Benign Giant Cell Tumor Research Articles

Radiological Appearance of Brown Tumors of the Maxilla Indicative of Primary Hyperparathyroidism: A Case Report

Brown tumors are a rare complication of hyperparathyroidism resulting from abnormalities in bone metabolism. Advances in biological exploration techniques enable early diagnosis before the onset of bone manifestations. While mandibular involvement is the most common in the maxillofacial region, maxillary involvement is extremely rare. We report a case of a patient with primary hyperparathyroidism revealed by a maxillary tumor. The pathological result concluded a benign giant cell tumor of the maxilla and fibrous dysplasia. The diagnosis of a brown tumor was considered based on the association of radiological data (osteolytic image and parathyroid adenoma), histological findings (giant cell tumor), and biological markers (hypercalcemia, hypophosphatemia, and elevated Parathyroid Hormone (PTH). This case highlights the difficulty in establishing a correct diagnosis in patients with an osteolytic maxillary process and the need to investigate hyperparathyroidism in the presence of a giant cell lesion.

Endoprosthetic replacement in benign giant cell tumor of the distal femur: A case report

Endoprosthetic replacement in benign giant cell tumor of the distal femur: A case report

GIANT CELL TUMOUR OF HAND: A CASE REPORT

Giant cell tumours commonly occur at the metaphysis-epiphysis region of long bones especially the distal femur. Giant-cell tumor (GCT) of the bone affecting the hand is a rare lesion that is usually diagnosed at an advanced stage and has a high rate of recurrence. These tumors are mostly benign ,multifocal. It is composed of three cell types: the neoplastic GCT stromal cells; mononuclear monocyte cells; and multinucleated giant cells. Clinical imaging is basic for the diagnosis of a GCT. We are presenting a case report of a 28 years old male, who presented with a swelling in right ring nger, following trauma. X-ray showed destructive expansile lytic lesion of the middle phalanx of right ring nger. CT also showed expansile lytic lesion with ballooning of cortex involving middle phalanx with sparing the neck of phalanx. FNAC of the swelling was done and it revealed a typical benign giant cell tumor composed of closely packed stromal cells with admixture of evenly distributed giant cells.

Extra Skeletal Myxoid Chondrosarcoma of Hand – A Case Report with Review of Literature

Extraskeletal myxoid chondrosarcoma (EMC) arising from soft tissues is a rare neoplasm morphologically characterized by ill-defined nodular masses composed of cords and strands of small eosinophilic cells separated by fibrous septae along with abundant myxoid stroma. We report a middle-aged man presenting with swelling on his hand for the last 11 years. He reports occasional pain in the swelling without any increase in its size or change of color. Radiologically, it mimicked a giant cell tumor of the tendon sheath. We received a white gelatinous tumor mass showing myxoid and hemorrhagic areas on cut section. Histo-morphological examinations suggested a diagnosis of EMC. Immunohistochemistry showed a strong expression of S100 and NSE. This slow-growing mass mimicking a benign giant cell tumor of tendon sheath warranted an urgent examination for metastasis and further treatment.

Up-regulation of matrix metalloproteinase-9 in primary bone tumors and its association with tumor aggressiveness.

Understanding the molecular mechanism underlying the pathophysiology of primary skeletal tumors is crucial due to the tumor-relatedcomplications, incidence at a young age, and tumor recurrence. The local expression pattern of MMP-9 as an active matrix-degrading protease was detected in 180 bone tissues, including 90 tumors and 90 noncancerous tissues, utilizing real-time qRT-PCR at the mRNA level and immunohistochemistry at the protein level. The correlation of the MMP-9 expression level with the patient's clinical pathological characteristics and the aggressiveness of the tumor was evaluated. The diagnostic significance of MMP-9 and the model of association of variables and MMP-9 expression and their predictive values were determined. Mean mRNA expression was higher in all types of primary bone tumors than their paired non-cancerous tissues. Osteosarcoma and Ewing'ssarcoma expressed higher levels of MMP-9 compared to benign giant cell tumors, and the MMP-9 expression level was significantly correlated with the size, metastasis, and recurrence of the malignant tumor. A consistent expression pattern was demonstrated for MMP-9 protein levels in tissues. In addition, the MMP-9 gene and protein levels significantly discriminate between bone tumors and normal tissue, as well as benign and malignant tumors, and could predict potentially malignant traits such as tumor grade and metastasis. The datapropose that MMP-9maybe involved intheproliferation and invasionof primary bone tumorsandhas the potentialto monitor andtreat theprogressionof malignant tumors.

Outcomes and complications of organ-sparing treatment of patients with tumor and tumor-like conditions of the forearm long bones

Introduction.Surgical treatment of tumors of the distal part of the forearm has always been complicated. Though there are many methods of reconstruction of defects of the long bones of this area after resection, the optimal technique is still debated.The study objectiveis to analyze the results of surgical treatment of tumors and tumor-like conditions of the forearm long bones, rate of postoperative complications, and techniques of their correction.Materials and methods. The article presents data on 75 patients with tumor and tumor-like conditions of the forearm long bones. Diagnosis was histologically verified. In 58 (77.3 %) patients, benign giant cell tumor was diagnosed, in 3 (4 %) patients – malignant giant cell tumor, in 3 (4 %) patients – osteogenic sarcoma, in 3 (4 %) patients – chondrosarcoma, in 6 (8 %) patients – fibrous dysplasia. All patients underwent surgical treatment of varying volume. During the study, analysis of complications of surgical treatment was performed.Results.Postoperative complications of various nature developed in 17 (22.7 %) of 75 patients between 2 weeks and 2 years after treatment. They mostly developed after segmental resection of the forearm bones with autoplasty (38.1 %) and segmental bone resection (21.4 %), as well as after excochleation with cementoplasty (16.1 %). The most frequent complications were autotransplant lysis (in 4 (19.1 %) cases), its fracture (in 2 (9.5 %) cases), and infections and inflammatory complications (in 2 (9.5 %) cases). After segmental resection with endoprosthesis, only 1 (11.1 %) patient experienced endoprosthesis head instability in the early postoperative period which led to re-endoprosthesis. Local recurrence was observed in 6 (19.4 %) of 31 patients with gigantic-cell tumors after excochleation and in 4 (28.6 %) of 14 patients after segmental resection. In 2 (14.3 %) cases, progression of the main disease was observed in the form of pulmonary metastases.Conclusion.Excochleation with cementoplasty is the method of choice in benign tumors of the forearm bones in the absence of soft-tissue component, destruction of the cortical layer, advancement of the tumor to the joint surface. In case of benign tumors in the absence of soft-tissue component and destruction of the cortical layer, and in the presence of tumor advancement to the joint surface, the optimal technique is segmental bone resection with substitution of the defect with endoprosthesis.

Clinical and radiological outcomes of combined modular prothesis and cortical strut for revision proximal femur in giant cell tumor of bone patients.

Femoral bone deficiency is a challenging problem in revision proximal femoral replacement. The purpose of this study is to evaluate the clinical and radiological outcomes of revision proximal femoral replacement as a salvage treatment for severe bone loss after oncologic proximal replacement surgery in patient with benign giant cell tumor of bone. 16 patients (6 men and 10 women) were included in this retrospective study, with a mean age of 46.6year at the time of revision surgery. All patients underwent revision proximal femoral replacement with the use of modular prosthesis and cortical strut allografts. The modified Harris Hip Score, Short Form 36, and musculoskeletal Tumor Society Score were used for patient evaluation. Regular follow-up was performed to evaluate the recurrence and metastases rate, limb function, and long-term complications of patients. The average follow-up was 46.3months (range, 26-75months), during which there was no local recurrence and metastases of patient. At the latest follow-up, the mean modified Harris Hip Score was 70.6 points, which was significantly improved compared with that of preoperative (p < 0.05). The final follow-up results of Short Form 36, Musculoskeletal Tumor Society Score, and limb-length discrepancy were also significantly improved compared to that of preoperative (p < 0.05). At the latest follow-up, the implanted femoral stems were all stable and all cortical strut allografts were also incorporated to their own bone. Using modular prosthesis and cortical strut allografts in revision, proximal femur replacement is an acceptable procedure for relatively young patient with severe proximal femoral bone loss after oncologic surgery with benign giant cell tumor of bone. More attentions should be paid to reduce the risk of complications in these complex reconstructions.

Outcome Analysis of En bloc Resection and Reconstruction by Non-vascularized Proximal Fibular Autograft in Giant Cell Tumor of Distal Radius supplemented with Brachytherapy.

Giant cell tumors of the bone are aggressive and potentially malignant lesions. Juxtaarticular giant cell tumors of the lower end radius are common and is a challenge for reconstruction after tumor excision. Several reconstructive procedures like vascularized and non-vascularized fibular graft, osteoarticular allograft, ceramic prosthesis, and megapros thesis are in use for substitution of the defect in the distal radius following resection. Here, we have analyzed the results of aggressive benign Giant cell tumor of the distal radius treated by en bloc excision and reconstruction using autogenous non-vascularized fibular graft along with brachytherapy. Eleven patients with either Campanacci Grade II or III histologically proven giant cell tumors of lower end radius were treated with en bloc excision and reconstruction with ipsilateral non-vascularized proximal fibular autograft. Host graft junction was fixed with low contact dynamic compression plate (LC-DCP) in all cases. Fixation of the head of the fibula with carpal bones and distal end of the ulna, if not resected, using K-wires at graft host junction was done. Brachytherapy was given in all 11 cases. Routine radiographs and clinical assessments regarding pain, instability, recurrence, hand grip strength, and functional status were done using Mayo modified wrist score at regular intervals. The follow-up ranged from 12 to 15 months. At last follow-up, the average combined range of motion was 76.1%. The average union time was 19 weeks. Out of 11 patients, two patients had good results, five patients had fair results, and four patient had poor results. There was no case of graft fracture, metastasis, death, local recurrence, or significant donor site morbidity. En bloc resection of giant cell tumors of the lower end radius is a widely accepted method. Reconstruction with non-vascularized fibular graft and internal fixation with LC-DCP along with brachytherapy minimizes the problem and gives satisfactory functional results with no recurrence.

Familial Fibrous Dysplasia in Two Subsequent Generations in a Family

Cherubism also known as familial fibrous dysplasia is a rare, non neoplastic, self-limiting fibro-osseous disorder commonly affecting the jaws in children and young adults. It is also described as familial multilocular cystic disease and familial benign giant cell tumor of the jaw. It is characterised by bilateral, painless swelling involving the mandible giving a cherubic (angel like) appearance. As it is a rare disorder, it is challenging to determine the disease frequency. A familial disorder like cherubism is more likely to be of autosomal dominant trait and shows 100% male sex predilection compared to female which is 50-70%. Although cherubism becomes noticeable in childhood (2-7 years of age), the treatment is however contentious as the disorder is believed to regress gradually after the onset of puberty and surgical intervention is to be decided based on aesthetics or functional difficulties. Numerous researches have been conducted to prove that cherubism is a genetically mediated disorder and the chromosome mapped is 4q16.3. This paper describes a case of an 11-year-old girl with cherubism and also encountered in two subsequent generations of a family, the father and his three children. The genetic and pedigree analysis carried out in this family proves hereditary basis in better understanding the genetic association related with the disorder. All the patients were educated about the disorder and were advised for surgical management after the onset of puberty

Outcome of Sellar and Suprasellar Brain Tumors with Retractorless Modified Subfrontal Approach

Aim: To determine the outcome of sellar and suprasellar brain tumors with retractorless modified subfrontal approach. Study design: Descriptive/observational study Place and duration of study: Department of Neurosurgery, Liaquat University Hospital Hyderabad/Jamshoro from 1st March 2020 to 28th February 2021. Methodology: Fifty patients of sellar and suprasellar brain tumors age between 15-70 years were enrolled. Patients details demographics age, sex and body mass index were recorded after taking written consent. The inter-hemispheric front-basal technique was used for all patients and the average follow-up time was 6 months. Postoperatively, magnetic resonance imaging (MRI) and computerized tomography (CT) scans in all patients were performed. After 12 hours, the postoperative CT scan was performed to monitor for persistent tumor and hemorrhage cerebral edema following an operation. Results: There were 30 (60%) male patients and 20 (40%) female patients. Mean age of the patients were 28.36±14.88 years with mean BMI 23.16±7.54 kg/m2. Most of the patients 20(40%) were from age group 15-30 years, followed by 31-40 years in 12 (24%) patients. Frequency of pituitary adenoma was found in 22 (44%) cases, craniopharyngioma found in 19 (38.7%) cases, arachnoid cyst found in 4 (8%), keratin flakes in 2 (4%) cases, benign giant cell tumor found in 2 (4%)olfactory groove meningioma in 1 (2%) and epidermoid in 1 (2%) cases. Post operatively 35 (70%) patients were completely recovered, complications found in 13 (26%) patients who were recovered later and frequency of not recovered patients was 2 (4%). According to Karnofsky performance, 16 (32%) patients had scale 30, scale 60 was in 2 (4%) cases, scale 70 in 4 (8%) patients, scale 80 in 19 (38%) and scale 90 in 9 (18%). Conclusion: This retractorless method is very effective and safe in the sellar and suprasellar region for excision of big tumors. This method allows the huge tumor to be removed without serious complication. Keywords: Sub-frontal approach, Sellar, Suprasellar, Brain tumor, Retractorless method

Case of Recurrence of 8 years back operated case of benign Giant Cell Tumor of the proximal Tibia with malignant fulminating mass over upper one third of leg

Giant cell tumor is a locally aggressive benign tumor of bone predominantly involving proximal tibia region. We are reporting Case of Recurrence of 8years back operated case of Giant Cell Tumor of the proximal Tibia previously treated with cementing and bone grafting now presented with malignant fulminating mass over upper one third of leg managed with amputation.

Primary malignant giant cell tumor of the sternum.

ABSTRACTPrimary malignant giant cell tumor (PMGCT) is a diagnosis based on the presence of a high-grade sarcomatous component along with a typical benign giant cell tumor (GCT). We report the first case of PMGCT of the sternum in a 28-year-old male with painless swelling over the manubrium sterni. The differential diagnoses of PMGCT and giant cell-rich osteosarcoma were considered. Surgical resection was performed, and the reconstruction was done with a neosternum using polymethyl methacrylate and prolene mesh. At 30 months follow-up, the patient is disease-free.

Drivers underpinning the malignant transformation of giant cell tumour of bone.

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.

Head and Neck Tumors in Children

The following chapter will provide a broad overview of the most common osseous lesions a surgeon will encounter when a pediatric patient presents with a bone-like head and neck tumor. The selected topics discussed below will be benign and malignant odontogenic cysts and tumors, and non-odontogenic benign (giant cell tumors, fibrous dysplasia, and juvenile ossifying fibroma) and malignant (osteosarcoma and ewing sarcoma) osseous tumors. This chapter has 4 figures, 3 tables, and 40 references Key Words: odontogenic cyst, non-odontogenic cysts, odontogenic tumors, giant cell tumor, fibrous dysplasia, juvenile ossifying fibroma, osteosarcoma, ewing sarcoma

An unusual cause of calcified pulmonary opacity: A metastasis of a benign giant cell tumour of bone

An unusual cause of calcified pulmonary opacity: A metastasis of a benign giant cell tumour of bone

Proton MR spectroscopy in characterization of focal bone lesions of peripheral skeleton

BackgroundThe aim of our study was to determine the value of single-voxel proton MR spectroscopy (1HMRS) in distinguishing benign from malignant focal bone lesions in the peripheral skeleton. MRI and 1HMRS was performed in 50 focal lesions (> 1 cm size) detected on radiographs of peripheral skeleton.1HMRS was performed at 1.5 T with TE of 144 ms with automatic shimming and water suppression. Qualitative analysis for a discrete choline peak at 3.2 ppm was done. Significance of the presence of choline peak on 1HMRS in distinguishing benign from malignant lesions was calculated using histopathology as a gold standard. Chi-square test was used and p value < 0.05 was considered significant.ResultsForty-one benign and 9 malignant lesions were confirmed by histopathological results. Amongst malignant lesions, choline peak was positive in all but 1 case of low-grade lymphoma. MR spectra of 11 benign lesions showed the presence of choline peak. All 7 benign giant cell tumors (GCT) were positive for choline peak. The sensitivity, specificity, PPV, NPV of proton MR spectroscopy in differentiating benign from malignant lesions were 87.5%,71%,38.8%, and 96.4% respectively. p value was significant (< 0.05).Conclusion1HMRS in focal bone lesions can help in the differentiation of malignant from benign musculoskeletal tumors. Although some benign lesions may show false-positive result, absence of choline peak is a reliable reassurance against malignancy. GCT is an exception amongst benign bone tumors as it consistently shows the presence of choline peak on 1HMRS.

Evaluation of neoplastic bone lesions: A morphological study at a tertiary care centre.

Objectives: To determine the pattern and frequency of various bone tumors at a tertiary care centre in rural area. Study Design: Retrospective study. Setting: Orthopaedic Peoples University of Medical and Health Sciences (PUMHS), Nawabshah. Period: June to December 2018. Material and Methods: The 5 years record from July 2015 to June 2018, of all the patients who were admitted and operated for any bone tumor was retrieved. A total of 68 cases were included in the study having complete file record, regardless of their age and sex, the histopathological report of their bone biopsy was mandatory for inclusion in the study. The patients of incomplete file record, without histopathological report and the cases of other bone non-neoplastic conditions were not included in the study. All the collected data was recorded on a proforma, analysed statistically and results were tabulated. Results: In present study 68 cases of bone tumors were evaluated. The patients were aged between 7.5 to 73 years with a mean of 26.4 + 12.7 years. Among these 68 cases 41 (60.3%) were male. The malignant tumors were diagnosed in 25 (36.8%) cases among these 14 (20.6%) cases in male patients and 11 (16.2%) cases in female. The overall benign tumors were observed in 43 (63.2%) cases, and among these 27 (39.7%) cases were male and 16 (23.5%) cases were female. Osteosarcoma was the most common malignant tumor diagnosed in 14/25 (56%) cases followed by chondrosarcoa in 4/25 (16%) cases. In benign tumors the osteochondroma was the commonest tumor detected in 11/43 (25.6%) cases followed by benign giant cell tumor which was diagnosed in 9/43 (21%) cases. Most of the malignant tumors (24%) were diagnosed in 11 - 20 years age group, and most of benign tumors were detected in 21-30 and 31- 40 age groups each comprising of 23.3% cases. Three cases of metastatic tumors were also diagnosed and all were present in older 61 – 70 years age group. Conclusion: The bone tumors are more common in males, occurred predominantly in the second decade of life. osteochondroma and osteosarcoma was the most common benign and malignant bone tumors. Metastatic lesions were observed in older age group.

Malignancy in Giant Cell Tumor of Bone: A Review of the Literature.

Background:Primary and recurrent giant cell tumor of bone is typically benign; however, rarely giant cell tumor of bone can undergo malignant transformation. Malignancy in giant cell tumor of bone may be primary (adjacent to benign giant cell tumor of bone at first diagnosis) or secondary (at the site of previously treated giant cell tumor of bone). Malignant giant cell tumor of bone has a poor prognosis; it is important to distinguish malignant from benign lesions to facilitate appropriate management. The true incidence of malignant giant cell tumor of bone is not known, probably owing to inaccurate diagnosis and inconsistent nomenclature. We have analyzed current data to provide a robust estimate of the incidence of malignancy in giant cell tumor of bone.Methods:A literature search was performed to source published reports of primary and secondary cases of malignant giant cell tumor of bone. Studies that reported a denominator were used to estimate the incidence of malignancy.Results:We identified 4 large series of patients with malignant giant cell tumor of bone that provided data on 2315 patients with giant cell tumor of bone. Across these studies, the cumulative incidence of malignancy was 4.0%; the cumulative incidence of primary malignancy was 1.6% compared with 2.4% for secondary malignancy. Our analyses confirmed that most malignant giant cell tumor of bone is secondary and occurs following radiation. In addition, data from 8 small series showed that 4.8% of patients with giant cell tumor of bone who received radiation therapy developed secondary malignancy.Conclusions:Malignant giant cell tumor of bone is rare, and its identification is hindered by a lack of clear diagnostic criteria. For optimal care of patients with giant cell tumor of bone, we recommend: comprehensive histologic sampling to ensure accurate diagnoses; watchful follow-up, particularly for patients treated with radiation; and timely treatment of local recurrence.

Malignant Sarcomatous Transformation of Benign Giant Cell Tumor of Bone after Treatment with Denosumab Therapy: A Literature Review of Reported Cases.

Giant cell tumor of bone (GCTB) is a biologically benign and locally aggressive tumor that most often affects the epiphyseal and metaphyseal sites of long bones in the young adult population. Overexpression of receptor activator of nuclear factor kappa B ligand (RANKL) by cancerous mesenchymal stromal cells stimulates a signal transduction cascade that recruits and activates multinucleated osteoclast-like giant cells, resulting in pathologic bone resorption. Denosumab, an RANKL inhibitor that blocks the RANKL-mediated osteoclast activation, has been recently approved by the United States Food and Drug Administration (FDA) for the treatment of aggressive GCTB. Although uncommon, several studies reported drug-related malignant morphological transformation of benign GCTB following treatment with denosumab therapy. The aim of the article was to review the clinicopathological characteristics of all the reported cases of malignant sarcomatous transformation of GCTB after treatment with denosumab therapy in patients without any history of prior exposure to radiotherapy.

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.