Adamantinoma

金刚烷瘤
  • 文章类型: Case Reports
    Adamantinoma是一种罕见的,主要影响长骨的局部侵袭性骨肿瘤,对胫骨有好感.尽管被认为是低度恶性肿瘤,金刚烷胺瘤因其高复发倾向而臭名昭著,这对受影响个体的管理提出了重大的临床挑战。本病例报告旨在探讨复发性胫骨金刚烷胺瘤的复杂性,阐明其临床表现,诊断方式,治疗策略,和预后因素。
    本病例报告旨在评估复发性金刚瘤切除和重建后的手术和功能结果。
    本回顾性病例报告包括在ShaukatKhanum纪念肿瘤医院和拉合尔研究中心就诊的复发性金刚烷胺瘤患者。数据收集后,患者的书面和知情同意,和患者的人口统计学的回顾性分析是通过社会科学统计软件包使用医院信息系统中的信息和预先设计的形式。组织学载玻片从病理学系收集。包含的变量被分类为人口统计,历史和检查结果,site,和原发性肿瘤的大小,放射学发现,组织学类型,舞台,和切除边缘。患者接受了自由血管化腓骨移植的广泛切缘切除和重建,并使用肌肉骨骼肿瘤社会评分评估了其功能预后,该评分显示出良好的功能预后。
    尽管面临治疗挑战,广泛切除肿瘤,然后进行游离血管化腓骨移植重建是治疗无法获得高级假体和同种异体移植物的患者的可行选择。
    UNASSIGNED: Adamantinoma is a rare, locally aggressive bone tumor that primarily affects the long bones, with a predilection for the tibia. Although considered a low-grade malignancy, adamantinoma is notorious for its high propensity for recurrence, which poses significant clinical challenges in the management of affected individuals. This case report aims to explore the intricacies of recurrent adamantinoma of the tibia, shedding light on its clinical presentation, diagnostic modalities, treatment strategies, and prognostic factors.
    UNASSIGNED: This case report aims to evaluate surgical and functional outcomes of post-excision and reconstruction in recurrent adamantinoma.
    UNASSIGNED: This retrospective case report includes the patient who presented in Shaukat Khanum Memorial Cancer Hospital and Research Center Lahore with recurrent adamantinoma. Data were collected after patient\'s written and informed consent, and a retrospective analysis of the patient\'s demographics was done with the Statistical Package for the Social Sciences using the information in the Hospital Information System with a predesigned pro forma. Histology slides were collected from the Department of Pathology. Variables that were included are categorized into demographics, history and examination findings, site, and size of the primary tumor, radiological findings, histological type, stage, and resection margins. The patient underwent wide margin excision and reconstruction with free vascularized fibula graft and his functional outcome was evaluated using the musculoskeletal tumor society score which showed good functional outcome.
    UNASSIGNED: Despite therapeutic challenges, wide excision of the tumor followed by free vascularized fibula grafting for reconstruction is a viable option to treat such patients where advanced prosthesis and allografts are not available.
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  • 文章类型: Journal Article
    我们介绍了一例起源于腓骨的金刚砂瘤,其软组织成分约为6厘米。临床,放射学,病理研究最初表明,该肿瘤可能是侵入骨的滑膜肉瘤。据我们所知,文献中没有其他病例报道过具有如此大的软组织成分的腓骨adamantinoma。
    We present a case of adamantinoma that originated from the fibula and had a large soft tissue component measuring approximately 6 cm. Clinical, radiological, and pathological investigations initially suggested that the tumor might be a bone-invading synovial sarcoma. To the best of our knowledge, no other case of fibular adamantinoma with such a large soft tissue component has been reported in the literature.
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  • 文章类型: Case Reports
    Adamantinmas罕见,低档,恶性骨肿瘤仅占原发性恶性骨肿瘤的0.33-0.48%。
    方法:一名11岁的尺骨金刚砂男孩接受整块切除术治疗,氪石骨水泥,和钢板固定。手术导致明显的疼痛缓解和良好的功能恢复。术后5年无复发迹象,肌肉骨骼肿瘤学会得分为93分。
    这个11岁的患者曾接受过动脉瘤样骨囊肿(ABC)治疗,说明了诊断金刚烷虫瘤的复杂性,尤其是尺骨等非典型部位.整体切除和合成骨移植的成功使用凸显了准确诊断和先进手术方法在小儿骨科肿瘤学中取得良好结果的重要性。
    结论:尺骨adamantinoma罕见且难以诊断。这种肿瘤的成功治疗,正如这个案例报告中所描述的,可以帮助指导今后类似案件的管理。
    UNASSIGNED: Adamantinomas are rare, low-grade, malignant skeletal tumors accounting for only 0.33-0.48 % of primary malignant bone tumors.
    METHODS: An 11-year-old boy with adamantinoma of the ulna was treated with en bloc resection, kryptonite bone cement, and plate fixation. The surgery resulted in marked pain relief and good functional recovery. No evidence of recurrence was observed for 5 years postoperatively, and the Musculoskeletal Tumor Society score was 93.
    UNASSIGNED: This case of an 11-year-old previously treated for an aneurysmal bone cyst (ABC) illustrates the complexity of diagnosing adamantinoma, especially in atypical locations like the ulna. The successful use of en bloc resection and synthetic bone graft highlights the importance of accurate diagnosis and advanced surgical methods in achieving favorable outcomes in pediatric orthopedic oncology.
    CONCLUSIONS: Ulnar adamantinomas are rare and can be difficult to diagnose. The successful treatment of this tumor, as described in this case report, can help guide the management of similar cases in the future.
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  • 文章类型: Case Reports
    Adamantinoma是一种罕见的,低度恶性骨肿瘤,主要影响胫骨,并且由于其非特异性影像学特征,通常存在诊断挑战。
    一名55岁的军事人员,没有外伤史,有一年右腿肿胀和疼痛的病史。放射学检查显示右前胫骨,中端骨干溶解性扩张性病变伴内部小梁和切除活检可诊断为金刚烷虫瘤。膝盖以下截肢是确定的治疗方法。
    尽管金刚烷胺瘤存在诊断挑战,患者被转诊至肿瘤科并接受截肢手术,强调在持续性骨病变的鉴别诊断中考虑金刚砂瘤的重要性。
    UNASSIGNED: Adamantinoma is an infrequent, low-grade malignant bone tumor, predominantly affecting the tibia and often presents diagnostic challenges due to its nonspecific radiographic characteristics.
    UNASSIGNED: A 55-year-old military personnel, with no history of trauma, who presented with a one-year history of right leg swelling and pain. Radiological examination showed right anterior tibial, mid diaphyseal lytic expansile lesion with internal trabeculations and excisional biopsy led to the diagnosis of adamantinoma. Below knee amputation was done as a definitive management.
    UNASSIGNED: Despite diagnostic challenges adamantinoma presents, the patient was referred to oncology and underwent amputation, underscoring the importance of considering adamantinoma in differential diagnosis for persistent bone lesions.
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  • 文章类型: Case Reports
    背景技术金刚烷胺瘤是一种罕见的低度恶性骨肿瘤,通常发现于胫骨干骨干和干phy端,与下颌成釉细胞瘤的组织学相似性。复发性金刚瘤的最有效治疗方法尚不清楚。该报告是一名22岁的女性,患有酪氨酸激酶抑制剂帕唑帕尼治疗的复发性胫骨金刚瘤。病例报告我们报告了一例22岁的女性,她因右侧胫骨的可疑骨病变而被转诊到我们中心。骨活检结果与金刚烷瘤一致。整块切除术成功完成,术后无并发症。五年后,发射断层扫描阳性显示,在先前病变区域附近和右腹股沟淋巴结中示踪剂摄取轻度增加。病变和腹股沟淋巴结的活检证实金刚烷瘤复发。由于腹部和盆腔转移,病人接受了手术切除,伴随着阑尾切除术,右输卵管卵巢切除术,术中放射治疗,和腹腔热化疗。随后,患者接受帕唑帕尼治疗4个月;然而,化疗4个月后肿瘤继续恶化.目前,患者正在接受吉西他滨和多西他赛作为二线药物治疗.结论该报告显示,帕唑帕尼作为独立治疗似乎对患者预后没有希望的作用。据我们所知,这是帕唑帕尼治疗金刚烷瘤的第二篇报道。
    BACKGROUND Adamantinoma is a rare low-grade malignant bone tumor, usually found in the tibial diaphysis and metaphysis, with histological similarities to mandibular ameloblastoma. The most effective treatment of recurrent adamantinoma is not yet clear. This report is of a 22-year-old woman with recurrent tibial adamantinoma treated with the tyrosine kinase inhibitor pazopanib. CASE REPORT We report the case of a 22-year-old woman who was referred to our center for a suspicious bone lesion in the right tibia. Bone biopsy findings were consistent with an adamantinoma. En bloc resection was completed successfully, with no postoperative complications. Five years later, a positive emission tomography scan revealed mildly increased tracer uptake near the area of the previous lesion and in the right inguinal lymph node. Biopsies of the lesion and inguinal lymph node confirmed recurrence of the adamantinoma. Due to abdominal and pelvic metastasis, the patient underwent surgical debulking, along with an appendectomy, right salpingo-oophorectomy, intraoperative radiation therapy, and hyperthermic intraperitoneal chemotherapy. Subsequently, the patient was placed on pazopanib for 4 months; however, her tumor continued to worsen after 4 months of chemotherapy. Currently, the patient is receiving gemcitabine and docetaxel as second-line medical therapy. CONCLUSIONS This report showed that pazopanib as standalone treatment does not appear to have promising role on patient outcomes. To the best of our knowledge, this is the second report of pazopanib in the treatment of adamantinoma.
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  • 文章类型: Case Reports
    Adamantinoma,构成原发性骨肿瘤的一小部分,由于其不明确的组织发生,因此提出了诊断挑战。本报告概述了一个独特的病例,涉及一名27岁的女性,有右胫骨金刚烷胺瘤的病史,表现为双侧肺栓塞并转移至卵巢和盆腔淋巴结。五年前的整块切除术后,作为姑息措施,患者接受了减积手术,包括腹腔热灌注化疗(HIPIC)和术中放疗(IORT).该手术实现了骨盆肿瘤的实质性缩小,随后的随访表明良好的术后轨迹。该病例强调了金刚烷胺瘤罕见地转移到卵巢和骨盆,作为第一个报告的实例,揭示了多模式姑息治疗方法的挑战和潜在好处。需要进一步的研究来完善转移性金刚烷瘤的治疗策略并提高患者的预后。
    Adamantinoma, constituting a minute fraction of primary bone tumors, poses a diagnostic challenge due to its ambiguous histogenesis. This report outlines a distinctive case involving a 27-year-old female with a history of right tibial adamantinoma, presenting with bilateral pulmonary emboli and metastasis to the ovaries and pelvic lymph nodes. Following en bloc resection five years earlier, the patient underwent debulking surgery with hyperthermic intraperitoneal chemotherapy (HIPIC) and intraoperative radiotherapy (IORT) as a palliative measure. The procedure achieved substantial pelvic tumor reduction, and subsequent follow-ups indicated a favorable postoperative trajectory. This case underscores the rarity of adamantinoma metastasis to the ovaries and pelvis, being the first reported instance, shedding light on the challenges and potential benefits of a multimodal palliative approach. Further research is warranted to refine treatment strategies for metastatic adamantinoma and enhance patient outcomes.
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  • 文章类型: Case Reports
    釉质瘤(adamantinoma)是一种罕见的具有双相分化特征的骨肿瘤,发生于长骨,也称为长骨釉质瘤。2020年第5版WHO骨肿瘤分类将釉质瘤分为经典型、去分化型和骨纤维结构不良(osteofibrous dysplasia,OFD)样型,OFD样型釉质瘤ICD-O编码为9261/1,是该版分类新增的中间型(局部侵袭性)肿瘤。本文报道1例OFD样型釉质瘤,并结合相关文献复习其病理形态学特征、诊断及鉴别诊断要点,以进一步提高广大医务工作者对该罕见肿瘤的认识。.
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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: Review
    ALES是一种罕见的亚型,证明了ES的EWSR1-FLI1易位特征,并证明了复杂的上皮分化,包括弥漫性细胞角蛋白和p40表达。它主要在头部和颈部被识别,在中年人群中很常见。这个病例是胰腺尾部报告的第一例ALES,在头部和颈部与ALES共享一些形态特征,包括单调的细胞学,渗透生长模式,复杂的上皮样分化,但头部和颈部的ALES通常具有高级组织学特征(例如,坏死,高有丝分裂率,等。),也可能发生突然的角质化,但这些特征并未反映在原发性胰腺尾部ALES中.尽管在胰尾和头颈部出现的ALES具有相同的免疫组织化学和分子特征,我们的病例可以为胰尾ALES的鉴别诊断提供新的思路,促进对ALES的认识和理解。
    ALES is a rare subtype that demonstrates the EWSR1-FLI1 translocation characteristic of ES and demonstrates complex epithelial differentiation including diffuse cytokeratin and p40 expression. It has predominantly recognized in the head and neck and is common in middle-aged population. This case is the first case of ALES reported in the pancreatic tail, sharing some morphological characteristics with ALES in the head and neck, including monotonous cytology, infiltrative growth pattern, and complex epithelioid differentiation, but ALES in the head and neck often has high-grade histological features (e.g., necrosis, high mitotic rate, etc.), and sudden keratinization can also occur, but these features were not reflected in this primary pancreatic tail ALES. Although ALES arising in the pancreatic tail and in the head and neck sites share the immunohistochemical and molecular profile, our case can provide new ideas in differential diagnosis of ALES arising in pancreatic tail and promote increased recognition and understanding of ALES.
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  • 文章类型: Case Reports
    背景:巨细胞瘤(GCT)是一种良性骨肿瘤,通常见于成熟长骨的骨phy骨或干is端。分散在单核梭形细胞和单核细胞中的多个大型多核巨细胞构成了骨GCT(GCTB)的特征性组织学背景。
    方法:我院收治一名15岁女孩,主诉为左腿疼痛和肿胀,行走困难2年。在左腿的X光片上,溶骨,膨胀性,胫骨干骨干上有硬化骨缘的偏心性病变,提示骨纤维发育不良。MRI显示发现与金刚烷瘤相符。随后的组织学报告相当令人惊讶,与骨巨细胞瘤一致.在高速毛刺的帮助下进行扩大的病灶内刮治,然后进行化学烧灼和植骨。患者随访2年。患者可以在没有帮助或任何复发迹象的情况下正常行走。
    结论:GCTB通常影响人的第三和第四个十年的生活,并涉及长骨的骨phy,但这是一例骨干GCT,在15岁的时候.诊断GCT具有挑战性,如果出现在不寻常的位置,除非经组织病理学检查证实。
    结论:当GCT发生在不常见的位置时,需要多学科的方法来正确诊断GCT。对于治疗的成功结果,必须进行早期诊断并进行适当的治疗和长期随访。
    BACKGROUND: Giant cell tumor (GCT) is a benign bone tumor typically seen in epiphysis or metaphysis of mature long bones. Multiple large multinucleated giant cells dispersed among mononuclear spindle cells and monocytes constitute characteristic histological background of GCT of bone (GCTB).
    METHODS: A 15-year-old girl was admitted to our hospital with the complaint of pain and swelling in the left leg with difficulty in walking for 2 years. On X-ray of the left leg, osteolytic, expansile, eccentric lesion with sclerotic bone margin on the diaphysis of the tibia was seen suggesting oesteofibrous dysplasia. MRI demonstrated findings compatible with adamantinoma. The subsequent histology report was rather surprising, consistent with giant cell tumor of the bone. Extended intralesional curettage was done with the help of a high-speed burr followed by chemical cauterization and bone grafting. The patient was followed up for 2 years. The patient could walk normally without assistance or any signs of a recurrence.
    CONCLUSIONS: GCTB commonly affects people in their third and fourth decades of life and involves epiphysis of the long bone, but this is a case of diaphyseal GCT, at an age of 15 years. It is challenging to diagnose GCT, if present in an unusual location, unless confirmed by histopathological examinations.
    CONCLUSIONS: A multi-disciplinary approach is required to correctly reach the diagnosis of GCT when it happens to be in an uncommon location(s). Early diagnosis with appropriate treatment and long-term follow-up is mandatory for the successful outcome of the treatment.
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