and pathogenesis of these tumors are still largely unknown because of the limited studies on this topic. Such tumors can spread over the soft tissues, although most cases reported involve the subcutaneous layer or dermis. Some studies have suggested that hybrid schwannoma/neurofibromas may be associated with neurofibromatosis. We present a case of a 51-year-old female patient with a neck hybrid schwannoma/neurofibroma diagnosed by histopathology and immunohistochemistry. The patient was referred to the neurology department for neurofibromatosis screening, which reported negative results. After 12 months, the patient showed no evidence of tumor recurrence.
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  • 文章类型: Journal Article
    原发性心脏神经鞘瘤(PCS)是由雪旺氏细胞产生的神经源性肿瘤。恶性神经鞘瘤(MSh)是一种侵袭性癌症,占所有肉瘤的2%。关于这些肿瘤的适当管理的信息是有限的。在四个数据库中搜索病例报告/PCS系列。主要结果是总生存期(OS)。次要结果包括治疗策略和相应结果。在439项可能符合条件的研究中,53符合纳入标准。纳入的患者为43.72±17.76岁,28.3%为男性。超过50%的患者患有MSh,9.4%也表现出转移。神经鞘瘤通常发生在心房(66.0%)。左侧PCS比右侧PCS更常见。几乎90%的病例进行了手术;16.9%和15.1%的病例使用了化疗和放疗,分别。与良性病例相比,MSh发生在较年轻的年龄,通常位于左侧。整个队列在1年和3年的OS为60.7%,54.0%,分别。女性和男性OS相似,随访2年。手术与较高的OS相关(p<0.01)。手术是良性和恶性病例的主要治疗选择,并且是与生存率相对改善相关的唯一因素。
    Primary cardiac schwannoma (PCS) is a neurogenic tumor that arises from Schwann cells. Malignant schwannoma (MSh) is an aggressive cancer comprising 2% of all sarcomas. Information on the proper management of these tumors is limited. Four databases were searched for case reports/series of PCS. The primary outcome was overall survival (OS). Secondary outcomes included therapeutic strategies and the corresponding outcomes. Among 439 potentially eligible studies, 53 met the inclusion criteria. The patients included had 43.72 ± 17.76 years and 28.3% were males. Over 50% of patients had MSh, with 9.4% also demonstrating metastases. Schwannoma commonly occurs in the atria (66.0%). Left-sided PCS were more common than right-sided ones. Surgery was performed in almost 90% of the cases; chemotherapy and radiotherapy were used in 16.9% and 15.1% of cases, respectively. Compared to benign cases, MSh occurs at a younger age and is commonly located on the left side. OS of the entire cohort at 1 and 3 years were 60.7%, and 54.0%, respectively. Females and males OS were similar up to 2 years follow-up. Surgery was associated with higher OS (p < 0.01). Surgery is the primary treatment option for both benign and malignant cases and was the only factor associated with a relative improvement in survival.
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  • 文章类型: Review
    神经鞘瘤是最常见的周围神经鞘瘤之一。这些肿瘤,其特征是缓慢生长和封装,可以单独或多种形式发生。虽然它们通常偶尔发生,它们可以在各种遗传性肿瘤易感性综合征中看到,例如2型神经纤维瘤病(NF-2)或神经鞘瘤病。然而,神经鞘瘤病是一种相对罕见的疾病。我们介绍了一例22岁的坐骨神经节段性神经鞘瘤病患者,并进行了全面的文献综述。
    Schwannomas are one of the most common peripheral nerve sheath neoplasms. These tumors, which are characteristically slow-growing and encapsulated, can occur in solitary or multiple forms. Although they usually occur sporadically, they can be seen with various genetic tumor predisposition syndromes such as neurofibromatosis type 2 (NF-2) or schwannomatosis. However, schwannomatosis is a relatively rare disease. We present a case of a 22-year-old patient with segmental schwannomatosis of the sciatic nerve and a comprehensive literature review.
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    文章类型: Case Reports
    Schwannomas or neurilemmomas are benign and slow-growing tumors that arise exclusively from Schwann cells in peripheral nerve sheaths. These neoplasms theoretically can occur anywhere in the body, but they most frequently affect extremities, as well as head and neck region. However, their presentation in the abdominal wall is extremely rare and only few cases have been reported in the literature. Subcutaneous lesions may be asymptomatic and only incidentally discovered upon physical examination or imaging. However, occasionally they induce mass effects on surrounding large nerves. We present the case of a 34-year-old man with abdominal wall pain localized in the right iliac fossa and palpable subcutaneous mass. Ultrasound and CT scan revealed a solid well-defined mass of the abdominal wall. Following surgical excision under general anesthesia, histological examination was consistent with the diagnosis of benign schwannoma.
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  • 文章类型: Journal Article
    Schwannomas are the most frequent peripheral nerve sheath tumors and are treated by surgical resection when symptomatic. Tumor removal is performed by intraneural dissection and enucleation. In order to safely remove the tumor from the nerve, the use of sodium fluorescein has recently been proposed to distinguish the tumor from the adjacent normal nerve fibers, before incision of the tumor pseudocapsule and during intraneural tumor dissection.
    We report a consecutive case series of 5 peripheral nerve schwannomas operated in 4 patients, in which we evaluate the usefulness of sodium fluorescein compared to usual visual landmarks, at each step of the surgical procedure.
    After exposition of the schwannoma, sodium fluorescein helped with the localization of intracapsular en passant nerve fascicles in only one case. Hence, the definition of a safe entry zone for capsular incision relied mainly on nerve monitoring and direct visualization of en passant nerve fascicles under microscope. During intraneural dissection, there was a sharp contrast between the fluorescent tumor and the non-fluorescent adjacent pseudocapsule in most cases but the colorimetric variation between tumor and normal tissue induced by fluorescence did not outperform the natural contrast between the yellow true capsule and the gray-red layers of the pseudocapsule.
    Based on these results, we consider that the limited additional value of sodium fluorescein in primary peripheral nerve schwannoma surgery does not warrant its use in daily clinical practice. Additional studies are needed to assess its usefulness during the surgery of recurrences and tumors which are intertwined with several fascicles of origin such as neurofibromas.
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