Non-ossifying fibroma

非骨化性纤维瘤
  • 文章类型: Case Reports
    背景:Jaffe-Campanacci综合征是一种罕见的综合征,以多个非骨化性纤维瘤(NOF)和咖啡色斑块为特征。这个名字是1982年由Mirra在Jaffe于1958年首次描述此案之后创造的。尽管提示与1型神经纤维瘤病有关,但对于Jaffe-Campanacci综合征是否是1型神经纤维瘤病(NF-1)的亚型或变体仍未达成共识。
    方法:在本文中,我们介绍了2例患者的病例系列。第一例是一名13岁男性,患有Jaffe-Campanacci综合征,表现为股骨远端骨折。他的父亲具有Jaffe-Campanacci综合征和NF-1的积极特征,而他的姐姐只有NF-1的特征,因此我们提出了两者。
    结论:Jaffe-Campanacci与1型神经纤维瘤病有明确的关系,这仍然需要基因建立。由于长骨的几个大的非骨化纤维瘤的存在,它与病理性骨折的显著风险有关。我们同意以前的作者,应该对所有新诊断的1型神经纤维瘤病患者进行骨筛查,识别非骨化性纤维瘤并评估病理性骨折的可能性。此外,NF-1患者的兄弟姐妹应筛查可能携带高病理性骨折风险的多个NOF。
    BACKGROUND: Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it\'s suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1).
    METHODS: In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both.
    CONCLUSIONS: Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures.
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  • 文章类型: Journal Article
    颌骨的非骨化性纤维瘤(NOF)很少见。虽然NOF是最常见的长骨良性骨肿瘤,具有病理放射学特征,并具有自回归的趋势。颌骨NOF的大小似乎相对较大,行为更积极。一名16岁女性患者报告面部右侧无痛性肿胀4个月。影像学分析显示下颌骨直角的单眼射线可透病变,边缘不明确,皮质穿孔和下边界变薄。暂时诊断为牙源性角化囊肿/单囊性成釉细胞瘤,并进行了切开活检。组织病理学特征和免疫组织化学特征有利于NOF的诊断。切除并重建病变。切除的标本证实了诊断。随访18个月无复发迹象。在颌骨的单/多房性放射性的鉴别诊断中,尤其是后下颌骨,应考虑NOF。
    Non-ossifying fibroma (NOF) of jaw bones are rare. While NOF is the most common benign bone tumor of long bones with pathognomonic radiological features and bear a tendency for self-regression, gnathic NOF appears to be comparatively larger in size and behave more aggressively. A 16 years old female patient reported with painless swelling of the right side of the face of 4 months duration. Radiographic analysis showed a unilocular radiolucent lesion of right angle of the mandible with ill-defined margins, cortical perforation and thinning of inferior border. The lesion was provisionally diagnosed as odontogenic keratocyst/unicystic ameloblastoma and incisional biopsy was performed. The histopathological features and immunohistochemical characteristics favored a diagnosis of NOF. The lesion was excised and reconstructed. The excised specimen confirmed the diagnosis. There are no signs of recurrence at 18 months follow-up. NOF should be considered in the differential diagnosis of uni-/multilocular radiolucencies of jaws particularly the posterior mandible.
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  • 文章类型: Case Reports
    非骨化性纤维瘤通常发生在儿童长骨干,在下颌骨中极为罕见。这里,我们介绍了一例发生在4岁男孩下颌骨中的非骨化纤维瘤。病人没有疼痛的主诉。血清钙的实验室血液检查,磷,副激素水平正常.颌面部区域的计算机断层扫描显示出明确的溶骨性病变,影响了右下颌骨。微观上,病变显示梭形成纤维细胞的轮生束,以及泡沫细胞,混合了很少的多核巨细胞,没有任何骨骼形成。由于下颌骨中很少发生这种病变,因此有必要将非骨化性纤维瘤与其他含巨细胞的病变区分开。我们回顾了14例非骨化性颌骨纤维瘤的组织学特征。
    Non-ossifying fibromas usually occur in the metaphysis of the long bones in children, and are extremely rare in the mandible. Here, we present a case of non-ossifying fibromas which occurred in the mandible of a 4-year-old boy. The patient had no complaint of ache. Laboratory blood examination of serum calcium, phosphorus, and parathormone levels was normal. Computed tomography of the maxillofacial region showed a well-defined osteolytic lesion affecting the right mandible. Microscopically, the lesion showed whorled bundles of spindle-shaped fibroblasts, as well as foam cells, mingled with scant multinucleated giant cells, without any bone formation. It is necessary to distinguish non-ossifying fibromas from other giant cell-containing lesions because of the extremely infrequent occurrence of this lesion in the mandible. We reviewed the histologic features of 14 cases of non-ossifying fibromas involved in the jaw.
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  • 文章类型: Case Reports
    运动损伤发生在体育运动中,或锻炼。然而,有一些病变通常与运动有关,在像泰拳这样要求苛刻的身体运动中,还没有发现典型的病变。我们对文献进行了叙述性回顾,以突出这项运动的典型病变,以分析这些疾病的鉴别诊断。
    一位28岁的女性,泰拳运动员16年来,有6个月的历史,在右胫骨干phy端外侧部分持续疼痛。压力伤害之间的区别,恶性骨肿瘤,和肿瘤样病变后重复的微创伤后的运动活动可能是困难的。诊断方法涉及常规X射线,echotomography,计算机断层扫描,和磁共振成像扫描有争议的发现。活检证实最终诊断为非骨化性纤维瘤。
    本病例报告的目的是展示一名专业泰拳运动员胫骨疼痛的诊断中的挑战,并讨论这类患者在文献中人口有限的临床表现。我们的案例说明,即使是现代成像技术也不能总是区分由运动引起的肿瘤和肿瘤样病变;同时记住我们永远不应该过于关注特定的特征遗忘,甚至是罕见的病变如金刚烷胺瘤。
    UNASSIGNED: Sports injuries occur during sport athletic activities, or exercising. However, there are some lesions which are typically associated to sports, in such a demanding and physical sport like Muay Thai that no typical lesion has been detected yet. We performed a narrative review of the literature to highlight the typical lesions of this sport to analyze the differential diagnosis of those conditions.
    UNASSIGNED: A 28-year-old female, Muay Thai athlete since 16 years, presented with a 6 months history of a persistent pain in the metaphyseal lateral part of the right tibia. Differentiation between stress injuries, malignant bone tumors, and tumor-like lesions after repetitive microtrauma following sport activities can be difficult. The diagnostic approach involved conventional X-ray, echotomography, computed tomography scan, and magnetic resonance imaging scan with controversial findings. The biopsy confirmed the final diagnosis of non-ossifying fibroma.
    UNASSIGNED: The purpose of this case report is to exhibit the challenges in the diagnosis of a professional Muay Thai athlete with tibial pain and to discuss the clinical presentation of this type of patients with a limited population in the literature. Our case illustrates that even modern imaging techniques cannot always distinguish between tumor and tumor-like lesions caused by sports; meanwhile remembered us that we should never be too focused on a particular characteristic forgetting even rare pathologies as adamantinoma.
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  • 文章类型: Journal Article
    背景:良性纤维组织细胞瘤(BFH)是一种罕见的骨肿瘤,很少在脊柱上。
    方法:我们介绍了一名52岁诊断为胸椎BFH的患者,该患者接受了全脊椎整块切除术。还对已发表的文献进行了综述。
    结果:非骨化性纤维瘤(NOF)和BFH被称为一种称为NOF/BFH的肿瘤。以前总共报道了20例脊髓BFHs,主要涉及后路元素。我们提出了一个完全受累于椎骨的BFH。刮治和切除是复发有限的主要治疗选择。
    结论:这是迄今为止第一个完整的椎体BFH。脊髓BFH占据相当低的侵袭性。复发和恶性变性相当有限,手术干预似乎足以应对其管理。
    BACKGROUND: Benign fibrous histiocytoma (BFH) is a rare bone tumor, extremely seldom in the spine.
    METHODS: We present a 52-year-old patient diagnosed with a BFH in the thoracic spine treated with total en bloc spondylectomy. A review of the published literature was also conducted.
    RESULTS: Non-ossifying fibroma (NOF) and BFH are named as one tumor called NOF/BFH. A total of 20 spinal BFHs have been previously reported, mainly involving the posterior elements. We present a BFH with total vertebral involvement. Curettage and excision are the main treatment options with limited recurrence.
    CONCLUSIONS: This is the first total vertebral BFH up to now. Spinal BFH occupies rather low aggressiveness. With rather limited recurrence and malignant degeneration, surgical interventions seem enough for its management.
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