Multicentric giant cell tumour of bone

Although giant cell tumour (GCT) is seen quite frequently, multicentric giant cell tumour (MCGCT) is a rare entity occurring in less than 1% of patients with GCT. The pathogenesis of MCGCT is debated; various mechanisms have been postulated, including contiguous spread, iatrogenic tumour cell seeding, benign metastasis, malignant transformation and de novo formation. A literature review revealed 101 cases of MCGCT reported worldwide, of which we could trace and review 83 cases. We noted that MCGCT, unlike the solitary GCT, more frequently involves the short bones of the hand and feet and is commoner in the meta-diaphyseal region of long bones. The present literature review noted a higher incidence in females and skeletally immature patients (21%). Individual lesions in a patient with MCGCT are radiologically and histologically indistinguishable from the solitary GCT. In our review we noted 42 recurrences in 157 lesions (26%), thus negating the commonly held point of view that MCGCT was clinically more aggressive. Four lung metastases and two histologically malignant lesions were found. The literature does not define the exact time period beyond which a lesion can be classified as metachronous; however a significant number of the subsequent lesions occur within 2-3 years of the index lesion. We recommend from our review, that with the present state of knowledge, special care should be taken in cases with primary meta-diaphyseal lesions, GCTs seen at atypical locations, and in females of younger age group, to ensure that multicentricity is picked up earlier.     

Multinucleated giant cells in cultured giant cell tumor of bone

Further observations on multinucleated giant cells (MGCs) in 12 giant cell tumors of bone (GCT) were made, by means of tissue culture, electron microscopy and immunohistochemistry. In continuous in vitro culture, two distinct types of MGCs were found and herein termed preliminarily short-lived MGCs and long-lived MGCs respectively. The former type had limited life span of about 2-3 weeks, whereas the latter type of MGCs maintained growth or continual formation. They had entirely different appearance and characteristics. This fact reflects two types of MGCs exist in GCT in vivo. Besides, the origin of MGCs were also investigated and discussed in considerable depth.     

Imaging of giant cell tumor of bone

Giant cell tumor (GCT) of bone is a benign but locally aggressive and destructive lesion generally occurring in skeletally mature individuals. Typically involving the epiphysiometaphyseal region of long bones, the most common sites include the distal femur, proximal tibia and distal radius. On radiographs, GCT demonstrates a lytic lesion centered in the epiphysis but involving the metaphysis and extending at least in part to the adjacent articular cortex. Most are eccentric, but become symmetric and centrally located with growth. Most cases show circumscribed borders or so-called geographical destruction with no periosteal reaction unless a pathological fracture is present. There is no mineralized tumor matrix. Giant cell tumor can produce wide-ranging appearances depending on site, complications such as hemorrhage or pathological fracture and after surgical intervention. This review demonstrates a spectrum of these features and describes the imaging characteristics of GCT in conventional radiographs, computerized tomography scans, magnetic resonance imaging, bone scans, positron emission tomography scans and angiography.     

An unusual giant cell tumour of bone

Summary This is a report of an 18-year old girl with an unusual giant cell tumour. Although it is difficult to distinguish between a multifocal giant cell tumour of bone and a primary giant cell tumour with metastases in other bones, we believe that our case is either a multicentric or a multifocal giant cell tumour.

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Résumé Présentation d'une tumeur à cellules géantes de type inhabituel chez une jeune fille de 18 ans. Bien qu'il soit difficile de distinguer une tumeur à cellules géantes à foyers multiples d'une tumeur primitive accompagnée de métastases au niveau d'autres os, il semble bien que ce cas soit une tumeur à cellules géantes multifocale.

     

脊柱骨巨细胞瘤的综合治疗

脊柱骨巨细胞瘤是一种原发于脊柱的良性肿瘤,血运丰富,侵袭性生长,易复发,并可发生肺转移,因此治疗难度大.文中综述了各种治疗方法及其效果,包括外科治疗、放射治疗、动脉栓塞治疗及其发生肺转移时的治疗方法.结果显示:目前,对于脊柱骨巨细胞瘤,最有效的治疗方法是广泛或边界的整块切除,如果不能达到边界切除,则应辅助放射治疗,消灭可能残留的肿瘤细胞,巨大的骶骨骨巨细胞瘤可采用连续动脉内栓塞治疗.对于发生肺转移的脊柱骨巨细胞瘤,可以通过肺叶切除和(或)采用化疗来控制.     

Radiation therapy for giant cell tumors of bone

For giant cell tumors of bone, does radiotherapy provide a safe and effective treatment? This retrospective review includes 24 patients with 26 histologically diagnosed tumors treated with megavoltage radiotherapy between March 1972 and July 1996. Of the 10 recurrent tumors, five had an intralesional resection, two had a biopsy, and three had no biopsy before radiotherapy. Of the 16 previously untreated tumors, one was irradiated after a marginal resection, five after an intracapsular resection, and 10 after biopsy alone. The total doses ranged from 35 to 55 Gy (median, 43 Gy) in fractions of 1.67 to 2.33 Gy per day. Twenty of 26 tumors (77%) were controlled locally. All of the local recurrences occurred within the irradiated field. Five of six patients with local recurrence were treated successfully with additional surgery. Salvage surgery after local recurrence required amputation of an extremity in three patients and a total knee replacement in one patient. The ultimate local control rate was 96% with one patient alive with progressive disease. Lung metastases in one patient were treated successfully with surgery, chemotherapy, and radiotherapy. In one patient a radiation-induced sarcoma developed 22 years after treatment. The authors conclude that radiation therapy is a safe and effective treatment option for benign giant cell tumors of bone. A total dose greater than 40 Gy is the only variable found to significantly influence local control.     

骨巨细胞瘤治疗进展

骨巨细胞瘤是一种局部具有侵袭性、溶骨活性的良性骨肿瘤,传统治疗手段为囊内刮除植骨,但有很高的局部复发率,通过辅助手段对瘤腔进行处理,有效降低了局部复发;对于不同部位骨巨细胞瘤应依据病变部位、大小、侵犯范围、复发率的高低可选择不同的手术方法,包括假体置换、广泛切除、En bloc切除等;对于特殊部位预计手术风险大、不能完整切除患者可行动脉栓塞,有利于病情得到控制或手术;双磷酸盐、地诺单抗的应用给骨巨细胞瘤的治疗带来新的希望,可有效降低肿瘤复发,目前主要应用于复发、难治、特殊部位、转移性骨巨细胞瘤的治疗。化疗主要应用于转移、恶性骨巨细胞瘤的治疗,放疗对于复发或难以手术切除部位骨巨细胞瘤可控制肿瘤进展,但有恶变可能。双磷酸盐、地诺单抗缺乏长期随访,远期疗效尚不明确,骨巨细胞瘤治疗领域新方法、新药的研发为其带来了更多希望。     

Local recurrences in giant cell tumour of bone

Summary. We studied the value of histopathological grading in determining the prognosis of giant cell tumour (osteoclastoma) and the rate of local and distant recurrences in a consecutive series of 31 patients. We found that grading had no prognostic value. Eighteen patients were treated by intralesional curettage and 13 by wide excision. Ten patients (56%), who were all treated by curettage, had local recurrences, but none of the tumours with wide excision recurred (p <0.05). Five (16%) had local recurrences as well as distant metastases, usually to the lungs. The recurrences developed later than an average of 12 years after primary treatment in 3 patients. Wide excision and life-long follow up should be considered in the management of these tumours. Résumé. Nous avons étudié l’interêt pronostic du classement anatomo-pathologique des tumeurs à cellules géantes (osteoclastomes) et le taux de récidives à long terme, locales et à distance, dans une série consécutive de 31 patients. Le classement anatomo-pathologique n’a eu aucun interêt pronostic. Dix-huit patients furent traités par curetage, alors que 13 patients furent traités par une excision large. Dix patients (56%), tous traités par curetage, récidivèrent localement, alors qu’aucune des tumeurs traitées par excisision large ne récidiva (p <0.05). Cinq patients (16%) récidivèrent localement et à distance, généralement au niveau pulmonaire. Chez 3 patients, la récidive se déclara en moyenne 12 ans après le traitement initial. L’excision large avec suivi à vie est nécessaire dans ces rares cas de tumeur osseuse. Accepted: 14 September 1995     

The study of giant cell tumors in bone

Twelve samples of giant cell tumor of bone were incubated in the authors' laboratory. The activity of the cells was documented by means of time lapse cinemicrography. The multinuclear giant cells (MGCs) with undegenerating nuclei migrating from the explants had active ameboid movement and continuously changed their shapes. The majority of them kept splitting themselves into smaller MGCs until mononuclear cells formed, which were indistinguishable from the original stromal cells in morphology and could take up tritiated thymidine as shown by autoradiography. This splitting process of MGCs is mainly responsible for their vanishing in culture. The authors believe that an MGC is one of the true neoplastic elements. MGCs are present merely in the form of a syncytium derived from neoplastic stromal cells. In culture, the factors maintaining the syncytium are lost and the process opposite to cell fusion appears. Therefore, from a morphological view, MGCs and neoplastic stromal cells are homologous.     

Radiosensitive giant cell tumour of the sphenoid bone

Giant cell tumours rarely occur in the cranial region. We encountered a radiosensitive giant cell tumour of the sphenoid in a 12-year-old girl. After a two-stage operation, the residual tumour regrew rapidly. The adjuvant radiotherapy subsequent to additional surgery has suppressed the growth of the residual tumour for 5 years.     

骨巨细胞瘤细胞生物学研究进展

骨巨细胞瘤(GCT)血管生成、局部浸润、细胞生物学方面的研究,近年有了新的进展.研究表明促血管内皮生长因子不仅可促进肿瘤血管生成,还可直接调节肿瘤细胞增殖;对基质金属蛋白酶在肿瘤细胞局部侵袭中的作用提出了怀疑;增殖调节基因p21、p27及细胞周期素D1之间的相互作用调节了肿瘤细胞增殖和巨细胞形成等;GCT可能来自成骨细胞谱系.这些新认识对GCT治疗、预后判断提供了帮助.     

骨巨细胞瘤诊断与治疗现状

骨巨细胞瘤(GCT)是最常见的原发性骨肿瘤之一.早期研究表明,病灶简单刮除、植骨术后关节功能满意,但局部复发率很高.随着更为先进的影像技术和各种物理化学辅助措施如液氮冷冻、骨水泥等的应用,GCT复发率降低,预后改善.GCT瘤体整块切除适用于部分患者,使复发率更低,但需行复杂的重建手术.普遍认为,GCT病理学及影像学分期...     

关于多中心骨巨细胞瘤的系统回顾

骨巨细胞瘤是一种具有侵袭性的良性骨肿瘤,常为单发病灶,多中心发病者罕见,报道以个案为主。为进一步分析多中心骨巨细胞瘤的基本特点,总结其治疗方法和预后情况,作者搜集了国内外数据库中关于多中心骨巨细胞瘤的病例研究,对其做出了系统的分析和归纳,旨在为临床医生提供更多参考。多中心骨巨细胞瘤的发病年龄相对年轻,好发于骨骼未成熟的患者,女性多于男性,干骺端及手足骨的发病率高于单发者。病理组织学检查是明确诊断的最终手段,对患者进行系统的影像和核医学检查可以避免漏诊,在治疗时要求坚持对各个病灶独立评估其特点,确定治疗方案。多中心骨巨细胞瘤患者及高危患者应坚持长期随访。     

Virus-like intranuclear inclusions in giant cell tumor of bone

An ultrastructural study of 13 cases of typical giant cell tumor of bone (GCT) revealed the presence of virus-like intranuclear inclusions (INI), morphologically identical to those reported in Paget's disease of bone. In two giant cells of two different patients, INI were found only after careful survey of a great number of cells. This finding, coupled with the scarcity of GCT with similar inclusions reported in the literature, reveals the extreme rarity of this finding and leaves open to discussion the specificity of these INI even in Paget's disease, as well as the possible etiological significance in giant cell tumor of bone.