ACRYLIC PROSTHESIS REPLACING LOWER END OF THE FEMUR FOR BENIGN GIANT-CELL TUMOR

Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs. A total of 354 consecutive patients with a GCTB underwent joint salvage by curettage and reconstruction with bone graft and/or cement or en bloc resection. Patient, tumour, and treatment factors were analyzed for their impact on LR. Patients treated with denosumab were excluded. There were 53 LRs (15%) at a mean 30.5 months (5 to 116). LR was higher after curettage (18.4%) than after resection (4.6%; p = 0.008). Neither pathological fracture (p = 0.240), Campanacci grade (p = 0.734), soft-tissue extension (p = 0.297), or tumour size (p = 0.872) affected the risk of recurrence. Joint salvage was possible in 74% of patients overall (262/354), and 98% after curettage alone (262/267). Of 49 patients with LR after curettage, 44 (90%) underwent repeated curettage and joint salvage. For patients treated by curettage, only age less than 30 years (p = 0.042) and location in the distal radius (p = 0.043) predicted higher LR. The rate of LR did not differ whether cement or bone graft was used (p = 0.753), but may have been reduced by the use of hydrogen peroxide (p = 0.069). Complications occurred in 15.3% of cases (54/354) and did not differ by treatment. Most patients with a GCTB can undergo successful joint salvage by aggressive curettage, even in the presence of a soft-tissue mass, pathological fracture, or a large lesion, with an 18.4% risk of local recurrence. However, 90% of local relapses after curettage can be treated by repeat joint salvage. Maximizing joint salvage is important to optimize long-term function since most patients with a GCTB are young adults. Younger patients and those with distal radius tumours treated with joint-sparing procedures have a higher rate of local relapse and may require more aggressive treatment and closer follow-up.

From 1950 to 1994, 257 cases of benign bone tumors of the foot were treated at the Bone Tumor Center of the Rizzoli Institute. Aneurysmal bone cysts (ABC) and giant cell tumors (GCT) are rare and often they are misdiagnosed. To define the clinical and radiographic features useful for diagnosis, x-rays of 24 ABCs and 21 GCTs of the foot were reviewed. Adding our series to the cases reported in the literature, the only important clinical data that emerged was the average age of patients affected by ABC and GCT (15 and 27 years, respectively). ABCs localized in small tarsal bones were rare (6%), whereas 19% of GCTs were found in this site. The eccentric, round shape of a lytic lesion was more characteristic of GCT. An aggressive pattern of radiographic bone destruction was observed more frequently in GCT than in ABC (P = 0.01). Septation was seen in both tumors. Typical of ABC was a sharp and trabeculated margin, whereas GCT had an ill-defined edge with normal cancellous bone (P = 0.007). The growth of ABCs often expanded the cortex, while GCTs eroded, thinned, and broke the cortical bone (P = 0.001). A saucerized cortex was observed only in ABCs. A bony shelf was more characteristic of ABC than of GCT (P = 0.002). Pathologic fractures (67%) and joint involvement, evaluated by computed tomography and magnetic resonance imaging (83%), were more frequent in GCTs, whereas fluid levels were more frequently observed in ABCs (47%). The majority of the tumors were stage 2, but GCTs represented 70% of stage 3 aggressive lesions.

BackgroundDenosumab (XgevaTM) is a fully human antibody to RANK-Ligand, an important signal mediator in the pathogenesis of giant cell tumour of bone (GCTB). The use of denosumab in the treatment of GCTB has changed the way in which these tumours are managed over the past years.Case presentationDescribed is the case of an acute fracture through a GCTB of the distal radius of a fit and well 32-year-old, non-smoking, female patient following a simple fall onto her outstretched, dominant hand. The aim was to enable joint sparing management for the patient, as opposed to an acute fusion procedure of the carpus. The patient underwent percutaneous k-wire fixation with application of plaster and immediate commencement with denosumab to halt the activity of the GCTB. Bone healing was rapid; plaster and k-wires were removed after 6 weeks. At 6 months denosumab, was ceased and an open curettage and grafting procedure of the tumour bed was undertaken (using MIIG X3, Wright Medical, aqueous calcium sulphate as graft material).ConclusionsThe use of denosumab in the acute setting of pathological fracture through giant cell tumour of bone allowing joint salvage has not been previously described. The treatment was well tolerated and functional outcomes are excellent, with very promising 4-year follow-up.This novel approach may allow for more joint sparing strategies in the future for other patients in this difficult situation. Further cases will need to be gathered to establish this technique as a suitable treatment pathway.

IntroductionGiant cell tumor is a type of benign tumor which has the characteristic of rapidly growing and a chance to metastasis. It is however locally aggressive and would typically affect young patients. They commonly present with pain and associated with pathological fracture. Presentation of caseThis is an uncommon case of 29 years old male with pathological fracture and giant cell tumor in proximal humerus. A plain radiograph revealed pathological fracture in head of humerus and histopathology examination was consistent with giant cell tumor. The patient had surgical option with en bloc giant cell tumor resection following hemiarthroplasty with cementless endoprosthetic implant for humerus, which aimed to provide a single step surgery without any interval debulking surgery. The patient had achieved bony union between 6 weeks after the surgery and recurrence was not found by the time of the last follow-up. DiscussionBased on Campanacci’s classification the tumor is divided into 3 stages. The management of giant cell tumors continues to be one of the most challenging areas in orthopedic oncology. Surgery is the first line option; however, it is depending on the tumor staging and can vary from intralesional curettage to total resection of the tumor. Since the local behavior of giant cell tumors has a high risk of local recurrence, en bloc resection and reconstruction were chosen for these Grade III lesions. ConclusionThe aim of this procedure is to preserve the shoulder joint shown satisfaction in a clinical, radiological, functional and esthetic result.

The rare benign giant cell tumour of bone (GCTB) is defined by an almost unique mutation in the H3.3 family of histone genes H3‐3A or H3‐3B; however, the same mutation is occasionally found in primary malignant bone tumours which share many features with the benign variant. Moreover, lung metastases can occur despite the absence of malignant histological features in either the primary or metastatic lesions. Herein we investigated the genetic events of 17 GCTBs including benign and malignant variants and the methylation profiles of 122 bone tumour samples including GCTBs. Benign GCTBs possessed few somatic alterations and no other known drivers besides the H3.3 mutation, whereas all malignant tumours harboured at least one additional driver mutation and exhibited genomic features resembling osteosarcomas, including high mutational burden, additional driver event(s), and a high degree of aneuploidy. The H3.3 mutation was found to predate the development of aneuploidy. In contrast to osteosarcomas, malignant H3.3‐mutated tumours were enriched for a variety of alterations involving TERT, other than amplification, suggesting telomere dysfunction in the transformation of benign to malignant GCTB. DNA sequencing of the benign metastasising GCTB revealed no additional driver alterations; polyclonal seeding in the lung was identified, implying that the metastatic lesions represent an embolic event. Unsupervised clustering of DNA methylation profiles revealed that malignant H3.3‐mutated tumours are distinct from their benign counterpart, and other bone tumours. Differential methylation analysis identified CCND1, encoding cyclin D1, as a plausible cancer driver gene in these tumours because hypermethylation of the CCND1 promoter was specific for GCTBs. We report here the genomic and methylation patterns underlying the rare clinical phenomena of benign metastasising and malignant transformation of GCTB and show how the combination of genomic and epigenomic findings could potentially distinguish benign from malignant GCTBs, thereby predicting aggressive behaviour in challenging diagnostic cases. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.

This study was done to investigate X-ray, computed tomography (CT) and magnetic resonance (MR) imaging features of recurrence in giant cell tumour of bone (GCTB) and to evaluate risk factors. Medical records and imaging data were reviewed for 55 cases of recurrent GCTB. All images were reviewed retrospectively and independently by two radiologists experienced in skeletal musculature. The common radiological findings; factors related to tumour recurrence such as gender, age, location; pathological fracture, Campanacci grading and surgical procedure were analysed by nonparametric test (Mann-Whitney U test for two independent samples test and Kruskal-Wallis H test for multiple independent samples test). p values <0.05 were considered to indicate a statistically significant difference. The imaging features of recurrent GCTB were as follows: osteolytic destruction or bone resorption of graft bone or around the polymethylmethacrylate (PMMA), soft tissue mass formation and expansile change. Tumour parenchyma showed markedly heterogeneous enhancement, except for necrotic cystic cavities, on contrast-enhanced MR images. Wide resection had a smaller (p=0.031) risk of local recurrence than did intralesional curettage. There was no statistical significance in gender, age, location, pathological fracture and Campanacci staging (p>0.05). The risk of recurrence in GCTB was influenced by the type of surgery and adjuvants. Bone transformaresorption, soft tissue mass formation and aggravated expansile change are reliable signs of recurrence on imaging.

Introduction: Giant cell tumor (GCT) also called osteoclastoma of bone is the most common osteolytic bone tumor encountered by an orthopedic surgeon. En bloc resection of major joints creates a problem for the reconstruction of large defects. Recent advances in tumor resection defects involve the use of custom-built joints for the reconstruction of defects near joints. This article analyzes the functional outcomes after resection of juxta articular GCTs and reconstruction by custom mega prosthetic arthroplasty. Aims and Objectives: To study the functional results of custom mega prosthetic reconstruction in juxta articular GCTs with intra articular extension. Materials and Methods: Four patients with juxta articular GCTs around the hip and knee with mean age of 40 yrs (range 30 to 50 yrs) underwent resection and reconstruction by custom mega prosthetic arthroplasty during the period 2011 to 2013. Two patients were males and two were females. All of them were in Enneking stage 3. Proximal femur was involved in one patient, distal femur in one and proximal tibia in two patients. Results: Functional results were analyzed using Ennekings criteria. Excellent results were obtained in all the patients without recurrence, periprosthetic fractures, infections or aseptic loosening. Conclusion: By using the technique of custom mega prosthetic reconstruction in juxta articular GCTs with pathological fractures or intra articular extension, the desired goals of reconstruction with good functional results and least complications can be achieved.

Marked nuclear atypia can be found in a variety of benign mesenchymal tumors, including ancient schwannomas, symplastic leiomyomas, osteoblastomas, and chondromyxoid fibromas. Such nuclear atypia is believed to represent a degenerative phenomenon and does not indicate aggressive behavior. Separation of these pleomorphic but benign lesions from true sarcomas may be difficult. We are aware of only one prior description of such degenerative atypia in benign giant cell tumor of bone. We report a benign giant cell tumor of bone arising in the sacrum of a 21-year-old white female. Preoperative embolization of this lesion had been performed, and subsequent curetting revealed marked atypia suggestive of an anaplastic malignancy. However, only very rare mitotic figures were present, and the radiographs were consistent with a benign giant cell tumor. A diagnosis of benign giant cell tumor was made, and no recurrence or metastases have been observed during the 4-year postoperative period.

Giant cell tumour of ulna is a rare and benign neoplasm but is locally invasive tumour. Distal ulna giant cell tumors have a reported incidence of 0.45 to 0.6%. 43 years old female came with alleged History of slip and fall at her residence sustaining injury to right wrist and suspected to be a giant cell tumor. The giant cell tumour was treated with en bloc resection and curettage (Darrach's procedure). Patient was followed up for 6 months during which she regained full range of motion. Giant cell tumor can masquerade as pathological fracture with En bloc Resection dramatically reducing the risk of recurrence.

Descriptive. To outline a novel multimodality approach for a difficult surgical resection of a giant cell tumor in the cephalad portion of the sacrum. Giant cell tumors of the sacrum are rare primary bone tumors. Recent reports have demonstrated diminished giant cell tumor recurrence with cryosurgery by using a "direct pour" technique with liquid nitrogen. Although successful in decreasing tumor recurrence, this technique is accompanied by a 4%-8% rate of skin necrosis and high rates of pathologic fracture. The authors describe resection and a novel, controlled method of argon-based cryotherapy (followed by a unique pelvic reconstruction) for a large, difficult giant cell tumor of the sacrum. A 29-year-old woman presented with complaints of right foot drop and decreased sensation of the right buttock, posterior thigh, posterior calf, and lateral aspect of the right foot. Radiographic evaluation revealed a mass in the right sacrum; histologic examination of CT-guided biopsy revealed a giant cell tumor. A combined anterior abdominal and posterior sacral approach was performed, the tumor was resected, and the margin of the cavity was treated with controlled argon-based cryotherapy. The combination of thermocouples, electromyographic monitoring, and rapid freeze-thaw cycles allowed a controlled ablation of the tumor margin while ensuring that surrounding structures, such as the rectal wall, sacral nerves, and gluteal muscles, were not damaged. Posterior spinal fusion L4 to sacrum, posterior spinal instrumentation L4 to pelvis, and allograft reconstruction of the right sacrum were performed. The patient recovered well without skin necrosis or pathologic fracture. Urinary and fecal continence were preserved. At the 20-month follow-up the patient has no evidence of local tumor recurrence and is fully ambulatory without a brace or narcotic medication. A novel multimodality approach, consisting of resection, controlled cryosurgery, and a unique lumbopelvic reconstruction, was safe and successful in managing a challenging proximal sacral giant cell tumor. Twenty months after surgery the patient has excellent bowel and bladder control, no tumor recurrence, and functional ambulation without a brace or pain.

Background and purpose Risk factors for local recurrence of giant-cell tumor of bone (GCTB) have mostly been studied in heterogeneous treatment groups, including resection and intralesional treatment. The aim of the study was the identification of individual risk factors after curettage with adjuvants in GCTB.Methods Of 147 patients treated for primary GCTB between 1981 and 2009, 93 patients were included in this retrospective single-center study. All patients were treated with curettage and polymethylmethacrylate (PMMA) with (n = 75) or without (n = 18) phenol. Mean follow-up was 8 (2–24) years. Recurrence-free survival was assessed for treatment modalities. Age, sex, tumor location, soft tissue extension, and pathological fractures were scored for every patient and included in a Cox regression analysis.Results The recurrence rate after the first procedure was 25/93. Recurrence-free survival for PMMA and phenol and for PMMA alone was similar. Eventually, local control was achieved using 1 or multiple intralesional procedures in 85 patients. Resection was required in 8 patients. A higher risk of local recurrence was found for soft tissue extension (HR = 5, 95% CI: 2–12), but not for age below 30, sex, location (distal radius vs. other), or pathological fracture.Interpretation Curettage with adjuvants is a feasible first-choice treatment option for GCTB, with good oncological outcome and joint preservation. Soft tissue extension strongly increased the risk of local recurrence, whereas age, sex, location, and pathological fractures did not.

To describe the surgical treatment of patients with a pathologic fracture through a benign tumor of the proximal femur to determine if there is a difference in local recurrence, complications or functional outcome compared to patients with tumors in the same location without pathologic fractures. From 1989-2010, of 97 patients, 29 presented with a pathologic fracture (PF) through a proximal femoral benign bone tumor and 68 presented without a pathologic fracture (NPF). Outcomes of the two groups were compared in terms of surgical management, postoperative complications, local recurrence and functional scores. Fibrous dysplasia, giant cell tumor of bone and chondroblastoma were the most common subtypes. Most patients were managed with joint preservation in both PF (86.2%) and NPF (98.5%) groups (P = 0.03). Local recurrence risk was similar for patients in the PF (10.3%) and NPF (8.8%) groups. Mean follow-up was 105.7 months (P = 0.8). Functional outcome scores were high in both groups and not statistically significantly different. The majority of pathologic fractures through a benign bone tumor of the proximal femur can be successfully treated with curettage, burring, bone grafting and internal fixation without increasing the risk of local recurrence or negatively impacting functional outcome.

During 1965-2002 one thousand two hundred thirty patients with bone tumors and tumor-like diseases were treated at Astrakhan Medical Academy, Clinic of Traumatology and Orthopaedics. Out of them 163 (13.3%) patients had pathologic fractures. One hundred eight fractures were localized within long bones: humerus (61), femur (37), tibia (29), fibula (6), radius (5). Long bones fractures were commonly resulted from destructive lesions: solitary bone cyst - 48 cases, fibrous dysplasia - 26, giant cell tumor - 24, aneurismal bone cyst - 18, metaphyseal fibrous defect -11. Clinical and roentgenologic semiotics was studied and complex of roentgenologic signs of long bone pathologic fractures was systematized. Peculiarities of fractures (injury mechanism, typical localization of pathologic process, pattern of destruction focus) in various nosologic forms of primary disease were detected. The data obtained simplify diagnosis and allow trustworthy making prehistologic diagnosis as well as to choose optimum treatment tactics.

Wide margin excision followed by tibialisation of fibula and ankle arthrodesis as novel surgical technique in giant cell tumor patient

The use of adjuvants after curettage has been well established for the treatment of giant cell tumor of bone. The purpose of this study was to analyze the rates of recurrence following different types of treatment as well as the influence of various factors of tumor presentation on those rates. The data regarding benign giant cell tumors of the appendicular skeleton from ten bone tumor centers were evaluated. Axial and malignant tumors were excluded. The recurrence rates associated with the different treatment modalities were analyzed, and hazard ratios for a recurrence were calculated for multiple factors of tumor presentation. The study included 384 surgical procedures, involving 256 primary and 128 recurrent tumors. The mean duration of follow-up was 64.2 months. Wide excision was performed in seventy-eight cases (20.3%), and an intralesional procedure was done in 306 (79.7%). Of the intralesional procedures, 103 (33.7%) were performed without the use of adjuvants, 102 (33.3%) included filling with polymethylmethacrylate, seventy-four (24.2%) included polymethylmethacrylate filling after phenolization, and twenty-seven (8.8%) included use of local toxins. The overall recurrence rate after the intralesional procedures was 49% when no adjuvants had been used, 22% when polymethylmethacrylate only had been used as an adjuvant, 27% when polymethylmethacrylate had been used after phenolization, and 15% when phenol or other local toxins had been used (without polymethylmethacrylate). The highest rate of recurrence (36%) after curettage with adjuvants was associated with extracompartmental tumors. Recurrent tumors were not at increased risk for another recurrence, even when they were extracompartmental. The recurrence rate following curettage of a primary tumor without the use of adjuvants (55%) was higher than that following the same treatment of a recurrent tumor (39%) (p = 0.033). Use of polymethylmethacrylate as an adjuvant significantly reduces the recurrence rate following intralesional treatment of benign giant cell tumors, and it appears to be the therapy of choice for primary as well as recurrent giant cell tumors of bone. The significantly better results following treatment of recurrent tumors without adjuvants compared with the results of the same treatment of primary tumors were probably related to increased surgical thoroughness brought about by the surgeon's awareness of dealing with a riskier tumor.