aneurysmal bone cyst

动脉瘤性骨囊肿
  • 文章类型: Case Reports
    动脉瘤性骨囊肿(ABC)是一种良性骨肿瘤,通常发生在生命的第一个和第二个十年。ABC通常表现为快速生长的髓内膨胀性肿块,在长管状骨的干phy端有多个充满血液的囊肿。这里,我们报告一例骨膜实性ABC,最初诊断为高级别表面骨肉瘤.一名10岁男性因左上臂肿胀和压痛被转诊到我们医院。X线摄影术显示骨膜肿块没有液体水平。在进行开放式活检时,肿瘤显示均匀纺锤体向上皮样细胞的高细胞增殖,有丝分裂活动活跃(高达12/2mm2)和蕾丝样类骨质形成,被诊断为高级别表面骨肉瘤。在使用阿霉素和顺铂进行一个疗程的化疗后,周围硬化明显,这导致病理检查和修订诊断为“可能是骨母细胞瘤”。“患者在边缘切除和刮宫后4年内无病。回顾性纳米孔DNA测序意外地检测到PAFAH1B1::USP6重排。使用逆转录-聚合酶链反应进一步验证融合基因,并将诊断修改为ABC。还鉴定了涉及17号染色体的嗜铬杆菌病。甲基化分析使用t分布随机邻居嵌入和无监督分层聚类将本肿瘤分类为ABC或非骨化性纤维瘤。本病例报告重点介绍了纳米孔DNA测序在软组织和骨肿瘤诊断中的应用。
    An aneurysmal bone cyst (ABC) is a benign bone neoplasm that typically occurs during the first and second decades of life. ABC usually presents as a rapidly growing intramedullary expansile mass with multiple blood-filled cysts in the metaphysis of the long tubular bones. Here, we report a case of a periosteal solid ABC that was initially diagnosed as a high-grade surface osteosarcoma. A 10-year-old male was referred to our hospital for swelling and tenderness of the left upper arm. Radiography revealed periosteal mass without fluid-fluid levels. On performing open biopsy, the tumor showed hypercellular proliferation of uniform spindle to epithelioid cells with brisk mitotic activity (up to 12/2 mm2) and lace-like osteoid formation, which was diagnosed as a high-grade surface osteosarcoma. After one course of chemotherapy using adriamycin and cisplatin, peripheral sclerosis was conspicuous, which led to pathological review and revision of diagnosis as \"possibly osteoblastoma.\" The patient was disease-free for 4 years after marginal resection and curettage. Retrospective nanopore DNA sequencing unexpectedly detected a PAFAH1B1::USP6 rearrangement. The fusion gene was further validated using reverse transcription-polymerase chain reaction and the diagnosis was revised to ABC. Chromothripsis involving chromosome 17 has also been identified. Methylation analysis classified the present tumor as an ABC or non-ossifying fibroma using t-distributed stochastic neighbor embedding and unsupervised hierarchical clustering. This case report highlights the utility of nanopore DNA sequencing for soft tissue and bone tumor diagnosis.
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  • 文章类型: Journal Article
    背景尚未采用一组广泛接受的成像标准或分类来评估经皮硬化疗法治疗动脉瘤样骨囊肿(ABC)的反应。在这篇文章中,我们描述并说明了皇家骨科医院(ROH)评分系统,这是一种新的,可重复,和客观工具来评估放射学反应。我们还报告了我们在计算机断层扫描(CT)引导的硬化疗法治疗此类病变的疗效方面的机构经验。患者与方法对19例CT引导下多西环素和白蛋白硬化治疗ABC的患者进行回顾性分析。后续磁共振成像,至少12个月,根据四个ROH评分系统参数进行评估:囊性成分,流体-流体液位,整合的存在,和皮质的完整性。累积分数用于将反应评分为:优秀,不错,模棱两可,或者穷。结果19例患者平均年龄17.8岁,11例发生在长骨,5例骨盆,在每个C3椎体中都有1个,肩胛骨,还有距骨.囊性成分的反应评分的平均参数为2,流体水平为1.3,巩固为2,皮质完整性为2.1。4例反应良好,12例反应良好,2例出现模棱两可的反应,1例反应不佳。评分者间可靠性优异(κ=0.9)。结论ROH评分系统为放射科医师和外科医生提供了一种客观的方法,可以独立地对响应的成像参数进行评分,并根据累积评分得出等级。
    Background  A widely accepted set of imaging criteria or classification has not yet been adopted to evaluate response to treatment by percutaneous sclerotherapy for aneurysmal bone cyst (ABC). In this article, we described and illustrated the Royal Orthopaedic Hospital (ROH) scoring system which is a new, reproducible, and objective tool to evaluate the radiological response. We also reported our institutional experience in the efficacy of computed tomography (CT)-guided sclerotherapy for treating such lesions. Patients and Methods  A retrospective analysis was conducted for 19 patients who underwent CT-guided sclerotherapy with doxycycline and albumin to treat ABC. Follow-up magnetic resonance imaging, at a minimum of 12 months, was assessed according to the four ROH scoring system parameters: cystic component, fluid-fluid level, presence of consolidation, and cortical integrity. The cumulative score was used to grade response as either: excellent, good, equivocal, or poor. Results  Out of 19 patients with a mean age of 17.8 years, 11 cases occurred in the long bones, 5 cases in the pelvis, and 1 in each of the C3 vertebral body, scapula, and talus. The mean parameter of response score for cystic component was 2, fluid-fluid level was 1.3, consolidation was 2, and cortical integrity was 2.1. Four cases showed excellent response, 12 cases showed good response, 2 cases showed equivocal response, and 1 case showed poor response. Interrater reliability was excellent (κ = 0.9). Conclusion  The ROH scoring system provides the radiologist and surgeon with an objective method to score imaging parameters of response independently and achieve a grade based on the cumulative score.
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  • 文章类型: Case Reports
    动脉瘤性骨囊肿(ABCs)是侵袭性和良性肿瘤,主要影响儿童和青少年。ABCs的标准治疗过程包括手术切除或刮除,并进行骨移植或水泥以修复缺陷。Denosumab,抑制核κB配体受体激活剂的单克隆抗体,用于治疗骨质疏松症,骨转移,和骨骼巨细胞瘤。
    本案例研究详细介绍了一名女性患者的治疗方法,年龄22岁,患有胫骨远端复发性侵袭性ABC。患者最初使用刮宫和病变填充进行治疗。然而,9个月后观察到骨质溶解的复发,这导致随后的干预措施包括在多个疗程中进行无水酒精硬化治疗.然而,这些干预措施未能实现骨化。在手术和硬化治疗不成功之后,病人服用了denosumab,这导致了积极的回应。定期的影像学和临床随访显示骨化和疼痛减轻的显着改善。在12个月的治疗过程中,访问频率逐渐减少。Further,随访和监测显示了局部控制和长期治疗的有效性。
    本病例报告强调了denosumab在手术或硬化治疗失败后治疗复发性侵袭性ABCs的能力。
    UNASSIGNED: Aneurysmal bone cysts (ABCs) are aggressive and benign tumors that primarily affect children and adolescents. The standard course of treatment for ABCs involves surgical excision or curettage with a bone transplant or cement to repair the deficiency. Denosumab, a monoclonal antibody that inhibits receptor activator of nuclear kappa B ligand, is used to treat osteoporosis, skeletal metastasis, and giant cell tumors of the bones.
    UNASSIGNED: This case study details the therapeutic treatment of a female patient, age 22, who had a recurring aggressive ABC of the distal tibia. The patient was initially treated using curettage and lesion filling. However, recurrence of the osteolysis was observed 9 months later that led to subsequent interventions involving absolute alcohol sclerotherapy in multiple sessions. However, these interventions failed to achieve ossification. Following unsuccessful surgical and sclerotherapy treatments, the patient was administered denosumab, which led to a positive response. Regular radiographic and clinical follow-up demonstrated significant improvements in ossification and pain reduction. During the course of the 12-month treatment, the frequency of visits was gradually reduced. Further, follow-up and monitoring revealed the effectiveness of the local control and long-term treatment.
    UNASSIGNED: This case report highlights the ability of denosumab to manage recurrent aggressive ABCs after surgical or sclerotherapy failure.
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  • 文章类型: Case Reports
    动脉瘤性骨囊肿是一种病因不明的罕见溶骨性病变,通常在下肢观察到,只有1-2%的报告在颌骨。我们介绍了一名27岁的男性患者的病例,该患者因精神区域感觉异常和下颌体积增加而转诊口腔颌面外科和创伤学服务。体格检查显示中线移位和硬一致性。影像学检查显示下颌皮质破裂,有射线可透/低密度病变。切开活检材料的组织病理学检查导致中央巨细胞病变的诊断。病人接受了手术切除,标本的组织病理学分析显示主要为实体病变,以不同大小的充满血液的空间为特征,不被上皮或内皮覆盖,随着梭形细胞的存在,多核巨细胞,和嗜碱性骨样物质,结论诊断为混合型动脉瘤样骨囊肿。尽管不常见,在年轻患者颌骨体积增加的鉴别诊断中,应考虑动脉瘤性骨囊肿。
    Aneurysmal bone cyst is a rare osteolytic lesion of uncertain etiology, commonly observed in the lower limbs, with only 1-2% of reports in gnathic bones. We present the case of a 27-year-old male patient referred to the oral and maxillofacial surgery and traumatology service due to complaints of paresthesia in the mental region and increased mandibular volume. Physical examination revealed midline shift and hard consistency. Imaging examinations demonstrated a radiolucent/hypodense lesion with disruption of the mandibular cortices. The histopathological examination of incisional biopsy material led to the diagnosis of a central giant cell lesion. The patient underwent surgical resection, and the histopathological analysis of the specimen revealed a predominantly solid lesion, characterized by blood-filled spaces of varying size, not covered by epithelium or endothelium, with the presence of spindle cells, multinucleated giant cells, and basophilic osteoid material, concluding the diagnosis of mixed-type aneurysmal bone cyst. Despite being uncommon, aneurysmal bone cysts should be considered in the differential diagnosis of volumetric increase in the gnathic bones of young patients.
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  • 文章类型: Journal Article
    动脉瘤性骨囊肿(ABCs)是罕见的良性骨病变,对长骨干meta端有好感。它们通常是囊性的,膨胀,和溶骨,并可能导致骨畸形。总的来说,关于ABC的最佳治疗仍存在争议;然而,主要由刮宫的组合组成,骨移植,并考虑内固定和截骨术的需要。治疗的目标包括保留骨解剖结构,同时消除病变。关于与良性骨肿瘤相邻的骨关节炎的治疗的文献很少。如果选择全髋关节置换术作为治疗选择,需要勤奋的术前计划,外科医生必须评估病人的骨存量,骨畸形的原因,并根据患者的特征使用特定的植入物和技术。我们介绍了一例患有股骨近端ABC和有症状的相邻髋关节骨关节炎的成年患者,该患者接受了全髋关节置换术的治疗。
    Aneurysmal bone cysts (ABCs) are rare benign bone lesions with a predilection for the metaphysis of long bones. They are often cystic, expansive, and osteolytic and may result in bony deformity. In general, there remains debate about optimal treatment for ABCs; however, the mainstay typically consists of a combination of curettage, bone grafting, and considering the need for internal fixation and osteotomies. The goals of treatment include preserving bony anatomy while eliminating the lesion. There is sparse literature regarding the treatment of osteoarthritis adjacent to benign bony tumors. If total hip arthroplasty is chosen as a treatment option, diligent preoperative planning is required, and the surgeon must assess the patient\'s bone stock, account for bony deformity, and utilize specific implants and techniques based on the patient\'s characteristics. We present a case of an adult patient with proximal femur ABC and symptomatic adjacent hip osteoarthritis who underwent treatment with total hip arthroplasty.
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  • 文章类型: Case Reports
    在胸壁上发展的肿瘤通常很少见。此病例报告强调了罕见的源自第四肋骨前弓的巨细胞瘤。病人,一个21岁的男性,呈现一个膨胀的质量,在八个月的时间里逐渐增加,达到12x8厘米的尺寸。尽管有明显的肿胀,患者报告无相关疼痛或不适,否认有任何体重减轻或外伤史.没有胸痛或心血管症状将这种情况与其他胸壁病变区分开。该报告强调了在胸壁肿块的鉴别诊断中考虑罕见实体如巨细胞瘤的重要性。尤其是在临床表现和患者病史与更常见的情况不一致的情况下。
    Tumors that develop on the chest wall are usually rare. This case report highlights a rare occurrence of a giant cell tumor originating from the anterior arch of the fourth rib. The patient, a 21-year-old male, presented with a bulging mass that had been gradually increasing in size over an eight-month period, reaching dimensions of 12 x 8 cm. Despite the noticeable swelling, the patient reported no associated pain or discomfort and denied any history of weight loss or trauma. The absence of chest pain or cardiovascular symptoms distinguished this case from other chest wall pathologies. This report underscores the importance of considering rare entities such as giant cell tumors in the differential diagnosis of chest wall masses, especially in cases where clinical presentation and patient history do not align with more common conditions.
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  • 文章类型: Journal Article
    背景:动脉瘤性骨囊肿(ABCs)是溶骨性的,非恶性,骨的血管病变。病理性骨折可能是ABCs的表现,发生在约8%的ABC中。文献中已经描述了不同的治疗方法,但是,如今,ABCs患者病理性骨折的最佳治疗仍存在争议,目前尚无标准的治疗指南,也没有关于最佳手术干预的共同指征.我们研究的目的是回顾有关此问题的现有文献,探索和面对ABC中病理性骨折的不同手术治疗方法,以阐明这些患者的手术方法。方法:对PubMed索引的文献进行系统回顾,MEDLINE,并进行了Cochrane图书馆数据库。遵循系统审查和荟萃分析(PRISMA)的首选报告项目。结果:共有37篇相关文章最终纳入研究。总的来说,我们达到了140名患者。在纳入审查的140名患者中,124例患者(88.6%)接受了刮宫手术,15例(10.7%)患者行整块切除手术。共有47%的患者(70)接受了钢板的综合手术,螺钉,钉,或外固定器。8.6%的患者使用辅助治疗(12)。并发症涉及20.7%的患者(29)。结论:总之,动脉瘤性骨囊肿病理性骨折的治疗需要仔细的患者评估,考虑到年龄等因素,开放生长板的存在,病变的位置,和外科医生的专业知识。
    Background: Aneurysmal bone cysts (ABCs) are osteolytic, non-malignant, vascular lesions of the bone. Pathological fractures can be a manifestation of the ABCs, which occur in about 8% of ABCs. Different treatments have been described in the literature, but, nowadays, an optimal management of the pathological fractures in patients with ABCs is still a matter of debate and there are no standard guidelines for treatment nor any shared indication about the best surgical intervention. The aim of our study is to review the current literature available on this matter exploring and confronting different surgical treatments for pathological fractures in ABC in order to clarify the surgical approach to these patients. Methods: A systematic review of the literature indexed in PubMed, MEDLINE, and Cochrane Library databases was carried out. The Preferred Reporting Items for Systematically Reviews and Meta-Analyses (PRISMA) were followed. Results: A total of 37 articles were relevant and were finally included in the study. In total, we reached a population of 140 patients. Of the 140 patients included in the review, 124 patients (88.6%) underwent curettage surgery, 15 patients (10.7%) underwent en bloc resection surgery. A total of 47% of patients (70) underwent synthesis surgery with a plate, screw, nail, or external fixator. Adjuvant treatments were used in 8.6% of patients (12). Complications involved 20.7% of the patients (29). Conclusions: In conclusion, the treatment of pathological fractures in aneurysmal bone cysts requires careful patient assessment, considering factors such as age, the presence of open growth plates, the location of the lesion, and the surgeon\'s expertise.
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  • 文章类型: Case Reports
    骨巨细胞瘤(GCT)的重建选择是有限的和具有挑战性的,由于结构损害的数量和高复发率。对于脚和脚踝的GCT尤其如此,因为该区域对承重和功能至关重要。目前GCT的典型治疗方法是切除,刮宫,和胶结,虽然这并不总是有效的。一名36岁的健康女性,最初诊断为胫骨远端大动脉瘤性骨囊肿(ABC),尽管先前曾两次尝试切除和骨水泥治疗,但仍复发。她接受了手术切除病灶的治疗,重建,以及踝关节和距下关节的关节固定术,使用胫骨骨髓内钉和小梁金属锥。术中标本的最终病理与GCT一致。术后,她恢复得很好,她的成像与成功的融合一致。此病例报告提供了证据,表明在一次手术中使用后足指甲和小梁金属锥结构的独特组合进行胫骨骨融合是治疗大型,胫骨远端复发性GCT病变。
    Reconstruction options for giant cell tumors (GCTs) of bone are limited and challenging due to the amount of structural compromise and the high recurrence rates. This is especially true for GCTs of the foot and ankle, as the area is vital for weight bearing and function. The typical treatment for GCTs is currently excision, curettage, and cementation, although that is not always effective. A 36-year-old otherwise healthy female presented with an original diagnosis of a large aneurysmal bone cyst (ABC) of the distal tibia that had recurred despite two previous attempts at treatment with resection and cementation. She was treated with surgical resection of the lesion, reconstruction, and ankle and subtalar joint arthrodesis with a tibiotalocalcaneal intramedullary nail in combination with a trabecular metal cone. The final pathology of the intraoperative samples was consistent with GCT. Postoperatively, she recovered well, and her imaging was consistent with a successful fusion. This case report provides evidence that tibiotalocalcaneal fusion with a unique combination of hindfoot nail and trabecular metal cone construct in a single procedure is a successful option for the treatment of large, recurrent GCT lesions in the distal tibia.
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    文章类型: Case Reports
    动脉瘤性骨囊肿(ABC)具有侵袭性,膨胀性,和局部破坏性血管病变。目前尚不清楚ABCs的确切病因,据推测与血管畸形或骨血管破坏有关。迄今为止,目前还没有关于阴茎内翻阴道成形术(PIV)后发生耻骨ABCs的报道.
    本报告描述了先前接受过PIV的跨性别患者的公共ABC的发展,可能表明这种性别确认手术非常罕见的并发症。
    一名37岁的变性人女性最初被转诊到骨科肿瘤诊所,以评估12个月的左髋和腹股沟疼痛病史。她在演讲前约19个月接受了性别确认PIV。增强磁共振成像(MRI)显示,左耻骨上支的T1信号强度低,T2高强度不均匀7.5×4.9×4.3cm破坏性肿块,延伸穿过耻骨联合进入右耻骨上支。针芯骨活检显示出可变的细胞纺锤体和圆形病变,并伴有类骨质形成和局灶性坏死。细胞对CD34、S100和结蛋白呈阴性。没有证据表明骨肉瘤,最终审查赞成ABC的诊断。鉴于质量的高度破坏性,它被切除了,用生物真皮网重建伤口。
    尽管在这种情况下无法区分巧合和因果关系,患者最近的PIV和附近骨骼中罕见的ABC的发展值得本报告中的推测和讨论。
    UNASSIGNED: Aneurysmal bone cysts (ABCs) are aggressive, expansile, and locally destructive vascular lesions. The exact etiology of ABCs is currently unknown and hypothesized to be related to vascular malformations or disruption of osseous vascularity. To date, there have been no reports describing the development of pubic ABCs following penile inversion vaginoplasty (PIV).
    UNASSIGNED: This report describes the development of a pubic ABC in a transgender patient who had previously undergone PIV, possibly indicating a very rare complication of this gender-affirming operation.
    UNASSIGNED: A 37-year-old transgender female was initially referred to the orthopedic oncology clinic for evaluation of a 12-month history of left hip and groin pain. She had undergone gender-affirming PIV about 19 months prior to presentation. Magnetic resonance imaging (MRI) with contrast revealed a low T1 signal intensity and heterogenous T2 hyperintensity 7.5 × 4.9 × 4.3-cm destructive mass in the left superior pubic ramus extending across the pubic symphysis into the right superior pubic ramus. A needle core bone biopsy demonstrated a variably cellular spindle and round lesion with islands of osteoid formation and focal necrosis. The cells were negative for CD34, S100, and desmin. There was no evidence suggesting osteosarcoma, and final review favored the diagnosis of an ABC. Given the highly destructive nature of the mass, it was resected, and the resulting wound was reconstructed with a biologic dermal mesh.
    UNASSIGNED: Although it is impossible to distinguish coincidence from causation in this case, the patient\'s recency of PIV and development of a rare ABC in a nearby bone warrants the speculation and discussion provided in this report.
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  • 文章类型: Journal Article
    背景:动脉瘤性骨囊肿(ABCs)是良性的,长骨中可能发生的溶骨性病变,椎骨,或者很少,头骨。在这里,作者介绍了一名15岁男性的左额顶颅骨原发性ABC的病例,并回顾了文献,以深入了解这种罕见疾病的性质。
    方法:一名健康的15岁男性表现出紧张,左额顶头皮疼痛病变。他无法识别任何煽动创伤,但在出现前不到2周首先注意到病变逐渐增大。颅骨成像显示颅骨溶解性病变,液体水平提示ABC。通过广泛切除病变并重建颅骨缺损提供治疗。这没有并发症,组织病理学评估证实了原发性ABC的诊断。
    结论:颅骨的ABCs是罕见实体,最常见于颅底和颅骨。通常,这些病变与潜在的骨病理学(继发性ABCs)相关,但很少见到孤立性病变(原发性ABCs).临床管理包括切除和适当的基础病理的辅助治疗。
    BACKGROUND: Aneurysmal bone cysts (ABCs) are benign, osteolytic lesions that can occur in long bones, vertebrae, or rarely, the skull. Here the authors present the case of a 15-year-old male with a primary ABC of the left frontoparietal skull along with a review of the literature to provide insight into the nature of this rare disease.
    METHODS: An otherwise healthy 15-year-old male presented with a tense, painful lesion of the left frontoparietal scalp. He could not identify any inciting trauma, but first noted the lesion less than 2 weeks prior to presentation with progressive enlargement. Cranial imaging revealed a lytic skull lesion with fluid-fluid levels suggestive of ABC. Curative therapy was provided via wide excision of the lesion and calvarial reconstruction of the resultant skull defect. This was performed without complication, and histopathological evaluation confirmed the diagnosis of primary ABC.
    CONCLUSIONS: ABCs of the skull are rare entities and most often arise in the skull base versus the calvaria. Typically, these lesions are associated with an underlying bone pathology (secondary ABCs) but can be rarely seen as isolated lesions (primary ABCs). Clinical management consists of excision and adjuvant therapy for underlying pathology where appropriate.
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