Bone Cysts, Aneurysmal

骨囊肿,动脉瘤
  • 文章类型: English Abstract
    Objective: To investigate the radiologic, pathologic, and molecular features of simple bone cysts (SBC), and their differential diagnoses. Methods: Fourteen cases of SBC were collected at the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from 2017 to 2022, and fluorescence in situ hybridization (FISH) was performed for retrospective analysis. Results: There were 14 patients, including 7 females and 7 males, with age range of 7 to 45 (median 29) years. The most common complaint was pain, including 4 cases with pathological fracture and 5 with history of previous trauma. The tumor size ranged from 3.4 to 13.5 (median 5.6) cm. The lesion involved the femur (n=4), humerus (n=5) and iliac bone (n=5). Radiologic diagnoses included SBC, aneurysmal bone cyst, and giant cell tumor of the bone or its combination with aneurysmal bone cyst-like region and fibrous dysplasia. Histologically, the cyst walls of the lesions were composed of fibrous tissue, fibrin-like collagen deposits, bone-like matrix and occasional woven bone. The lesional cells were spindled to ovoid, with scattered osteoclast-like giant cells, foamy histiocytes, hemosiderin deposits and cholesterol clefts. In 6 cases there were nodular fasciitis-like areas. Immunohistochemically, the spindled to ovoid cells were positive for SMA, EMA and SATB2 in varying degrees. FISH detection was performed in all 14 cases and EWSR1/FUS rearrangement were found in 9 cases. One case of FUS::NFATC2 fusion was detected by next-generation sequencing. Nine cases of SBC with the rearrangement were more cellular, and there were more mitotic figures in the recurrent FUS::NFATC2 fusion tumor. Clinical follow-up was obtained in all 14 cases with the time ranging from 5 to 105 (mean 46) months. Amongst them, the tumor with FUS::NFATC2 rearrangement had local recurrence twice after the first local excision, but had no more recurrence or metastasis 34 months after the subsequent segmental resection. The other 13 cases had no recurrence. Conclusions: EWSR1 or FUS rearrangement is most commonly identified in SBC, suggesting that SBC might be a neoplastic disease. In cases where the radiologic appearance and histomorphology are difficult to differentiate from aneurysmal bone cyst, FISH detection can aid in the definitive diagnosis.
    目的: 探讨单纯性骨囊肿(simple bone cyst,SBC)的临床影像学、病理形态学、分子遗传学特点、诊断及鉴别诊断。 方法: 收集南京医科大学第一附属医院病理学部2017—2022年诊断为SBC的病例14例,行荧光原位杂交(FISH),回顾性分析影像学、病理学及分子遗传学特征。 结果: 14例SBC中,男性7例,女性7例,年龄7~45岁(中位年龄29岁)。临床表现以局限性疼痛最为常见,其中4例伴有病理性骨折,5例伴有既往创伤史。最大径3.4~13.5 cm(中位5.6 cm),累及肱骨(5例)、髂骨(5例)、股骨(4例)。影像学诊断有SBC、动脉瘤性骨囊肿、骨巨细胞瘤、骨巨细胞瘤合并动脉瘤性骨囊肿样区域,以及纤维结构不良。形态学上,低倍镜下囊壁由纤维结缔组织构成,可以见到纤维蛋白样沉积物,部分有骨样基质及编织骨的形成。高倍镜下,囊壁可见呈胖梭形、卵圆形病变细胞,散在的破骨样巨细胞,堆积的泡沫样组织细胞,含铁血黄素的沉积以及胆固醇裂隙,6例可见类似结节性筋膜炎样的形态。免疫表型上,囊壁内衬细胞可见平滑肌肌动蛋白、上皮细胞膜抗原、SATB2不同程度的表达。14例均行FISH检测,发现9例涉及FUS或ESRW1基因的重排,其中1例行二代测序发现FUS::NFATC2融合。9例发生重排的SBC细胞密度略高,其中FUS::NFATC2融合患者复发标本可见核分裂象。14例随访5~105个月(平均46个月),其中FUS::NFATC2重排患者术后2次局部复发,第2次复发后行瘤段切除术34个月未复发,其余13例均无复发。 结论: SBC中有很大一部分存在EWSR1或FUS的重排,提示SBC可能是一个肿瘤性疾病。当影像学和形态学特征对于鉴别诊断SBC和动脉瘤性骨囊肿困难时,可结合FISH检测辅助鉴别。.
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  • 文章类型: Case Reports
    背景:青少年沙瘤样骨化纤维瘤(JPOF)是一种非癌性骨肿瘤,通常影响青少年的颅颌面区域。临床表现通常是肿瘤侵袭性压迫周围组织引起的症状。动脉瘤性骨囊肿(ABC)也是一种良性骨肿瘤,它通常发生在长骨和脊柱中。只有2%至3%的病例发生在头颈部。由于这种临床病例的罕见,临床医生在全面了解这一复杂病变方面面临困难.因此,对于外科医生来说,全面审查临床表现和特征性影像学表现是必要的。
    方法:2019年4月6日,一名13岁男孩出现左侧颌面隆起和疼痛1个月。鼻旁窦的磁共振成像显示左上颌窦有不规则的蜂巢样肿块信号,和囊性改变与液体水平在病变中可见。初次ABC诊断为JPOF后,我们决定对上颌窦肿瘤进行面部中面部切除术以切除肿瘤组织。最后,经过3次复发和4次操作,最后一次手术后20个月没有肿瘤复发,患者仍在持续随访中。
    结论:该病例为JPOF合并ABC的诊断和治疗提供了参考。特别是,提出了对这两种疾病之间的关联和复发管理的新认识,这有可能提高临床对这种复杂疾病的认识。
    BACKGROUND: Juvenile Psammomatoid Ossifying Fibroma (JPOF) is a type of noncancerous bone tumor that usually affects adolescents in the craniomaxillofacial area. Clinical manifestations are usually symptoms caused by the tumor\'s invasive compression of surrounding tissues. Aneurysmal Bone Cyst (ABC) is also a benign bone tumor, and it typically occurs in long bones and the spine. Only 2% to 3% of cases occur in the head and neck. Due to the rarity of this combination of clinical cases, clinicians face difficulties in comprehensively understanding this complex lesion. Therefore, a comprehensive review of the clinical manifestations and characteristic imaging findings is necessary for surgeons.
    METHODS: On April 6, 2019, a 13-year-old boy presented with left maxillofacial bulge and pain for 1 month. Magnetic resonance imaging of the paranasal sinuses showed an irregular hive-like mass signal in the left maxillary sinus, and cystic changes with fluid levels were seen in the lesion. After the initial diagnosis of JPOF with primary ABC, we decided to perform a facial mid-facial resection of maxillary sinus tumor to remove the tumor tissue. Finally, after 3 recurrences and 4 operations, there was no tumor recurrence for 20 months after the last operation, and the patient was still under continuous follow-up.
    CONCLUSIONS: This case provided a reference for the diagnosis and treatment of JPOF combined with ABC. In particular, a new understanding of the association between the two diseases and the management of recurrence were proposed, which had the potential to improve clinical understanding of this complicated condition.
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  • 文章类型: Journal Article
    目的:我们报告了一例纤维发育不良(FD)伴动脉瘤样骨囊肿(ABC)样改变的儿童眼眶受累,审查相关案件,并讨论临床特征,治疗,和这种疾病的预后。
    方法:一名10岁女孩右眼球突出(眼球突出程度:OD16mm,OS13mm)和有限的视力(视力:OD1.0,OS0.8),无外伤。术前CT显示右侧颅眶交通瘤5.0*4.3cm。MRI显示明确的多囊性肿块,流体水平分散,肥皂泡样改变。患儿接受了全肿瘤切除和眶壁钛网重建。在20个月的随访中,孩子已经从眼部问题中恢复过来,肿瘤没有复发.
    结论:FD合并ABC很少发生在眼眶,通常以眼部症状开始。病因尚不确定。早期诊断和手术至关重要。建议尽可能完全切除,因为残留病变可能会复发。
    OBJECTIVE: We report a case of fibrous dysplasia (FD) with aneurysmal bone cyst (ABC)-like change in a child with orbital involvement, review the related cases, and discuss clinical features, therapy, and prognosis of this disease.
    METHODS: A 10-year-old girl had right proptosis (degree of exophthalmos: OD 16 mm, OS 13 mm) and limited vision (visual acuity: OD 1.0, OS 0.8) without trauma. Preoperative CT showed a 5.0*4.3 cm right-sided crania-orbital communicating tumor. MRI indicated a well-defined multicystic mass with scattered fluid levels and soap bubble-like alterations. The child underwent total tumor resection and orbital parietal titanium mesh reconstruction. At 20 months of follow-up, the child has recovered from ocular problems, and the tumor has not recurred.
    CONCLUSIONS: FD combined with ABC rarely occurs in orbit and generally begins with ocular symptoms. The etiology is uncertain. Early diagnosis and surgery are essential. Complete resection is suggested whenever possible because residual lesions may recur.
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  • 文章类型: Case Reports
    背景:动脉瘤性骨囊肿(ABC)是一种罕见的原发性或继发性肿瘤,通常发生在10至20岁之间的年轻女性中,主要在长管状骨和脊柱。然而,它的临床治疗没有明确的标准。据我们所知,这是一例年轻女性桡骨远端ABC患者首次报道,该患者通过肿瘤切除和自体腓骨头移植成功治疗.
    方法:一名28岁已婚中国女青年到我院就诊,右手腕肿胀疼痛2年,手腕活动受限加重1周。
    方法:病理活检证实ABC。
    方法:我们对右腕部肿瘤进行了病理检查,初步证实了ABC的诊断。通过在右腕关节中完全手术切除ABC肿瘤并自体腓骨头移植重建右腕关节。
    结果:在7年内的随访中,确认右手腕功能良好。肿瘤没有复发,右手腕的肿胀消失了,关节疼痛和运动受限明显改善,右手腕的功能在日常活动中没有受损。射线照相显示骨折已愈合。
    结论:我们的结果表明,自体腓骨头移植是重建桡骨远端ABC成年患者腕关节功能的有效治疗方法。
    BACKGROUND: Aneurysmal bone cyst (ABC) is a rare primary or secondary tumor that usually occurs in young women aged between 10 and 20 years, mostly in the long tubular bone and spine. However, there are no definite standards for its clinical treatment. To our knowledge, this is the first report of a young female patient with distal radius ABC who was successfully treated with tumor resection and autogenous fibular head transplantation.
    METHODS: A 28-year-old married Chinese young woman presented to our hospital with swelling and pain in her right wrist for 2 years and aggravation of wrist movement restriction for 1 week.
    METHODS: Pathological biopsy confirmed ABC.
    METHODS: We performed a pathological examination of the tumor on the right wrist and preliminarily confirmed the diagnosis of ABC. The right wrist joint was reconstructed by total surgical resection of the ABC tumor in the right wrist joint and autogenous fibular head transplantation.
    RESULTS: During follow-up within 7 years, good right wrist function was confirmed. The tumor did not recur, the swelling of the right wrist disappeared, the joint pain and limitation of movement significantly improved, and the function of the right wrist was not impaired in daily activities. Radiography showed that the fracture had healed.
    CONCLUSIONS: Our results suggest that autofibular head transplantation is an effective treatment for reconstruction of wrist function in adult patients with ABC of the distal radius.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    目的:为了确定计算机断层扫描(CT)纹理分析参数是否可以用作定量生物标志物,以帮助区分骨巨细胞瘤(GCT),原发性动脉瘤性骨囊肿(PABC),和继发于骨巨细胞瘤(GABC)的动脉瘤性骨囊肿(ABCs)。
    方法:一百零七例患者,63例GCT,31个PABC,对13例GABC进行了回顾性分析。所有患者术前均行CT检查。两名放射科医生独立评估CT图像的定性特征并提取纹理参数。患者人口统计学,定性特征,和GCT之间的纹理参数,PABC,和GABC进行统计学比较。还评估了ABC和GCT之间这些参数的差异。获得ROC曲线以确定最佳参数值。
    结果:区分GCT的最佳术前CT参数,PABC,GABCs包括一个定性特征(膝盖周围的位置)和四个纹理参数(第95百分位数,最大强度,偏斜度,和峰度)。年龄和三个纹理参数(第5百分位数,不均匀性,和峰度)使GCT和ABC之间具有统计学上的显着差异。上述四个参数的组合产生了GCT和ABC分化的最大ROC曲线下面积(AUC)。
    结论:CT纹理分析参数可作为GCT术前鉴别的定量生物标志物,PABC,和GABCs。
    To determine whether computed tomography (CT) texture analysis parameters can be used as quantitative biomarkers to help differentiate giant cell tumour of bones (GCTs), primary aneurysmal bone cysts (PABCs), and aneurysmal bone cysts (ABCs) secondary to giant cell tumours of bone (GABCs).
    One hundred and seven patients with 63 GCTs, 31 PABCs, and 13 GABCs were analysed retrospectively. All patients underwent preoperative CT. Two radiologists independently evaluated the qualitative features of the CT images and extracted texture parameters. Patient demographics, qualitative features, and texture parameters among GCTs, PABCs, and GABCs were compared statistically. Differences in these parameters between ABCs and GCTs were also assessed. ROC curves were obtained to determine optimal parameter values.
    The best preoperative CT parameters to differentiate GCTs, PABCs, and GABCs included one qualitative feature (location around the knee) and four texture parameters (95th percentile, maximum intensity, skewness, and kurtosis). Age and three texture parameters (5th percentile, inhomogeneity, and kurtosis) enabled statistically significant differentiation between GCTs and ABCs. Combination of the above four parameters generated the largest area under the ROC curve (AUC) for the differentiation of GCTs and ABCs.
    CT texture analysis parameters can be used as quantitative biomarkers for preoperative differentiation among GCTs, PABCs, and GABCs.
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  • 文章类型: Journal Article
    目的:这项回顾性研究的目的是调查和比较临床,射线照相,病态,发病机制,以及颌骨单纯性骨囊肿(SBC)和动脉瘤性骨囊肿(ABCs)的治疗特征。
    方法:在口腔颌面外科接受治疗的35例SBCs和6例ABCs患者,对2017年至2022年浙江大学医学院进行回顾性随访。
    结果:该研究包括41名患者,占所有颌骨病变的2.14%,35例患者患有SBCs,6例患者患有ABCs;他们的平均年龄为26.63±13.62岁和17.83±7.88岁,分别。SBC和ABC的患病率在性别之间没有显着差异。下颌骨是最脆弱的区域。只有5.71%(2/35)的SBC患者和16.7%(1/6)的ABC患者报告了在假囊肿同一区域的先前创伤史。共42.86%(15/35)的SBC病例和66.67%(4/6)的ABC病例发生错牙合。假性囊肿的影像学特征在形状上有所不同,与根相关联,和单眼或多房性。所有患者均进行刮宫术或不进行植骨或替代植入,平均随访时间为26.23±15.47个月和21.67±19.75个月后,94.29%(33/35)的SBC患者和100%(6/6)的ABC患者均未出现复发,分别。
    结论:假性囊肿,包括SBC和ABC,是良性溶骨性病变,没有上皮衬里,偶尔发生在颌骨,主要是青少年和年轻人,且其发病率因性别而无显著差异。最脆弱的部位是下颌骨,他们通常没有明显的攻击性。创伤在假性囊肿中的作用不那么重要,但是轻微的创伤,比如错牙合,有可能影响假性囊肿的发展。假性囊肿的临床表现缺乏特异性,大多数患者无症状,在X光片上偶然发现。牙科全景X光片和CBCT不能准确区分SBC和ABC,最终的诊断取决于病理诊断。刮治结合植骨是目前两者的最佳治疗方法,SBC复发率为5.71%(2/35),ABC无复发。
    The purpose of this retrospective study was to investigate and compare the clinical, radiographic, pathological, pathogenesis, and therapeutic features of simple bone cysts (SBCs) and aneurysmal bone cysts (ABCs) of the jaw.
    35 patients with SBCs and 6 patients with ABCs who received treatment at the Department of Oral and Maxillofacial Surgery, Zhejiang University School of Medicine from 2017 to 2022 were followed up and reviewed retrospectively.
    The study included 41 patients, accounting for 2.14% of all jaw pathologies, with 35 patients having SBCs and 6 patients having ABCs; their average ages were 26.63 ± 13.62 years and 17.83 ± 7.88 years, respectively. The prevalence of SBC and ABC did not differ significantly by sex. The mandible was the most vulnerable area to be involved. Only 5.71% (2/35) of patients with SBCs and 16.7% (1/6) of patients with ABCs reported histories of previous trauma in the same region of the pseudocysts. A total of 42.86% (15/35) of SBC cases and 66.67% (4/6) of ABC cases had malocclusions. The radiographic features of pseudocysts varied in shape, were associated with the root, and unilocular or multilocular. All patients had curettage with or without bone graft or substitute implantation, and recurrences did not occur in 94.29% (33/35) of SBC patients and 100% (6/6) of ABC patients after a mean follow-up time of 26.23 ± 15.47 months and 21.67 ± 19.75 months, respectively.
    Pseudocysts, including SBCs and ABCs, are benign osteolytic lesions without an epithelial lining that occur occasionally in the jaw, mostly in adolescents and young adults, and their incidence did not significantly differ by sex. The most vulnerable site of involvement is the mandible, and they are generally not overtly aggressive. Trauma has a less significant role in pseudocysts, but minor trauma, such as malocclusion, has the potential to influence pseudocyst development. The clinical presentation of pseudocysts lacks specificity, and most patients are asymptomatic and found incidentally during radiographs. Dental panoramic radiographs and CBCT cannot accurately distinguish between SBC and ABC, and the final diagnosis depends on pathological diagnosis. Curettage combined with bone grafting is currently the best treatment for both, with a 5.71% (2/35) recurrence rate for SBC and no recurrence found for ABC.
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  • 文章类型: Case Reports
    背景:动脉瘤性骨囊肿是扩张性良性病变,伴有压迫性破坏和不明发病机制。颞骨受累的最常见部位是岩尖,鳞状部分和乳突。
    方法:本文报道了一名51岁男性的右颞部动脉瘤样骨囊肿,该患者临床上患有面神经麻痹,听力下降和前庭功能受损。磁共振成像和计算机断层扫描结果与动脉瘤性骨囊肿的诊断一致。术中发现,病变对内听道造成了压迫性损伤。手术切除后,患者出现眩晕,显示前庭功能的恢复。随访影像学显示完全切除,无临床复发。
    结论:据我们所知,这是动脉瘤样骨囊肿侵犯内耳道的首次报道。我们的临床经验表明,前庭神经损伤恢复相对少见。该病例报告有望为未来的研究提供信息。
    BACKGROUND: Aneurysmal bone cysts are expansile benign lesions associated with compressive destruction and obscure pathogenesis. The most common sites of temporal bone involvement are the petrous apex, squamous portions and mastoid.
    METHODS: This paper reports a right temporal aneurysmal bone cyst in a 51-year-old man who presented clinically with facial palsy, and hearing loss and impaired vestibular function. Magnetic resonance imaging and computed tomography findings were consistent with a diagnosis of aneurysmal bone cyst. Inter-operative findings showed that the lesion had caused compressive damage to the internal auditory canal. Following surgical excision, the patient experienced vertigo, indicating recovery of vestibular function. Follow-up imaging revealed complete resection without clinical recurrence.
    CONCLUSIONS: To our knowledge, this is the first report of aneurysmal bone cyst invasion of the inner auditory canal. Our clinical experience indicates that vestibular nerve damage recovery is relatively uncommon. This case report will hopefully inform future studies.
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  • 文章类型: Review
    脊柱巨细胞瘤(GCT)合并继发性动脉瘤样骨囊肿(ABC)是一种局部侵袭性原发性骨肿瘤。从未报道过完全脊椎切除术可治疗胸部GCT联合继发性ABC。我们回顾性分析了2例脊柱GCT合并继发性ABC的病例。一名41岁的男性患者因涉及T6椎体和椎管内占位性病变的不规则扩张性骨破坏而出现背痛。T6椎体全脊椎整块切除术,术后神经功能良好。一名29岁的女性患者由于涉及T5椎体和椎管内占位性病变的不规则扩张性骨破坏而出现右肩胛骨区域疼痛。T5椎体全脊椎整块切除术,术后神经功能良好。术后12个月或24个月随访无局部复发,辅助放疗。脊柱GCT合并继发性ABC似乎具有较高的局部复发率。因此,胸部GCT联合二次ABC治疗应采用全脊椎整块切除术。
    Spinal giant cell tumor (GCT) combined with secondary aneurysmal bone cyst (ABC) is a locally aggressive primary bone tumor. Total en bloc spondylectomy has never been reported to treat thoracic GCT combined with secondary ABC. We retrospectively reviewed two cases of spinal GCT combined with secondary ABC. A 41-year-old male patient was presented with back pain due to irregular expansive bone destruction involving the T6 vertebral body and intraspinal space-occupying lesion. Total en bloc spondylectomy of T6 vertebra was performed with good neurological status after the surgery. A 29-year-old female patient was presented with right scapular region pain due to irregular expansive bone destruction involving the T5 vertebral body and intraspinal space-occupying lesion. Total en bloc spondylectomy of T5 vertebra was performed with good neurological status after the surgery. Adjuvant radiation therapy was applied after the surgery without local recurrence at the 12-month or 24-month follow-up. Spinal GCT combined with secondary ABC appears to have a high local recurrence rate. Therefore, total en bloc spondylectomy should be applied to treat thoracic GCT combined with secondary ABC.
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