Giant cell tumor of bone in a child

Giant cell tumor (GCT) of bone was first established as a distinct clinicopathological radiographic entity in 1940 when Jaffe distinguished it from other lesions containing giant cells. GCT is rare in patients under 15 years of age. We report a case of GCT in a 10-year-old boy whose X-ray films showed osteolysis suggesting a malignant bone tumor. We believe this to be the youngest patient with giant cell bone tumor ever reported in the Japanese literature. Received: March 23, 1999 / Accepted: August 19, 1999     

Giant cell tumor of bone: Oncologic and functional results

Giant cell tumor of bone is a challenging surgical problem due to its mostly aggressive growth with tendency to recur locally, to develop in rare instances pulmonary metastases without histologic evidence of malignant changes, and due to its potential to dedifferentiate into a frankly malignant tumor in a limited number of patients. It is treated in many different ways because of the difficulties in finding a type of treatment with the best functional results without compromising oncologic results. This paper describes 19 patients with giant cell tumor of bone. Following 19 procedures (including 6 intracapsular resections [curettage]) in 17 patients in our hospital only 2 recurrences (10.5%) occurred, both after curettage. Functional results after curettage without recurrence were favorable. Marginal or wide resections did not result in any recurrence, but were functionally inferior to curettage; an exception to the latter was the resection-arthrodesis of the distal radius in one patient. © 1994 Wiley-Liss, inc.     

Giant cell tumor of bone: oncological and functional results of long-term follow-up

BACKGROUND: Giant cell tumor (GCT) of bone is a rare and unpredictable lesion. Its standard treatment has ranged from surgical curettage to wide resection and varying oncological and functional results have been reported. METHODS: A retrospective review of 47 patients (17 males and 30 females) with GCT of bone was performed to evaluate the oncological and functional results. The patients were followed up for at least 5 years. The average age of the patients was 32 years (range 15-66 years). The tumor sites were distal femur in 15 cases, proximal tibia in 10, distal radius in five, spinal column in four, proximal femur in three, proximal humerus in three, proximal fibula in two, pelvis in two and others in three. Enneking's surgical stages were Stage 1 in three, Stage 2 in 34 and Stage 3 in 10 cases. In these 47 patients, 80 surgical procedures were performed. RESULTS: The rate of local recurrence was 75% in the 28 patients undergoing intralesional excision, 50% in those receiving excision and curettage and 0% in those receiving wide resection. Although there was no statistical significance, surgical stages tended to be correlated with the local recurrence rate (Stage 1, 0%; Stage 2, 53%; Stage 3, 70%). Functional evaluation was performed according to the most recent system of the Musculoskeletal Tumor Society. Functional results of the patients with extremity tumors were 28.2 (average) in those undergoing intralesional excision, 30 (average) in those receiving excision and curettage and 27.1 (average) in those receiving wide resection. Functional results were significantly correlated with the initial surgical stages (Stage 1, 30; Stage 2, 27.5; Stage 3, 24.4; Kruskal-Wallis test, P = 0.016). CONCLUSIONS: To preserve good function of the extremities and avoid local recurrence, we consider that intralesional excision with adjunctive therapy such as phenol cauterization should be employed for the treatment of benign GCT of bone.      

Giant cell tumor of bone. A clinicopathologic and DNA flow cytometric analysis

Flow cytometric DNA analysis was performed on 60 cases of giant cell tumor of bone and the results were correlated with the clinicopathologic features. Tumors studied were from 31 men and 29 women whose ages ranged from 18 to 62 years (median, 29 years). The most common sites were the distal end of the femur and proximal end of the tibia, accounting for 75% of the lesions. Treatment consisted of resection in 29 patients (48%), curettage with bone chip packing in 15 patients (25%), or curettage with cement packing in 16 patients (27%). Ten patients (17%) had local relapse within 1 to 3 years, and two had lung metastases. Forty-two patients (70%) exhibited tumors with a diploid DNA content, 16 aneuploid (27%), and two tetraploid (3%). Six (37.5%) of the aneuploid patients had relapses: one of those had been treated by resection of the tumor and five by curettage. Of the remaining ten (62.5%) unrelapsed aneuploid patients, nine had been treated by resection of the tumor and one by curettage. Four of the 42 diploid patients (9.5%) had relapses; all had been treated by curettage of the tumor. The two tetraploid tumors were treated by resection and none relapsed. Histologic parameters did not correlate with relapse rate or DNA pattern. Although relapse was more common among aneuploid tumors, our study shows that this appears to be influenced by the treatment modality rather than the ploidy status. Based on this study the DNA analysis of giant cell tumor of bone has a limited utility for predicting the tumor's biologic behavior.     

Giant cell tumor of hard palate

The giant cell tumors usually present in epiphysis of long bones. These are uncommon tumors in head and neck region. These tumors have benign historical features but can be aggressive locally and even can metastasise to lungs. This rare tumor in hard palate is being reported here which was excised transpalatly.     

Giant cell tumor in Paget's disease of bone: familial and geographic clustering.

Four patients with benign giant cell tumor and one patient with probable benign giant cell tumor associated with Paget's disease of bone are reported. The familial and geographic clustering of these cases is unique in that three patients were related and all patients traced ancestral roots to the same area of southern Italy. Tumors arose from the cranial or facial bones in three patients and from vertebral bodies in two patients. All caused symptoms by local compression, and treatment by curettage or radiotherapy was successful in all patients. Three separate tumors in one patient shrank dramatically in response to treatment with high doses of dexamethasone, and one patient whose tumor caused spinal cord compression showed marked improvement in neurologic function on therapy with dexamathasone.     

涉及桡骨干的骨巨细胞瘤1例报道

Giant cell tumor (GCT) of the bone is a benign but locally aggressive & destructive lesion. This tumor is usually seen in patients over 20 years of age. Less than 2% are found in part with open epiphysis. The epiphyseal portion of the bone is characteristic site of giant cell tumor. In rare instances, giant cell tumor can occur in the diaphysis of long tubular bone without involving the epiphysis. Although age, clinical and radiological features are helpful, it is still the histology that helps to clinch the diagnosis. It is important to distinguish giant cell tumor of diaphysis from the giant cell rich lesions, more common in this site.     

Giant cell tumor of the skull.

BACKGROUND. Most giant cell tumors (GCT) occur at the ends of long bones. There is little information about GCT of the skull bones. METHODS. The authors reviewed the Mayo Clinic files, which contained 546 cases of GCT, and their own consultation files, which contained approximately 1500 cases. RESULTS. Eleven tumors occurred in the sphenoid bone with extension to the surrounding bones and structures in 8 patients. One tumor (in Paget's disease) occurred in the frontal bone, one tumor was in the occipital bone, and one tumor was in the temporal bone. There were 4 men and 11 women whose ages ranged from 8 to 78 years, with a mean of 36.5 years. Radiographic findings were not suggestive of a specific diagnosis, although the features were those of an aggressive lesion. Histologically, the tumors had features typical of GCT. However, a prominent spindle cell component was seen in five tumors. The initial treatment in all patients but one was intralesional excision that was as complete as possible. The last patient had a wide excision and had soft tissue recurrence at 1 year. This was excised and she was free of disease at 2.7 years. Three patients died, one in the immediate postoperative period and the other two at 1.6 and 4 years with progression of tumor. One patient had postoperative radiation therapy and was without evidence of disease for 2 years when he was lost to follow-up. The remaining 10 patients all had postoperative radiation therapy; 6 patients were alive without disease from 4 to 34 years. However, one of these six patients had a recurrence that was treated surgically with additional radiation. Four patients were alive with tumor from 2.1 to 26 years at the time of this report. CONCLUSIONS. GCT of the skull bones is rare but should be distinguished from giant cell reparative granuloma because of the tendency for progression. Surgical ablation (as complete as possible) and postoperative radiation therapy seem to be the treatment of choice for GCT of the skull bones.     

Giant cell tumor of bone: a model for the in vitro human osteoclast characterization

The in vitro growth pattern of cells obtained from bioptic material of ten patients with giant cell tumor of bone (GCT) was investigated. Cytochemical reactions and monoclonal antibodies raised against macrophage markers were tested on the two histologically identifiable GCT cell populations. Only monoclonal antibody EBM/11 stained both mononuclear and giant cells. EBM/11 positivity and resistance of acid phosphatase to high doses of tartrate strongly suggest that both mononuclear and giant cells belong to the same lineage.     

Giant cell tumour of the petrous bone.

Giant cell tumours (GCT) are rare in the skull bones. In the cranium the sphenoid bone is the most common site. The petrous bone is a very uncommon location for such tumours. The chance location of GCT at the base of the skull makes total surgery difficult and hazardous. A case of GCT of the petrous bone is presented. The clinical course in the patient and role of primary radiotherapy in GCT in such an unusual site is discussed.     

Giant cell tumour of bone in the denosumab era

Giant cell tumour of bone (GCTB) is an intermediate locally aggressive primary bone tumour, occurring mostly at the meta-epiphysis of long bones. Overexpression of receptor activator of nuclear factor kappa-B ligand (RANKL) by mononuclear neoplastic stromal cells promotes recruitment of numerous reactive multinucleated osteoclast-like giant cells, causing lacunar bone resorption. Preferential treatment is curettage with local adjuvants such as phenol, alcohol or liquid nitrogen. The remaining cavity may be filled with bone graft or polymethylmethacrylate (PMMA) bone cement; benefits of the latter are a lower risk of recurrence, possibility of direct weight bearing and early radiographic detection of recurrences. Reported recurrence rates are comparable for the different local adjuvants (27–31%). Factors increasing the local recurrence risk include soft tissue extension and anatomically difficult localisations such as the sacrum. When joint salvage is impossible, en-bloc resection and endoprosthetic joint replacement may be performed. Local tumour control on the one hand and maintenance of a functional native joint and quality of life on the other hand are the main pillars of surgical treatment for this disease. Current knowledge and development in the fields of imaging, functional biology and systemic therapy are forcing us into a paradigm shift from a purely surgical approach towards a multidisciplinary approach. Systemic therapy with denosumab (RANKL inhibitor) or zoledronic acid (bisphosphonates) blocks, respectively inhibits, bone resorption by osteoclast-like giant cells. After use of zoledronic acid, stabilisation of local and metastatic disease has been reported, although the level of evidence is low. Denosumab is more extensively studied in two prospective trials, and appears effective for the optimisation of surgical treatment. Denosumab should be considered in the standard multidisciplinary treatment of advanced GCTB (e.g. cortical destruction, soft tissue extension, joint involvement or sacral localisation) to facilitate surgery at a later stage, and thereby aiming at immediate local control. Even though several questions concerning optimal treatment dose, duration and interval and drug safety remain unanswered, denosumab is among the most effective drug therapies in oncology.     

Giant cell tumour of the sphenoid and temporal bone

A Giant cell tumour of the sphenoid and temporal bone is a rare entity. It presents usually as headache, diplopia and failing vision. Though labelled benign, it is very aggressive with a tendency to local recurrence and malignant change.     

Giant cell tumor of the proximal fibula: Surgical management

Five patients with giant cell tumor of the proximal fibula were treated with intralesional excision of the lesion, preservation of the peroneal nerve, and reconstruction of the lateral collateral ligament. At minimum 24-month follow-up there have been no local recurrences. Four patients exhibit normal function of the peroneal nerve and one has grade 4 strength of the muscles innervated by this nerve. No patient demonstrated varus instability. Marginal excision with nerve preservation and reconstruction of the ligament is a worthwhile procedure for treatment of this relatively uncommon lesion. © 1996 Wiley-Liss, Inc.     

Malignancy in giant cell tumor of bone

BACKGROUND: The term malignant giant cell tumor embraces multiple entities and therefore can be confusing. The goals of the current study were to define the clinicopathologic and histologic features of malignancy in giant cell tumors and to clarify the terminology. METHODS: The authors reviewed all cases from the Rizzoli Institute (Bologna, Italy) of primary (PMGCT) and secondary (SMGCT) malignancy in giant cell tumors. PMGCT is a high-grade sarcoma that arises side by side with benign giant cell tumors. SMGCT is a high-grade sarcoma that occurs at the sites of previously treated giant cell tumors of bone. RESULTS: The authors report 5 PMGCTs and 12 SMGCTs; half of the SMGCTs were postradiation sarcomas. Patient age ranged from 20 to 68 years (median, 62 years) for PMGCT and from 30 to 77 years (median, 40 years) for SMGCT. The average latent period between diagnosis of giant cell tumor and diagnosis of SMGCT was 9 years (range, 3-15 years) for patients with postradiation SMGCT and 19 years (range, 7-28 years) for patients with SMGCT resulting from spontaneous transformation. The histologic classification of high-grade sarcomas in the PMGCT group was osteosarcoma in four cases and malignant fibrous histiocytoma in one case. In the SMGCT group, the histologic classification was osteosarcoma in nine cases, fibrosarcoma in two cases, and malignant fibrous histiocytoma in one case. The outcomes associated with all malignancies in giant cell tumors were poor, with the worst outcome associated with postradiation SMGCT. CONCLUSIONS: Malignancies in giant cell tumors of bone always are high-grade sarcomas with a poor prognosis. These lesions must be distinguished from benign giant cell tumors of bone. SMGCT usually is easy to diagnose upon malignant clinicoradiographic presentation. In contrast, PMGCT often mimics giant cell tumors both clinically and radiographically. In addition, upon histologic examination, PMGCT shows areas of conventional giant cell tumor, which can lead to difficulties in making the correct diagnosis.     

骨巨细胞瘤治疗分析

目的 比较不同手术方法治疗骨巨细胞瘤的临床效果.方法 回顾性分析1998年11月至2008年11月手术治疗的83例四肢骨巨细胞瘤患者,男52例,女31例,其中胫骨近端26例,股骨远端28例,桡骨远端16例,肱骨近端7例,肱骨远端2例,股骨近端3例,足趾骨1例.于本院确诊时平均年龄30.3 (14～53)岁,Campanacci分级:I级45例,II级35例,III级3例;初发55例,复发28例,合并病理性骨折7例.Jeffer病理分级,一级35例,二级46例,三级2例.结果 83例患者均获得随访,随访时间8个月至9年2个月,平均4年11个月.病灶刮除+液氮冷冻+骨水泥填充术45例,肿瘤复发4例;病灶刮除+液氮冷冻+自体骨植骨术35例,肿瘤复发3例;瘤段骨切除人工假体置换术2例,肿瘤复发0例;瘤段骨切除煮沸灭活后回植1例,肿瘤复发0例.病灶刮除组总复发率8.75%,瘤段切除组织复发率为0.结论 病灶刮除+液氮冷冻+骨水泥填充术、自体骨植骨术和肿瘤瘤段切除术均是治疗骨巨细胞瘤的可靠方法.     

原发恶性骨巨细胞瘤11例临床病理分析

复习解放军济南军区总医院和山东医科大学附院20年间治疗的11例原发恶性骨巨细胞瘤。男4例,女7例。年龄平均22.6岁。发生于股骨下端5例,胫骨上端6例。疼痛和肿胀为主要临床症状。所有患者均行手术治疗,平均随访6年1个月(2年～13年7个月)。无复发、转移和死亡。另对8例标本进行了流式细胞计分析。证明了原发恶性骨巨细胞瘤的恶性程度。作者认为瘤段切除术是一种可取的手术方法。化疗对提高保肢手术成功率和生存率有一定作用。     

Giant cell tumor of the bones of the hand and foot

BACKGROUND: Giant cell tumor of the small bones of the hand and foot is suspected of having some peculiar features compared with giant cell tumor in other sites. Moreover, it could share some features with other giant cell rich lesions involving the hand and foot, and this may affect the differential diagnosis. The aim of this study was to analyze the features of lesions such as these in the files of the Rizzoli Orthopedic Institute. METHODS: The incidence of giant cell tumors of the bones of the hand and foot seen at the Rizzoli Orthopedic Institute over 50 years (1947-1997) was taken into consideration. There were 8 lesions of the hand and 21 of the foot. Clinical information and follow-up of the patients were studied and updated. Radiographs were studied and radiographic features analyzed. Histopathologic material was thoroughly reviewed and histologic features analyzed. RESULTS: Although the location of tumor was helpful information, radiographic features were not specific. Giant cell tumors of the small bones of the hand and foot showed a predominance in females, younger patients and more aggressive behavior than giant cell tumors of large bones. The authors did not observe multicentricity or pulmonary metastases. CONCLUSIONS: Because the radiographic features of giant cell tumor of the hand and foot overlap those of other giant cell rich lesions in these locations, histologic diagnosis is mandatory, although it may be difficult and require the establishment of diagnostic criteria for giant cell tumor. As this tumor tends to be more aggressive than other giant cell rich lesions, treatments of choice are aggressive curettage or resection.