Incidence Of Giant Cell Tumor Research Articles

Giant Cell Tumor of Intermediate Cuneiform Evolving into Aneurysmal Bone Cyst: A Rare Case Report

Introduction: Giant cell tumor (GCT) most commonly involves distal femoral condyles, distal end of radius, proximal tibial plateau, and proximal humerus. GCT is uncommon to occur in small bones of hand and feet. 2% of GCT occur in hand. The incidence of GCT in foot is 1.2–1.8%. Only a few cases have been reported in literature worldwide. GCT is the most common cause of secondary ABC. We report a case of GCT of intermediate cuneiform in a 25-year-old female evolving into aneurysmal bone cyst (ABC). Case Report: A 25-year-old female presented to us with complaints of pain and swelling over the dorsum of right foot for a period of 1 year. On examination, there was a localized ovoid-shaped swelling of 2 by 2 cm over the dorsum of right foot. Radiographs revealed a well-defined osteolytic lesion in the intermediate cuneiform. T2 MRI showed hyper-intense lesion in intermediate cuneiform. The patient was taken up for surgery, and the intermediate cuneiform was excised completely. Removed bone was sent for histopathological examination which confirmed it to be GCT evolving into secondary ABC. The patient was followed for 1 year and had no complaints. The patient was able to weight bear and walk without any difficulty. There was no recurrence of lesion. Conclusion: GCT of the cuneiform evolving into ABC is a very rare presentation. The treatment of choice is excision of the tumor with or without bone grafting. Any osteolytic lesion in the small bones must be evaluated and should be intervened in the early stage. Keywords: Giant cell tumor, osteolytic lesion, surgical resection, intermediate cuneiform.

Comparison of outcomes of 2 surgical treatments for proximal humerus giant cell tumors: a multicenter retrospective study

The incidence of giant cell tumors in the proximal humerus is low. We evaluated 2 surgical treatments for giant cell tumors of the proximal humerus and postoperative upper-extremity function. This study retrospectively analyzed the clinical data of 27 cases of giant cell tumors of the proximal humerus at 4 Chinese medical centers specializing in bone oncology collected between January 2002 and June 2015. All patients were followed up for more than 2 years. The surgical procedures performed for treatment included curettage in 14 patients and segmental resection in 13. The Campanacci grade, occurrence of pathologicfracture, surgical method, complications, and Musculoskeletal Tumor Society scorewere recorded for each cohort. The recurrence rate was 7.1% in the curettage group and 15.4% in the segmental resection group. Other postoperative complications occurred in 4 patients with segmental resection, including resorption of the osteoarticular allograft in 2, subluxation of the glenohumeral joint in 1, and prosthetic loosening and exposure in 1. A significant difference in postoperative upper-extremity function was noted between the 2 groups (P < .001). Postoperative upper-extremity function in the curettage group was significantly better than that in the segmental resection group. Segmental resection and reconstruction with a large segmental osteoarticular allograft were considered unadvisable. We suggest that extensive curettage should be selected to treat proximal humerus giant cell tumors as much as possible.

Is vitamin D deficiency behind the scenes for high incidence of Giant cell tumor amongst the Indian population? Unraveling the vitamin D – RANKL association

IntroductionThe major neoplastic and proliferative component of GCTB is the stromal tumor cells; that they have shown no evidence of bone destruction, instead the massive tissue destruction appears to be a result of tumor induced osteoclastogenesis.The discovery of receptor activator of nuclear factor kB (RANK) and RANK binding ligand (RANKL) uncovered the bone homeostasis and molecular mechanism by which multiple compounds (including vitamin D) regulated osteoclast differentiation; a function mediated by osteoblastic cells and osteoclast-precursor cells. HypothesisIn a country burdened by vitamin D deficiency, causal relation between hypovitaminosis D and GCTB was hypothesized based on the vitamin D mediated RANKL expression and osteoclastogenesis, as India is also a population with higher incidence of GCTB as compared to Western populations described in the literature.The possibility of vitamin D regulated osteoclastogenesis in GCTB is postulated on the evidence from molecular research linking it to the RANK/RANKL/OPG pathway.The aim of this study was to analyse the prevalence of Vitamin D deficiency in patients with primary GCTB and to elucidate any difference in serum Vitamin 25(OD)D3 levels amongst the matched control population data. Materials and results130 patients of primary GCTBs were matched to 310 controls from the general health check population and serum levels of 25(OH)D3 were analyzed. Statistical analysis performed on the non-parametric data and Mann Whitney U Test used to derive inference with significance set at p < 0.05.56 females and 76 males with median Vitamin D level in the GCTB group was 15.9 ng/ml (Mean 19.41; Range 1.03 to 92) as compared to the control population with median level of 22.2 ng/ml (Mean 25.1; Range 2.6 to 87.9). The results were significant (p value < 0.05) as compared to the control population in all decades except the third decade (p value 0.0548). DiscussionThe differential expression of RANKL and OPG in response to levels of vitamin D has been established. The stromal cells of osteolytic GCTB express high levels of RANKL, which is a key signal regulator in development of this disease and bone destruction typical of GCTBs. This has resulted in research targeting this pathway for therapeutic approach in GCTBs.As vitamin D supplementation is simple and safe, increased awareness to assess and if necessary correct vitamin D status of patients is warranted, however the question as to whether patients with low vitamin D levels are more prone to develop GCTB and thus would profit from vitamin D supplementation remains unanswered.To conclude, it is essential to assess vitamin D levels in patients with GCTB as deficiency is pronounced. Future research on this hypothesis might lead to an association between Vitamin D deficiency and the onset/natural history of GCTB that may in the future help us cure or prevent GCTBs.

Epidemiology of benign giant cell tumor of bone in the Chinese population

BackgroundQuantifying the incidence of giant cell tumor (GCT) of bone is challenging because it is a rare, histologically benign bone tumor for which population-level statistics are unavailable in most countries. We estimated the 2017 incidence of GCT in China using a direct (registry-based) approach with available population-based data. Materials and MethodsThe most recent age- and sex-specific incidence rates of GCT recorded in the Bone Tumor Registry in Japan (2015) were applied to 2017 age- and sex-matched populations projected by the United Nations for China in order to estimate 2017 incidence. An adjustment factor calculated using registry data suggesting that GCT may represent a greater proportion of bone tumors in China than in Japan (Guo, 1999) was applied to provide secondary estimates. ResultsAnnual GCT incidence was estimated to be 1.49 per million population or 2094 new cases in China for 2017. A comparison of this estimated incidence with Japan (1.25 per million) and the United States (1.38 per million) indicates that the incidence is somewhat higher in China using identical methods. Secondary estimates suggest that GCT incidence in China may be as high as 2.57 per million or 3625 new cases in 2017. The corresponding 3-year limited-duration prevalence of GCT in China using a registry-based approach and general age-specific mortality is 6276 (secondary estimate: 10,876). ConclusionsLeveraging unique population-based registry data, we estimated that GCT is a rare disease in the Chinese population with an incidence ranging between 1.49 and 2.57 cases per million persons per year. Possible differences in diagnostic classification of GCT, urban-rural demographics, and the younger demographic distribution of the Chinese population may underlie observations that GCT, a condition that primarily affects young individuals (20–40 years of age), accounts for a higher proportion of skeletal tumors in China than in other regions.

Giant cell tumor of the spine masquerading as spinal tuberculosis: An uncommon entity

The spine is an infrequent location for giant cell tumor (GCT). The incidence of GCT in the spine, above sacrum, is only 1%-1.5%. We report a case of recurrent GCT of the lumbar spine which was clinically diagnosed as spinal tuberculosis. A 24-year-old female presented with complaints of pain in the right leg during the last trimester of her pregnancy. One month later, she complained of retention of urine along with weakness in both the lower limbs. X-ray showed destruction and sclerosis of L3 vertebra with involvement of the adjacent discs. The magnetic resonance imaging showed severe compression and collapse of L3 vertebra with diffusely altered marrow signals and an associated expansible lobulated soft tissue component. With a provisional clinical diagnosis of spinal tuberculosis, the mass was decompressed and sent for histopathological examination, where it was diagnosed as GCT of the spine. Follow-up details were not available. However, the patient presented with similar complaints 6 months later. A near total resection of the tumor was done with fixation of the spine. Histopathology confirmed it as GCT. Although rare, still GCT needs to be considered in the differential diagnosis of spinal lesions so that specific treatment modality may be provided to the patient.

Primary tumors of the patella.

The patella is an uncommon location for cancerous occurrence and development. The majority of tumors of the patella are benign, with a significant incidence of giant cell tumors and chondroblastoma. With the development of modern diagnostic technologies, there appear however many other histological types which raise challenges of diagnosis and treatment. In this article, we review the reported histological types of primary patellar tumors. Specifically, epidemiology, symptomatology, imageology, histopathology, and treatment options for these histological lesions will be discussed, respectively. As there is an increasing focus on the diagnosis and the treatment of these lesions, the availability of the integrated information about primary patellar tumors becomes more significant.

Clinical characteristics and evolution of giant cell tumor occurring in Paget's disease of bone.

Patients with Paget's bone disease (PDB) have an increased risk of developing giant cell tumor (GCT). This study was performed to evaluate the clinical characteristics and evolution of GCT complicating PDB and to compare these clinical characteristics to those observed in two large PDB cohorts, the PDB Italian Registry and the United Kingdom's Multi-Centre Randomised Controlled Trial of Symptomatic Versus Intensive Bisphosphonate Therapy for Paget's Disease (PRISM) study. A systematic literature review identified 117 cases of PDB complicated by GCT (PDB-GCT), which involved the skeletal sites affected by PDB (110 patients) or the extraskeletal tissues adjacent to affected bones (7 patients). In contrast to what previously reported for GCT patients without GCT patients (83.2%) were white and one-fourth of them (24.8%) had multifocal GCTs. Compared to PDB patients without GCT, PDB-GCT patients showed a higher male/female ratio (2.1 versus 1.2) and more severe disease (age at PDB onset 52.1 ± 12.1 versus 63.3 ± 10.6 years; number of affected sites 6.1 ± 2.9 versus 2.34 ± 1.6; prevalence of polyostotic PDB 93.3% versus 60.6%). The mortality rate of PDB-GCT patients was higher than those occurring in GCT patients without PDB (about 50% versus 0% to 5% at 5 years) or in PDB patients without GCT (log rank = 29.002). Moreover, up to 98% of PDB-GCT cases had elevated total alkaline phosphatase levels at neoplasm diagnosis, suggestive of active PDB. Importantly, PDB-GCT patients from Southern Italy (45.6% of all GCT patients) showed a higher prevalence of multifocal GCT (51.7%) and of positive familial history for PDB (70.8%) and GCT (65.0%). Finally, indirect evidence suggests a decline in the incidence of GCT in PDB patients. The occurrence of GCT in PDB patients is associated with severe disease and reduced life expectancy of affected patients. The increased prevalence of familial diseases in PDB-GCT patients from Southern Italy suggests a founder effect. The observed changes over time in the incidence of GCT in PDB patients could be related to improved clinical management and/or living conditions of patients.

Giant Cell Tumor of the Extremity

There are no recent reports of giant cell tumors of bone in a large series of Chinese people. The present study was designed to review the epidemiological characteristics and outcomes of surgical management in a large series of Chinese patients with giant cell tumor of an extremity, treated at a single institution. The records and images of 621 patients in whom a benign giant cell tumor in an extremity was treated between 1989 and 2009 were reviewed retrospectively. There were 359 male and 262 female patients. The mean age at diagnosis was 31.4 years (range, eleven to seventy-one years). Sixty-six percent of the giant cell tumors were localized around the knee. Surgical treatments primarily included curettage, extensive curettage, and resection. The median duration of follow-up was forty-nine months (range, eighteen to 256 months). Giant cell tumor accounted for 13.7% of all primary bone tumors treated at our institution. Multivariate Cox regression analysis indicated that the only variable that contributed to recurrence-free survival was the type of surgical treatment. The local recurrence rate after extensive curettage was 8.6%, which was significantly lower than the 56.1% recurrence rate after curettage alone. Bone-grafting did not affect local tumor control after extensive curettage; the local recurrence rate was 11.1% if bone graft was used. Recurrent giant cell tumor can be treated by further curettage or resection, with acceptable re-recurrence rates of 6.7% and 9.3% respectively. The Musculoskeletal Tumor Society Score for patients treated with extensive curettage was 92.6%, which was significantly higher than that for patients treated with resection. Twenty-one (3.4%) of the 621 patients developed benign pulmonary metastasis, with a favorable outcome, and three patients presented with multifocal giant cell tumors. The incidence of giant cell tumor in the Chinese population may be higher than that in Western countries, and it has a male predilection. The results of the present study suggest that extensive curettage provides favorable local control and satisfactory functional outcomes.

GCT of proximal phalanx of ring finger: A case report

Giant-cell tumor (GCT) of bone arising from phalanx of a finger is extremely rare. Rizzoli Orthopedic Institute in their study on 900 treated cases of GCT from 1947-1997 reported only 0.9% incidence of GCT in bones of the hand. There was no case of GCT of the phalanges in that series. We report here a case of GCT of bone arising from phalanx of finger because of its very unusual location. The tumor was diagnosed on the basis of fine-needle aspiration cytology and confirmed by histopathology.

Surgical consideration for benign bone tumors

The surgical management of symptomatic benign bone tumor has been described in various manners in medical literature. However, there are few published reports on the presentation and surgical management of benign bone tumors in black African patients. To determine the pattern of presentation of benign bone tumors and evaluate the common indications for surgery in a Nigerian Orthopedic Center. This is a prospective study of 67 patients, surgically treated for benign bone tumors, over a three-year period, at the National Orthopedic Hospital, Lagos, Nigeria. The common histological types include, osteochondroma, giant cell tumor, and the simple bone cyst. These tumors have varying anatomic locations, but are more commonly located around the knee joint. In this series, most of the patients have presented with an active or aggressive stage of the disease. The most common indication for surgery is painful swelling; other indications include a pathological fracture, restricted range of movement, and peripheral nerve compression. The surgical procedures performed are simple excision, curettage, and stabilization; and 1-stage and 2-stage wide resection with reconstruction. Patients with significant bone defects have autologous bone grafting or methylmethacrylate cement application. Further stabilization is achieved with intramedullary or compression plate and screw fixation. Amputation has only been necessary in one patient with a huge aneurysmal bone cyst. At the average follow-up period of 28.6 months, five patients showed recurrence. All were with a histological diagnosis of giant cell tumor. The mode of presentation of benign bone tumors in this group of black African patients is heterogenous, demanding various surgical options. Limb sparing is a largely feasible option, but the recurrence rate is particularly higher for giant cell tumors. Increase in the number of patients presenting with giant cell tumors raises the possibility of an increase in the incidence of this condition in the black African population. Larger multicenter studies in the black African population may shed more light on the actual incidence of giant cell tumors and other bone tumors in this group of patients.

Interferon and giant cell tumors.

The article by Kaban et al1 appearing in this month's issue ofPediatrics is of interest because it describes, to the best of my knowledge, the first completely successful medical treatment of a primary giant cell tumor of the bone with the angiogenesis inhibitor interferon α-2a. Previously, a 37-year-old woman with a benign giant cell tumor of the leg that spread to the lungs demonstrated a significant decrease in pulmonary metastases and stabilization of disease following treatment with interferon α-2a.2 Giant cell tumors of the bone have 3 major cell types: 1) proliferating mononuclear cells thought to be the neoplastic element of the tumor, 2) nonproliferating mononuclear cells, and 3) multinucleated giant cell that are fused mononuclear cells.3 The incidence of giant cell tumors among primary bone neoplasms is 4% to 5%, with most occurring around the knee and <1% in the skull. Because 95% are benign, the incidence of these tumors among benign bone tumors rises to 18%.4,5 More than 50 cases of pulmonary metastases associated with benign giant cell tumors have been described.6 Aneurysmal bone cysts are associated with almost 15% of giant cell tumors, as was the case in this present study. With rare exceptions, giant cell tumors occur in skeletally mature individuals, with about 75% occurring in patients between 15 and 40 years of age. It has been estimated that only 1.7% of giant cell tumors occur in skeletally immature individuals.4The treatment for giant cell tumors is problematic. Approximately 40% to 70% of these lesions recur after marginal resection (curettage), although this rate can be decreased to about 25% if the lesion has a low histologic stage. Less than 10% of these tumors recur after wide resection; a procedure often limited because of the location of the lesion and/or the desire to preserve limb function.4 Conventional anticancer chemotherapy has occasionally been reported to be effective,5 while radiation therapy is associated with a recurrence rate of about 50%. The problem with radiotherapy is that it not only carries the usual risk of radiation-induced sarcoma but also the risk of radiation-induced giant cell tumor-specific sarcomas. This later risk has been estimated to be from 7% to 33%, although modern radiation techniques are said to decrease this risk.4Because of the rather limited success of current therapeutic modalities, the apparent success of interferon α-2a therapy is a welcome discovery. Many more typical patients, (ie older with extremity lesions), will have to be successfully treated before one can say with any surety that this inhibitor of angiogenesis is effective.7 After attending a lecture at which Dr Folkman discussed the case described in this month's issue, we gave interferon α-2a to a 17-year-old female with a nonosseous, pelvic, giant cell tumor that could not be removed surgically and was completely resistant to radiotherapy and embolization. A significant reduction in tumor size was observed and the patient regained some of the function that was compromised as a result of compression and local infiltration.In addition to those tumors discussed by Kaban et al that are responsive to interferon, interferon has also been shown to be effective in immunoblastic lymphadenopathy8 and, along with interleukin 2, renal cell carcinoma.9 Administration of interferon has been associated with fever, fatigue, anorexia, myelosuppression, and central nervous system and gastrointestinal toxicity.10 Cardiovascular complications have been observed and they include rhythm abnormalities, myocarditis, cardiomyopathy, myocardial infarction, and hypotension. Some of these cardiac problems may be attributable, in part, to the inhibitory effects of interferon on neovascularization which, in turn, may lead to an increase in the thickness of the endothelial processes of myocardial capillary walls.11The web site for the National Cancer Institute lists more than 20 phase I-III trials, each evaluating a different angiogenesis inhibitor, that are currently open for patients with various types of cancer. If only some of these studies are successful, we will have added to our armamentarium antitumor agents that, either alone or in combination with current antineoplastic agents, are less toxic and more tumor-specific than our present therapeutic regimens. Furthermore, because these agents have a unique mechanism of action, they may prove effective when other, more conventional, therapy fails. The study of angiogenesis inhibitors is of intense interest and promise.

Case report 810

And unusual case of giant cell tumor in a 3-year-old girl has been presented. The topic of giant cell tumors has been discussed at length. The pathology and the differential diagnosis have been considered. The incidence of giant cell tumors in children and adolescents has also been reviewed and proved to be extremely rare.