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(AD)是一种罕见的,生长缓慢的原发性恶性骨肿瘤,其特征是上皮细胞簇和梭形细胞骨纤维成分的双相形态。已提出AD与骨纤维发育不良(OFD)之间的强关系,而纤维发育不良(FD)很少与AD相关。我们提出了一个AD病例,该病例在6年内进行了3次随访和组织学评估,并具有不同的形态学模式。原发活检和完全切除后的肿瘤表现出典型的AD和骨纤维样模式,而复发性病变仅表现为梭形细胞形态,因此被诊断为FD。然而,在所有3个病变中进行的广泛的免疫组织化学分析显示,对全细胞角蛋白有很强的反应性,波形蛋白,p63和podoplanin,这是AD的特征。虽然,在第一次复发的FD样肿瘤切片中,podoplanin的阳性率强于pancitokeratin,梭形细胞呈可变阳性。本病例突出了诊断AD的问题,基于单一的活检,其中一个肿瘤的组成部分占主导地位的另一个,同时强调了当(骨)纤维性病变的组织病理学特征与AD相关时,对角蛋白和足planin进行免疫组织化学染色的重要性。
    Adamantinoma (AD) is a rare, slow-growing primary malignant bone tumor characterized by a biphasic morphology of clusters of epithelial cells and spindle cell osteofibrous components. A strong relationship between AD and osteofibrous dysplasia (OFD) has been proposed, while fibrous dysplasia (FD) has been rarely associated with AD. We present an AD case that was followed and histologically evaluated 3 times over 6 years with different morphological patterns. The tumor in the primary biopsy and after complete resection showed classical features of AD and osteofibrous-like pattern, while the recurrent lesion presented with exclusively spindle cell morphology and was thus diagnosed as FD. However, the extensive immunohistochemical analysis in all 3 lesions revealed strong reactivity for pancytokeratin, vimentin, p63, and podoplanin, which are characteristic for AD. Although, in the FD-like section of the tumor from the first recurrence the positivity of podoplanin was stronger than pancitokeratin, which was variably positive on spindle cells. The present case highlights the problem of diagnosing AD based on a single biopsy with one tumor\'s component predominating over the other, and at the same time emphasizes the importance of using immunohistochemical staining for keratin and podoplanin when the histopathological features of (osteo)fibrous lesion can be linked to AD.
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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: Journal Article
    骨纤维发育不良(OFD)是一种罕见的,良性,自限性骨病发病率相对较低,约占所有原发性骨肿瘤的0.2%。经常在胫骨中轴的皮质内发现。OFD也可以发生在其他骨骼区域,包括腓骨,尺骨,半径,股骨,肱骨,坐骨,肋骨,tarsus,meta骨,椎骨,和头饰。OFD可以表现为无症状,质量,疼痛,肿胀,畸形,甚至是病理性骨折.OFD可能被误诊为金刚烷瘤(AD),因为它们是来自同一骨肿瘤家族的三种亚型,并且具有相似的影像学特征。此外,病理学可以为OFD的准确诊断提供证据,但由于取样材料少,可能会出现误诊。迄今为止,很少有研究全面介绍流行病学,临床表现,发病机制,放射学特征,病理学,和OFD的治疗。我们在这里讨论临床症状,诊断方法,以及OFD的治疗方案,以提高对OFD的理解,这有助于准确诊断和适当治疗。
    Osteofibrous dysplasia (OFD) is a rare, benign, self-limited bone disorder with a relatively low incidence, accounting for approximately 0.2% of all primary bone tumors. It was frequently found intra-cortical of the mid-shaft of the tibia. OFD can also occur in other skeletal regions, including the fibula, ulna, radius, femur, humerus, ischium, rib, tarsus, metatarsals, vertebral, and capitate. OFD can present with asymptomatic, mass, pain, swelling, deformity, and even pathological fracture. OFD might be misdiagnosed as adamantinoma (AD) and because they are three subtypes origin from the same family of bone tumors and have similar imaging features. Moreover, pathology could provide evidence for an accurate diagnosis of OFD, but misdiagnosis may occur due to small sampling materials. To date, few studies have comprehensively introduced the epidemiology, clinical manifestations, pathogenesis, radiological features, pathology, and treatment for OFD. We herein discuss clinical signs, diagnosis methods, and treatment options of OFD to improve the understanding of OFD, which is helpful for accurate diagnosis and appropriate treatment.
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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: Journal Article
    背景:金刚烷胺瘤是一种罕见的恶性骨肿瘤。由于发病率低,关于金刚烷瘤的临床结果的报道很少。
    目的:本研究旨在使用来自国家骨和软组织肿瘤注册中心的数据阐明金刚烷胺瘤患者的预后。
    方法:从2006年至2019年,纳入38例胫骨起源患者。二十四个是男性,十四个是女性,平均年龄37(6-87)岁,平均随访35(1-128)个月。
    结果:手术33例(87%)(刮宫:4例,广泛切除:27例,截肢:2例)。对27例接受广泛切除的患者进行了重建。11例患者共进行了12次手术。增加手术的主要原因是6例移植骨不连。肿瘤学结果为DOC(其他原因死亡)1例,NED(无疾病证据)37例。
    结论:在日本,金刚烷胺瘤的治疗效果非常好。这可能部分是由于大量病例广泛切除。
    BACKGROUND: Adamantinomas are rare malignant bone tumors. Due to their low incidence, there are few reports on the clinical results of adamantinoma.
    OBJECTIVE: This study aims to clarify outcomes in patients with adamantinoma using data from the National Bone and Soft Tissue Tumor Registry.
    METHODS: From 2006 to 2019, 38 cases of tibial origin were included. Twenty-four were male and 14 were female, with a mean age of 37 (6-87) years and a mean follow-up of 35 (1-128) months.
    RESULTS: Surgery was performed in 33 cases (87%) (curettage: 4 cases, wide resection: 27 cases, amputation: 2 cases). Reconstruction was performed in 27 patients who underwent wide resection. A total of 12 additional surgeries were performed in 11 patients. The main reason for the additional surgeries was nonunion of grafting bone in 6 cases. Oncologic outcomes were DOC (death from other causes) in one case and NED (no evidence of disease) in 37 cases.
    CONCLUSIONS: The results of treatment of adamantinomas in Japan have been extremely favorable. This may be due in part to the large number of cases with wide resection.
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  • 文章类型: Case Reports
    Adamantinoma,罕见的低度原发性恶性骨肿瘤,青少年很少引起腿部疼痛,通常表现在下肢,明显偏爱胫骨,虽然发生在其他骨骼,如股骨,腓骨,和骨盆都有记录.局部复发和区域转移的情况很少见。本病例报告旨在全面回顾临床表现,成像特征,组织学发现,和金刚烷瘤的管理。该病例涉及一名17岁的男性患者,有4年的右前腿水肿和不适史。胫骨近端影像学检查显示,扩张性溶血性硬化性病变,有多个隔膜和部分硬化边界。随后的磁共振成像(MRI)证实了病变的性质,还有活检,然后进行组织学分析,确诊为金刚砂瘤。这个案例突出了多学科方法的重要性,强调放射科之间的密切合作,病理学,和整形外科肿瘤学在金刚砂瘤管理中的应用。长期随访对于监测复发和及时给予治疗至关重要。本病例报告的目的是促进对金刚烷胺瘤的了解,并为这种罕见的骨肿瘤的治疗提供指导。
    Adamantinoma, an uncommon low-grade primary malignant bone tumor, rarely causes leg pain in adolescents and typically manifests in the lower extremities, with a notable preference for the tibia, although occurrences in other bones such as the femur, fibula, and pelvis have been documented. Instances of local recurrence and regional metastasis are infrequent. This case report aims to comprehensively review the clinical presentation, imaging features, histological findings, and management of adamantinoma. The presented case involves a 17-year-old male patient with a four-year history of edema and discomfort in the right anterior leg. Radiographic examination of the proximal tibia revealed a well-defined, expansile lytic-sclerotic lesion with multiple septae and a partially sclerotic border. Subsequent magnetic resonance imaging (MRI) confirmed the nature of the lesion, and a biopsy, followed by histological analysis, confirmed the diagnosis of adamantinoma. This case highlights the significance of a multidisciplinary approach, emphasizing close collaboration among radiology, pathology, and orthopedic oncology in adamantinoma management. Long-term follow-up is imperative for monitoring recurrence and administering timely therapy. The objective of this case report is to contribute to an improved understanding of adamantinoma and offer guidance on the treatment of this uncommon bone tumor.
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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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