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Background: Giant cell tumor of bone (GCTB) is the most common primary tumor of proximal fibula. Because of its close proximity to vascular structures, common peroneal nerve (CPN) and attachment of lateral collateral ligament (LCL), proximal fibulectomy poses unique challenges. We analyzed oncological and functional outcome of patients who underwent proximal fibulectomy for GCTB of proximal fibula. Material and methods: Between January 2006 and December 2020, 23 patients underwent proximal fibulectomy for GCTB of proximal fibula, four were recurrent tumors. Mean resection length was 9 cm (5 to 15 cm). The LCL and biceps tendon were not reconstructed in 22 cases. The common peroneal nerve was sacrificed in seven patients including three recurrent cases. Functional status was assessed using the Musculoskeletal Tumour Society (MSTS) scoring system. Results: There were two vascular complications and one infection. With 4 patients lost to follow up, mean follow up was 90 months (12 to 197). No patient had local or distant recurrence. Mean MSTS score was 26 (21 to 30). Eleven of 23 patients (48%) had loss of common peroneal nerve function with poorer functional outcome. No patient had symptoms suggestive of knee instability. Conclusion: Proximal fibulectomy is oncologically safe. Reconstruction of the LCL attachment is not mandatory and patients do not have symptomatic knee instability. Functional outcomes are compromised after sacrifice of common peroneal nerve and may be potentially improved with tendon transfers at index surgery. [ABSTRACT FROM AUTHOR]

Background and Objectives: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive, benign neoplasm arising from the synovium of joints, tendon sheaths, and bursa. There are two main subtypes of TGCT: localized-type TGCT(L-TGCT) and diffuse-type TGCT (D-TGCT). While surgical excision is still considered the gold standard of treatment, the high recurrence rate, especially for D-TGCT, may suggest the need for other treatment modalities. Materials and Methods: This study reviews current literature on the current treatment modalities for refractory-relapsed TGCT disease. Results: The gold standard of treatment modality in TGCT remains surgical excision of the tumor nevertheless, the elevated recurrence rate and refractory disease, particularly in D-TGCT indicates and underscores the necessity for additional treatment alternatives. Conclusions: TGCT is a benign tumor with inflammatory features and a potential destructive and aggressive course that can lead to significant morbidity and functional impairment with a high impact on quality of life. Surgical resection remains the gold standard current treatment and the optimal surgical approach depends on the location and extent of the tumor. Systemic therapies have been recently used for relapsed mainly cases. [ABSTRACT FROM AUTHOR]

Giant cell tumors (GCTs) are rare neoplasms, primarily found in long bones, typically affecting the epiphysis of the distal femur, proximal tibia, and distal radius. However, their occurrence in the cervical spine is exceedingly rare. Here, we present a case report of a 21-year-old female patient who presented with progressive neck pain, radiating numbness, and right hemiparesis. Radiographic imaging revealed a lytic lesion in the C3 vertebral body, further characterized by magnetic resonance imaging (MRI) and computed tomography (CT) scans. The patient underwent surgery for stabilization of the cervico-occipital hinge, decompression, and biopsy. Histopathological examination confirmed the diagnosis of a giant cell tumor. Postoperatively, the patient showed improvement in motor impairment, cervical pain, and numbness. She was proposed for adjuvant treatment based on Denosumab. However, she returned 1 month after surgery with worsened motor deficit, developing tetraparesis. Control MRI revealed a tumor flare-up. The decision was made not to reoperate on the patient and to accelerate the administration of Denosumab. Meanwhile, she experienced a pulmonary embolism leading to her demise. This case underscores the importance of considering giant cell tumors in the differential diagnosis of cervical spine lesions and emphasizes the successful and prompt management through a multidisciplinary approach involving surgical intervention and adjuvant therapy.

Background: Giant cell tumor is a locally aggressive tumor of the bone. In the predenosumab era, treatment comprised surgery or radiotherapy in potentially resectable lesions. After the advent of denosumab, utilizing neoadjuvant denosumab followed by surgery has emerged as an effective treatment option. Objectives: We aimed primarily to assess and compare the locoregional failure-free survival of patients with giant cell tumor of the bone (GCTB) treated with either external beam radiotherapy or neoadjuvant denosumab followed by surgery. The secondary objective was to assess and compare the quality of life (QoL) of patients between the two arms. Materials and Methods: In this single institutional retrospective study, conducted between August 2022 and August 2023 in the Department of Radiotherapy in the Institute of Post Graduate Medical Education and Research and SSKM Hospital Kolkata, West Bengal, India, we included patients with histopathologically proven GCTB. These patients were treated with either radiotherapy (54 Gray in 27 fractions) or neoadjuvant denosumab (for 8 doses) followed by surgery and were followed up at least for 6 months after completing the first-line therapy. Patients were monitored every 3 months for 2 years and then every 6 months thereafter. Locoregional control of the tumor was assessed by clinical examination and imaging. QoL was assessed and compared by the EQ-5D-5L questionnaire and health today scoring system. Results: We enrolled 22 patients in the radiotherapy alone group and 26 patients in the group receiving neoadjuvant denosumab followed by surgery. The 5-year locoregional failure-free survival was achieved in 18/22 patients (81.8%) in the radiotherapy arm and in 20/26 patients (76.9%) in the neoadjuvant denosumab group (P, 0.680). There was no significant difference in the QoL between the two groups of patients (P, 0.850). Conclusion: Radiotherapy and neoadjuvant denosumab followed by surgery result in similar locoregional control and quality of life in the patients with giant cell tumor of bones, and both can be considered appropriate treatment options in patients with initially unresectable tumors.

Abstract Background Tenosynovial giant cell tumor is a rare soft tissue tumor of the synovium of joint, bursae, or tendon sheath. It is divided into localized or diffuse types on the basis of the growth pattern. Localized tenosynovial giant cell tumors are usually benign and treated successfully by excision. Diffuse tenosynovial giant cell tumors, in contrast to localized type, can destroy bone and cartilage and are associated with frequent local recurrences and distant metastasis. Localized type tenosynovial giant cell tumors rarely metastasize to distant organs. Here, we report a case of localized tenosynovial giant cell tumor presenting with lung metastases and systematically review literature. Case presentation A 55-year-old Asian male presented with a dry cough, right-sided chest pain and progressive dyspnea for 1 month. At 18 months before this presentation, he had undergone excision of a painless swelling on his right index finger. The swelling recurred within 3 months of excision, and a biopsy was then suggestive of a giant cell tumor. Given the suspicion of a giant cell tumor, a wide excision of the lesion was performed and the excisional biopsy was consistent with a diagnosis of tenosynovial giant cell tumor, localized type. At admission to our hospital, the patient had tachypnoea and absent breath sounds on the right side. A chest radiograph showed a right-sided pleural effusion with a homogenous opacity in the left mid-zone. A contrast-enhanced computed tomography of the chest and abdomen showed right massive pleural effusion and bilateral multiple lobulated heterogeneously enhancing pleural-based masses with areas of internal calcification. Pleural fluid analysis revealed an exudate with no malignant cells on cytology. A lung biopsy showed osteoclast-like giant cells and mononuclear spindle cells with areas of hemorrhage and necrosis, suggesting tenosynovial giant cell tumor metastasis. A final diagnosis of localized type tenosynovial giant cell tumor of the right index finger with metastases to the lungs and pleura was made. The patient passed away after receiving three cycles of denosumab injection owing to progressive disease. Conclusion Lung metastasis is extremely rare in patients with localized tenosynovial giant cell tumor. The survival is usually poor in patients with lung metastasis. A close follow-up of patients with localized type tenosynovial giant cell tumor is necessary for early detection of pleuropulmonary complications.

Initial diagnostic ultrasound of a 22-year-old female patient presenting with a palpable breast mass revealed a suspicious mass initially thought to arise from the breast. However, follow-up diagnostic mammography was normal without evidence of the 5 cm mass seen on ultrasound, and pathology results from ultrasound-guided core needle biopsy raised suspicion for giant cell tumor, making chest wall origin of the mass more likely. Further CT and MRI imaging indeed revealed a locally invasive mass arising from the anterior fifth rib. The patient was treated with denosumab to decrease tumor burden before surgery, and subsequently underwent successful surgical resection of the tumor with mesh overlay and flap reconstruction of the chest wall defect. This case highlights the importance of keeping chest wall lesions in the differential for lesions presenting clinically as breast lesions. Despite the rarity of giant cell tumor of the anterior rib and its unusual presentation as a breast mass, appropriate diagnostic imaging work-up allowed for successful diagnosis and treatment in this case.

Background: Tenosynovial giant cell tumor is a rare soft tissue tumor of the synovium of joint, bursae, or tendon sheath. It is divided into localized or diffuse types on the basis of the growth pattern. Localized tenosynovial giant cell tumors are usually benign and treated successfully by excision. Diffuse tenosynovial giant cell tumors, in contrast to localized type, can destroy bone and cartilage and are associated with frequent local recurrences and distant metastasis. Localized type tenosynovial giant cell tumors rarely metastasize to distant organs. Here, we report a case of localized tenosynovial giant cell tumor presenting with lung metastases and systematically review literature. Case presentation: A 55-year-old Asian male presented with a dry cough, right-sided chest pain and progressive dyspnea for 1 month. At 18 months before this presentation, he had undergone excision of a painless swelling on his right index finger. The swelling recurred within 3 months of excision, and a biopsy was then suggestive of a giant cell tumor. Given the suspicion of a giant cell tumor, a wide excision of the lesion was performed and the excisional biopsy was consistent with a diagnosis of tenosynovial giant cell tumor, localized type. At admission to our hospital, the patient had tachypnoea and absent breath sounds on the right side. A chest radiograph showed a right-sided pleural effusion with a homogenous opacity in the left mid-zone. A contrast-enhanced computed tomography of the chest and abdomen showed right massive pleural effusion and bilateral multiple lobulated heterogeneously enhancing pleural-based masses with areas of internal calcification. Pleural fluid analysis revealed an exudate with no malignant cells on cytology. A lung biopsy showed osteoclast-like giant cells and mononuclear spindle cells with areas of hemorrhage and necrosis, suggesting tenosynovial giant cell tumor metastasis. A final diagnosis of localized type tenosynovial giant cell tumor of the right index finger with metastases to the lungs and pleura was made. The patient passed away after receiving three cycles of denosumab injection owing to progressive disease. Conclusion: Lung metastasis is extremely rare in patients with localized tenosynovial giant cell tumor. The survival is usually poor in patients with lung metastasis. A close follow-up of patients with localized type tenosynovial giant cell tumor is necessary for early detection of pleuropulmonary complications. [ABSTRACT FROM AUTHOR]

Introduction Neoadjuvant chemotherapy is now considered an effective way to treat Campanacci grade 2 and 3 giant cell tumors (GCTs). Assessment of these drugs is essential clinically, radiologically, and pathologically. This study analyzes the early results of angiogenesis inhibitors (interferons) in the aggressive GCT of bone. Methodology A prospective pilot study was conducted from January 2021 to July 2022 including eight biopsy-proven GCT patients subjected to interferon therapy. Radiological assessment was done with changes on plain radiograph, computerized tomography scan, and magnetic resonance imaging. Histopathological examination was done by changes in the biopsy and resected segment. Results Out of the eight patients included in the study, 26% (n = 3) were males and 62% (n = 5) were females, with mean age of the patients being 24.6 ± 8.48 years (range: 22–38). There was significant reduction of the size of swelling (p -value: 0.049), significant reduction in Visual Analog Scale score (p -value: 0.011), significant decrease in swelling size on radiograph (p -value: 0.012), significant marginal sclerosis (p -value: 0.001), significant neocortex formation on radiographs (p -value: 0.001), significant result in and osteoid formation (p -value: 0.001) on histology. Whereas Campanacci grade on plain radiographs, number of viable cells, and number of viable stromal cell were not statistically different in comparison with pretherapy and posttherapy status. Conclusion Interferon therapy in a GCT has potential beneficiary effect in terms of clinical, radiological, and pathological outcomes. It might prove to be an effective alternative to standard neoadjuvant chemotherapy in the management of aggressive GCT of bones. Level of Evidence III. [ABSTRACT FROM AUTHOR]

Giant Cell Tumours (GCT) are benign tumours with aggressive potential that disrupt the local bony architecture, which can be especially problematic in peri-articular locations. Our aim was to assess the outcomes of patients with GCT of the distal ulna who were treated by resection without reconstruction. The study included 21 patients with distal ulna GCT that were treated with resection without reconstruction. There were 12 males and 9 females, with a mean age of 30.4years (range 14–45 years). The patients mean follow-up period was 4.4 years, with a two-year minimum follow-up. Painful swelling was the presenting symtom in all cases. Nineteen patients had Campanacci grade 3 and two had Campanacci grade 2. The mean resected length of the distal ulna was 6.8 cm (range 4–10) cm. The Musculoskeletal Tumor Society score (MSTS) was 26.1. (range 22–28). Grip strength of the affected hand was reduced by 10.5% on average. (range 0%–16%). Two patients were having multi-centric disease on presentation and none of the cases had pathological fracture on presentation. One case had a local recurrence which was treated with surgery. Based on current study, GCT of the distal ulna, en bloc resection without reconstruction can be recommended as a valuable treatment option for Campanacci grades 2 and 3 tumours. Resection of the distal end of the ulna without reconstruction results in excellent functional outcomes, with forearm rotational movement and hand function preserved. According to review of literature this is the largest series of GCT Ulna and we recommend a multicentre and comparitive studies on this topic. [ABSTRACT FROM AUTHOR]

Many hand surgeons treat benign bone tumors without referral to orthopedic oncologists. However, there have been considerable advances in medical therapy for some of these tumors, with which hand surgeons may not be as familiar. This review focuses on the mechanism and uses of denosumab in the treatment of benign tumors of bone. Although the hand surgeon may not be directly prescribing this therapy, they are often the only physician treating the patient for these conditions. As such, awareness regarding the use of this therapy in reducing pain, decreasing tumor volume, and treatment of potential lung metastases is critical to those taking on these cases without the support of an orthopedic oncologist. This article aims to familiarize hand surgeons with denosumab to help promote knowledge of this therapeutic option and the potential role of this medication in the treatment of primary bone tumors in the hand.

The two cases presented demonstrate the management of aneurysmal bone cysts of the metacarpal, which destroyed the normal bone architecture. Treatment of both cases included wide resection and metacarpal reconstruction with an intercalary fibular allograft. Denosumab use contrasts these two cases and is helpful in reestablishment of a cortical rim for fixation in the absence of a 1-cm margin proximally or distally to preserve the native carpometacarpal and metacarpophalangeal joints. Surgical resection and allograft reconstruction is a viable treatment for expansile metacarpal aneurysmal bone cysts, and neoadjuvant denosumab has utility in creating an ossified margin for fixation.

Giant cell tumor of bone (GCTB) is characterized by uncertain biological behavior due to its local aggressiveness and metastasizing potential. In this study, we conducted a meta-analysis of the contemporary literature to evaluate all management strategies for GCTB metastases. A combination of the terms “lung metastases”, “giant cell tumor”, “bone”, “treatment”, and “oncologic outcomes” returned 133 patients meeting our inclusion criteria: 64 males and 69 females, with a median age of 28 years (7–63), at the onset of primary GCTB. Lung metastases typically occur at a mean interval of 26 months (range: 0–143 months) after treatment of the primary site, commonly presenting as multiple and bilateral lesions. Various treatment approaches, including surgery, chemotherapy, radiotherapy, and drug administration, were employed, while 35 patients underwent routine monitoring only. Upon a mean follow-up of about 7 years (range: 1–32 years), 90% of patients were found to be alive, while 10% had died. Death occurred in 25% of patients who had chemotherapy, whereas 96% of those not treated or treated with Denosumab alone were alive at a mean follow-up of 6 years (range: 1–19 years). Given the typically favorable prognosis of lung metastases in patients with GCTB, additional interventions beyond a histological diagnosis confirmation may not be needed. Denosumab, by reducing the progression of the disease, can play a pivotal role in averting or delaying lung failure.

Background Giant cell tumors of the tendon sheath (GCTTS) of the hand are considered the second most common benign tumors of the hand after ganglion cysts. Excision biopsy is considered the standard treatment at present. They are notorious for having a very high rate of recurrence as given in many studies. Many factors are said to be associated with recurrence of the tumors. The goal of this study is to evaluate the long-term results of a series of 48 patients operated on at a single institute and to find out if there is any correlation between the proposed risk factors with recurrence.

Paralaryngeal mass with secondary extension to the thyroid cartilage involving confluent cysts, subjected to conservative surgical treatment with a diagnosis of giant cell tumor of soft tissues, a neoplasm morphologically similar but genetically unrelated to osseous giant cell tumors.

Purpose: Giant cell tumor (GCT) of the proximal femur is relatively rare, with only a few case series reported thus far. This study aimed to evaluate the clinical outcomes of GCT of the proximal femur treated with intralesional curettage and expand the understanding of their characteristics and treatment considerations. Materials and Methods: Fifteen cases treated with curettage for GCT of the proximal femur between 2007 and 2020 were reviewed. The median follow-up was 46 months (25–150 months). There were 10 males and 5 females with a median age of 26 years (17–71 years). After curettage, the bone defect was filled with either an allograft (7 cases) or bone cement (8 cases). Results: The postoperative complications were local recurrences in three cases (20.0%), including malignant transformation in one case and a femur neck fracture in one case (6.7%) following curettage and strut allograft. Among the 15 cases, 13 (86.7%) retained their native joint at the last follow-up. No patients developed degenerative changes or osteonecrosis. Conclusion: The results of proximal femoral GCT with curettage were acceptable despite local recurrences in three cases (20.0%), and femur neck fracture in one case. An appropriate surgical approach and reconstruction according to the extent of the lesion are necessary for successful treatment. [ABSTRACT FROM AUTHOR]

Giant cell tumor (GCT) of bone is most commonly a benign but locally aggressive primary bone tumor. Spinal GCTs account for 2.7% to 6.5% of all GCTs in bone. En bloc resection, which is the preferred treatment for GCT of the spine, may not always be feasible due to the location, extent of the tumor, and/or the patient's comorbidities. Neoadjuvant denosumab has recently been shown to be effective in downstaging GCT, decreasing the size and extent of GCTs. However, the risk of neurologic deterioration is of major concern for patients with epidural spinal cord compression due to spinal GCT. We experienced this concern when a patient presented to our institution with a midthoracic spinal GCT with progressive epidural disease. The patient was not a good surgical candidate due to severe cardiac disease and uncontrolled diabetes. In considering nonoperative management for this patient, we asked ourselves the following question: What is the risk that this patient will develop neurologic deterioration if we do not urgently operate and opt to treat him with denosumab instead? The purpose of this study was to assess the literature to (1) determine the risk of neurological deterioration in patients receiving neoadjuvant denosumab for the treatment of spinal GCT and (2) to evaluate the secondary outcomes including radiographic features, surgical/technical complexity, and histological features after treatment. Meta-analysis of the literature. Surgical cases of spinal GCT that (1) presented with type III Campanacci lesions, (2) had epidural disease classified as Bilsky type 1B or above and (3) received neoadjuvant denosumab therapy. The primary outcome measure of interest was neurologic status during denosumab treatment. Secondary outcome measures of interest included radiographic features, surgical/technical complexity, histological features, tumor recurrence, and metastasis. Using predetermined inclusion and exclusion criteria, PubMed and Embase electronic databases were searched in August 2022 for articles reporting spinal GCTs treated with neoadjuvant denosumab and surgery. Keywords used were "Spine" AND "Giant Cell Tumor" AND "Denosumab." A total of 428 articles were identified and screened. A total of 22 patients from 12 studies were included for review. 17 patients were female (17/22, 77%), mean age was 32 years (18–62 years) and average follow-up was 21 months. Most GCTs occurred in the thoracic and thoracolumbar spine (11 patients, 50%), followed by 36% in the lumbar spine and 14% in the cervical spine. Almost half of the patients had neurological deficits at presentation (10/22 patients, 45%), and more than 60% had Bilsky 2 or 3 epidural spinal cord compression. None of the patients deteriorated neurologically, irrespective of their neurological status at presentation (p-value=.02, CI −2.58 to −0.18). There were no local recurrences reported. One patient was found to have lung nodules postoperatively. More than 90% of cases had decreased overall tumor size and increased bone formation. Surgical dissection was facilitated in more than 85% of those who had documented surgical procedures. Four patients (18%) underwent initial spinal stabilization followed by neoadjuvant denosumab and then surgical excision of the GCT. Regarding the histologic analyses, denosumab eradicated the giant cells in 95% of cases. However, residual Receptor Activator of Nuclear Factor Kappa B Ligand (RANKL)-positive stromal cells were noted, in 27% (6 cases). Neoadjuvant denosumab was a safe and effective means of treating spinal GCTs prior to surgery. Neurologic status remained stable or improved in all cases included in our review, irrespective of the presenting neurologic status. The most appropriate dosage and duration of denosumab therapy is yet to be determined. We recommend future well-designed studies to further evaluate the use of neoadjuvant denosumab for patients with spinal GCT. [ABSTRACT FROM AUTHOR]

Giant cell tumors of bone (GCT) are rare soft tissue tumors, that account for 3%–5% of primary bone tumors with <2% occurring in the head and neck. The nasal cavity is a highly unusual site of presentation. We reviewed 15 cases of GCT of nasal cavity and paranasal sinuses. We add 1 case to the literature. The case herein reported, appears to be the second nasal fossa GCT described in the literature and the first documented case with multifocal localization. A case of multifocal GCT of the nasal cavity is described. Although rare in the general population, GCT should be included among the possibilities in the differential diagnosis when evaluating tumors of the head and neck. Management of this particular tumor remains challenging; surgical removal is still the gold standard treatment, preferring a minimally invasive trans‐nasal approach to reduce intra and post‐operative morbidity. Laryngoscope, 134:2774–2778, 2024 [ABSTRACT FROM AUTHOR]

Objective: To compare the clinical outcomes of microwave-assisted intralesional curettage(MAIC) with those of en bloc resection and autogenous fibular reconstruction (EBR-AFR) for treating grade III giant cell tumor of the bone (GCTB) of the distal radius and to elucidate the indications for wrist preservation surgery. Materials and methods: In this retrospective study, 19 patients with grade III GCTB of the distal radius who underwent surgery at three medical institutions were included and categorized based on their surgical pattern. Seven patients underwent MAIC and internal fixation with bone cement (MAIC group) and 12 underwent EBR-AFR (EBR-AFR group). To evaluate the function of the affected limb postoperatively, wrist range of motion, grip strength, Musculoskeletal Tumor Society (MSTS) scores were recorded. Results: The follow-up time of the MAIC group was 73.57 ± 28.61 (36-116) months, with no recurrence or lung metastasis. In contrast, the follow-up time of the EBR-AFR group was 55.67 ± 28.74 (36-132) months, with 1 case of local recurrence (8.3%, 1/12) and 1 case of lung metastasis (8.3%, 1/12). The wrist flexion, extension, supination, pronation, grip strength were better in the MAIC group than in the EBR-AFR group. Although there was no statistically significant difference in the MSTS score between the two groups, it is noteworthy that the MAIC group exhibited significantly superior emotional acceptance and hand positioning compared to the EBR-AFR group(p < 0.05). Conclusion: The functional outcomes of the MAIC group are better. The treatment strategy for grade III GCTB of the distal radius should be determined based on the specific preoperative imaging findings. Nevertheless, MAIC can be the preferred surgical approach for most patients with grade III GCTB of the distal radius, particularly for young patients. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Giant cell tumor of bone (GCT) is a rare neoplasm which often presents as a lytic lesion in the epiphyseal region of long bones and which are usually accompanied by pain, swelling, and restricted movement. Giant cell tumor of bone (GCT) is a rare neoplasm that affects individuals in their third and fourth decades of life. Clinically, it often presents as a lytic lesion in the epiphyseal region of bones, notably the distal femur and proximal tibia. Radiologically, GCT appears as a distinct lytic lesion in the epiphyseal region. Histopathologically, GCTs are composed of mononuclear cells, macrophages, and multinuclear giant cells, indicative of osteoclastogenic stromal tumors. A 37‐year‐old man presented with left wrist pain, swelling, and restricted movement persisting for a year, worsening over the last 7 months. Radiographic assessments revealed a distal radius bone mass involving the radiocarpal joint. Biopsy confirmed a GCT with extension into peripheral muscle. PET/CT scan showed localized pathology without metastasis. Histopathologically, GCT exhibited multinucleated giant cells, spindle cells, and aneurysmal bone cyst‐like regions with coagulation necrosis. Surgical resection involved en‐bloc removal and reconstruction with a non‐vascularized radius bone graft. Postoperatively, the patient showed no complications at the one‐year follow‐up, suggesting successful intervention. [ABSTRACT FROM AUTHOR]

Context Denosumab is an effective treatment for many receptor activator of nuclear factor kappa-B ligand (RANKL)-mediated disorders but there are potential safety considerations and limited data to guide its use in children and adolescents. Objective This document seeks to summarize the evidence and provide expert opinion on safe and appropriate use of denosumab in pediatric RANKL-mediated disorders. Participants Ten experts in pediatric bone and mineral medicine from 6 countries with experience in the use of denosumab participated in the creation of this document. Evidence Data were sourced from the published literature, primarily consisting of case reports/series and review articles because of the lack of higher level evidence. Expert opinion of the authors was used substantially when no published data were available. Conclusion Denosumab is an effective treatment for RANKL-mediated disorders in children and adolescents but is often not curative and, in some cases, is best used in conjunction with surgical or other medical treatments. Careful multidisciplinary planning is required to define the goals of treatment and expert oversight needed to manage the risk of mineral abnormalities. Substantive, collaborative research efforts are needed to determine optimal treatment regimens and minimize risks. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is characterized by uncertain biological behavior due to its local aggressiveness and metastasizing potential. In this study, we conducted a meta-analysis of the contemporary literature to evaluate all management strategies for GCTB metastases. A combination of the terms "lung metastases", "giant cell tumor", "bone", "treatment", and "oncologic outcomes" returned 133 patients meeting our inclusion criteria: 64 males and 69 females, with a median age of 28 years (7–63), at the onset of primary GCTB. Lung metastases typically occur at a mean interval of 26 months (range: 0–143 months) after treatment of the primary site, commonly presenting as multiple and bilateral lesions. Various treatment approaches, including surgery, chemotherapy, radiotherapy, and drug administration, were employed, while 35 patients underwent routine monitoring only. Upon a mean follow-up of about 7 years (range: 1–32 years), 90% of patients were found to be alive, while 10% had died. Death occurred in 25% of patients who had chemotherapy, whereas 96% of those not treated or treated with Denosumab alone were alive at a mean follow-up of 6 years (range: 1–19 years). Given the typically favorable prognosis of lung metastases in patients with GCTB, additional interventions beyond a histological diagnosis confirmation may not be needed. Denosumab, by reducing the progression of the disease, can play a pivotal role in averting or delaying lung failure. [ABSTRACT FROM AUTHOR]

Giant cell tumor is locally aggressive primary benign neoplasm of bone with tendency of frequent recurrence, metastasis and malignant transformation. Because of the rarity of the disease involving mandible, no definite treatment guideline is established. Surgical treatment is the treatment of choice for giant cell tumor. Due to its proximity to vital structures including skull base, the recurrent disease associated with less invasive procedure could be difficult to manage while more invasive procedure will result in higher morbidity and complex reconstruction. Medical management with denosumab or zoledronic acid has been advocated in surgically unresectable disease. We present a rare case of giant cell tumor of mandible. Patient was a 33 years old lady who presented with gradually progressive painful swelling in chin. After exclusion of distant metastasis by F-18 FDG PET scan, she underwent en-bloc resection of the tumor with free fibula flap reconstruction. During 6 months of follow up visit patient had no recurrence. [ABSTRACT FROM AUTHOR]

Background Giant cell tumors of the tendon sheath (GCTTS) of the hand are considered the second most common benign tumors of the hand after ganglion cysts. Excision biopsy is considered the standard treatment at present. They are notorious for having a very high rate of recurrence as given in many studies. Many factors are said to be associated with recurrence of the tumors. The goal of this study is to evaluate the long-term results of a series of 48 patients operated on at a single institute and to find out if there is any correlation between the proposed risk factors with recurrence. Methods A retrospective analysis was done in cases of GCTTS operated on between 2015 and 2021. The patients were invited for follow-up for a minimum of 2 years, and the patient files were reviewed. Further data were collected at follow-up including recurrence, range of movement, sensation, skin necrosis, scarring, and digital neuropathy. A data analysis was done. The correlation between the proposed risk factors and recurrence was calculated with the Pearson correlation coefficient. A p -value of less than 0.05 was considered statistically significant. Results During the 6 years, 48 patients were operated on. Recurrence was observed in eight patients (16%) at an average of 38.7 months from the time of surgery. Of the risk factors, tumors with satellite nodules and tumor adjacency to joint correlated significantly with recurrence. No complications were observed during follow-up. Conclusion GCTTS of the hand has a high propensity to recur. The presence of satellite nodules and proximity to interphalangeal joints are two important risk factors for recurrence. Magnification during surgery ensures complete excision of the tumor and reduces the chance of recurrence. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and occasionally metastatic tumor. In this article, we present a case of a 37-year-old woman who presented with fracture at the distal femur due to GCTB. Bone segment resection and reconstruction were done, and histopathology showed tumor features for GCTB. Later, multiple lung metastasis was found 22 months post-operation, which was verified by biopsy. Then systemic denosumab therapy with different intervals (1-month and 2-month) was tried as the treatment. It was clarified that monthly denosumab administration, instead of 2-month interval, was required to control the progression of the unresectable multiple lung metastasis from GCTB, which could be a choice for the future treatment of these patients.

Objective Re‐resection of spinal giant cell tumors is an exceedingly difficult procedure. Moreover, the prognosis of patients with en bloc resection or intralesional excision for re‐resection has rarely been reported. This study aimed to compare the prognostic value of en bloc resection with that of intralesional excision in patients undergoing re‐resection for giant cell tumors of the spine. Methods This retrospective analysis evaluated patients who underwent revision surgeries for relapse of giant cell tumors of the spine at our center between January 2005 and January 2021. Local progression‐free survival represents the duration between en bloc resection or intralesional excision and tumor recurrence. Neurological recovery, survival rates, local control, and complications were evaluated. The Kaplan–Meier estimator was used for survival analysis. Results A total of 22 patients (nine men and 13 women) with a mean age of 34.1 (range 19–63) years were included. Significant statistical differences were found in the local tumor recurrence rate between patients treated with en bloc resection and those treated with intralesional excision (p

Giant cell tumor (GCT) is typically a benign tumor of the skeletal system that mainly presents with bone pain. Pulmonary metastasis is one of the distant presentations of GCT in patients who have previously undergone surgical resection of the tumor. Among the various presentations of pulmonary metastasis in GCT, lesions with arteriovenous malformation (AVM) features are rare and have only been reported in a few cases. In this case report, we present the case of a 29-year-old female patient who had previously undergone surgical resection of a GCT in her right lower extremity 4 years ago. The patient was referred to us with progressive dyspnea, and a lesion resembling an AVM was found during radiologic evaluation using chest computed tomography. Pathologic evaluation of the lesion after biopsy revealed that it was a metastasis of GCT presenting with vascular-like features in the lung. This study reports on a very rare occurrence of GCT pulmonary metastasis with an AVM appearance on imaging, highlighting the clinical importance of atypical presentations of pulmonary metastasis in patients with a history of GCT. Appropriate and timely screening and management of such lesions may prevent adverse outcomes such as massive hemorrhage and deterioration of lung function.

Tenosynovial giant cell tumors represent a group of typically non-malignant tumors found within the joints and soft tissues. The occurrence of tenosynovial giant cell tumor alongside hematologic malignancies is an infrequent finding. Herein, we report a patient who presented with coinciding Hodgkin Lymphoma (HL) and tenosynovial giant cell tumor before chemotherapy initiation. The case was discovered during initial assessment using [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography/computed tomography (PET/CT) imaging for HL staging. An unrelated hypermetabolic mass within the left knee joint led to the discovery of this unusual case, which led to a CT-guided biopsy and tenosynovial giant cell tumor discovery. This was clearly demonstrated in interim and end-of-therapy PET/CT studies when all lymphomatous lesions had resolved but the tenosynovial giant cell tumor remained. This case serves as a reminder of the intricate nature of oncological pathology and emphasizes the need for thorough and vigilant diagnostic evaluation for optimal management plan.

Background and Objectives: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive, benign neoplasm arising from the synovium of joints, tendon sheaths, and bursa. There are two main subtypes of TGCT: localized-type TGCT(L-TGCT) and diffuse-type TGCT (D-TGCT). While surgical excision is still considered the gold standard of treatment, the high recurrence rate, especially for D-TGCT, may suggest the need for other treatment modalities. Materials and Methods: This study reviews current literature on the current treatment modalities for refractory-relapsed TGCT disease. Results: The gold standard of treatment modality in TGCT remains surgical excision of the tumor nevertheless, the elevated recurrence rate and refractory disease, particularly in D-TGCT indicates and underscores the necessity for additional treatment alternatives. Conclusions: TGCT is a benign tumor with inflammatory features and a potential destructive and aggressive course that can lead to significant morbidity and functional impairment with a high impact on quality of life. Surgical resection remains the gold standard current treatment and the optimal surgical approach depends on the location and extent of the tumor. Systemic therapies have been recently used for relapsed mainly cases.

Objective: Re‐resection of spinal giant cell tumors is an exceedingly difficult procedure. Moreover, the prognosis of patients with en bloc resection or intralesional excision for re‐resection has rarely been reported. This study aimed to compare the prognostic value of en bloc resection with that of intralesional excision in patients undergoing re‐resection for giant cell tumors of the spine. Methods: This retrospective analysis evaluated patients who underwent revision surgeries for relapse of giant cell tumors of the spine at our center between January 2005 and January 2021. Local progression‐free survival represents the duration between en bloc resection or intralesional excision and tumor recurrence. Neurological recovery, survival rates, local control, and complications were evaluated. The Kaplan–Meier estimator was used for survival analysis. Results: A total of 22 patients (nine men and 13 women) with a mean age of 34.1 (range 19–63) years were included. Significant statistical differences were found in the local tumor recurrence rate between patients treated with en bloc resection and those treated with intralesional excision (p < 0.05). The 5‐ and 10‐year local progression‐free survival rates were both 90% in the en bloc resection group, while in the intralesional excision group, the 5‐year local progression‐free survival rate was 80% with a 10‐year rate of 45.7%. The en bloc resection group had a lower local tumor recurrence rate than that of the intralesional excision group (p < 0.05), but the former had a higher rate of complications (p = 0.015). Conclusions: This study revealed a low local recurrence rate in patients who underwent en bloc resection for giant cell tumors, while the perioperative complication rate was high. [ABSTRACT FROM AUTHOR]

The aim of this study is to determine the treatment modalities and clinical characteristics of 12 patients diagnosed with giant cell tumor (GCT) of the hand and foot. The clinical findings, treatment modalities, and treatment failures of 12 patients with giant cell tumors of the hand and foot bones between 2007 and 2018 years were evaluated retrospectively. The average age at diagnosis was 29.2 ± 14.9 std. (between 16 and 62 years old), 8 males (66.6%) and 4 females (33.3%). Tumor was more frequently located in the talus, metacarpal, and metatarsal bones. The mean tumor size was 3.1 ± 1.1 cm (between 2.2 and 5.3 cm). The mean post-operative follow-up period was 76.3 ± 42.5 (between 12 and 139 months). The most of patients' (58.3%) common complaints were pain. The most commonly used surgical method was curettage + autografting (91.7%). Infection was seen in one patient after relapse surgery. Recurrence occurred in 33.3% of the patients in the first year. Only one patient was detected to have knee and lung metastases. Swelling and pain in the hand and foot should be examined for tumor lesions. When a bone lesion is detected, the giant cell tumor of the bone should be included in the differential diagnosis. Patients with giant cell tumors should be followed closely for recurrence and metastasis after treatment. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is a rare tumor of the bone that is locally invasive. Surgery is the primary treatment that is usually done by intralesional curettage. In pelvis and spine surgery may be associated with high rate of complications, recently, Denosumab has been proposed for the treatment of these tumors in latter anatomical regions. Denosumab may be administered alone or as an adjuvant to surgery. This study aimed to assess the treatment effects of Denosumab in patients with unresectable GCTB. This study was a case series. Patients with unresectable GCTB of vertebra and sacrum were enrolled in this study. Patients received 120 mg of monthly Denosumab and additional doses on days 8th and 15th of treatment. Images of patients before and after treatment were evaluated. Nine patients with a median age of 30 years with spine and sacrum GCTB were included in this study. The median time of treatment with denosumab was 28 months (range: 3–67). Tumor control was seen in all patients. According to Inverse Choi density/size (ICDS), criteria objective response (complete response and partial response) was seen in 8 patients, and one had stable disease. Based on CT scan images, in 4 patients (44.44%), less than 50% of the transverse diameter of the tumor became ossified, and in the other five patients (55.55%), more than 50% of the tumor's transverse diameter became ossified. The median tumor volume before treatment was 829 cm3, and after treatment was 504 cm3 which was significantly reduced (P = 0.005). No complication related to therapy was seen. Tumor response was seen in all patients, and tumor control according to ICDS criteria was evident in all cases. This finding was in line with previous studies. Clinical improvement of signs and symptoms was also seen in all patients. Generally, our study demonstrates a sustained clinical benefit and tumor response with Denosumab, as tumor response ≥ 24 weeks was evident in all cases. No side effects were seen in patients despite long-term treatment with Denosumab. [ABSTRACT FROM AUTHOR]

Giant cell tumors (GCTs) are rare, locally aggressive neoplasms that commonly affect the long bones. However, GCTs can also occur in the craniofacial region, including the maxilla. This case report presents a rare case of recurrent giant cell tumor of the maxilla in a 32-year-old male patient. The patient underwent a total maxillectomy with clear margins, which resulted in successful local control. This report highlights the clinical presentation, diagnostic approach, treatment modalities, and favorable prognosis associated with recurrent giant cell tumors of the maxilla. [ABSTRACT FROM AUTHOR]

Tenosynovial giant cell tumors represent a group of typically non-malignant tumors found within the joints and soft tissues. The occurrence of tenosynovial giant cell tumor alongside hematologic malignancies is an infrequent finding. Herein, we report a patient who presented with coinciding Hodgkin Lymphoma (HL) and tenosynovial giant cell tumor before chemotherapy initiation. The case was discovered during initial assessment using [18FF]fluorodeoxyglucose ([18FF]FDG) positron emission tomography/computed tomography (PET/CT) imaging for HL staging. An unrelated hypermetabolic mass within the left knee joint led to the discovery of this unusual case, which led to a CT-guided biopsy and tenosynovial giant cell tumor discovery. This was clearly demonstrated in interim and end-of-therapy PET/CT studies when all lymphomatous lesions had resolved but the tenosynovial giant cell tumor remained. This case serves as a reminder of the intricate nature of oncological pathology and emphasizes the need for thorough and vigilant diagnostic evaluation for optimal management plan. [ABSTRACT FROM AUTHOR]

Key Clinical Message Giant cell tumor of bone (GCT) is a rare neoplasm which often presents as a lytic lesion in the epiphyseal region of long bones and which are usually accompanied by pain, swelling, and restricted movement. Abstract Giant cell tumor of bone (GCT) is a rare neoplasm that affects individuals in their third and fourth decades of life. Clinically, it often presents as a lytic lesion in the epiphyseal region of bones, notably the distal femur and proximal tibia. Radiologically, GCT appears as a distinct lytic lesion in the epiphyseal region. Histopathologically, GCTs are composed of mononuclear cells, macrophages, and multinuclear giant cells, indicative of osteoclastogenic stromal tumors. A 37‐year‐old man presented with left wrist pain, swelling, and restricted movement persisting for a year, worsening over the last 7 months. Radiographic assessments revealed a distal radius bone mass involving the radiocarpal joint. Biopsy confirmed a GCT with extension into peripheral muscle. PET/CT scan showed localized pathology without metastasis. Histopathologically, GCT exhibited multinucleated giant cells, spindle cells, and aneurysmal bone cyst‐like regions with coagulation necrosis. Surgical resection involved en‐bloc removal and reconstruction with a non‐vascularized radius bone graft. Postoperatively, the patient showed no complications at the one‐year follow‐up, suggesting successful intervention.

We present the case of a 9-year-old girl who developed striking bone changes following two years of denosumab therapy for giant cell lesions of the jaw. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCT) is a benign tumor of bone that is known to be locally aggressive rarely metastasizing to distant sites, most commonly to the lungs. The reported pulmonary metastasis incidence is 1 - 9%. We report a case of GCT with solitary pulmonary metastasis who had significant clinical benefit and disease control with sequential application of surgical resection of pulmonary metastasis, local external beam radiation therapy (EBRT), and systemic Denosumab. We wish to highlight that even in metastatic GCT, there is significant clinical benefit in aggressive treatment. [ABSTRACT FROM AUTHOR]

ObjectivesTo explore the predictive value of preoperative imaging in patients with spinal giant cell tumor of bone (GCTB) for postoperative recurrence and risk stratification.MethodsClinical data for 62 cases of spinal GCTB diagnosed and treated at our hospital from 2008 to 2018 were identified. All patients were followed up for more than 2 years according to the clinical guidelines after surgery. Medical history data including baseline demographic and clinical characteristics, computed tomography (CT) and magnetic resonance imaging (MRI) findings of recurrent and non-recurrent patients were compared. Two musculoskeletal radiologists read the images and were blinded to the clinical data. The imaging features associated with postoperative recurrence were analyzed by multivariate logistic regression, and receiver operating characteristic (ROC) curve analysis was performed to determine the optimal cutoff value of the largest lesion diameter predicting recurrence after surgery.ResultsAccording to whether the disease recurred within the follow-up period, patients were divided into the recurrence group and the non-recurrence group. Of 62 patients (29 males and 33 females), 17 had recurrence and 45 did not. The recurrence rate was 27.4%. The mean follow-up time was 73.66 (± 32.92) months. The three major treatments were total en bloc spondylectomy (n = 26), intralesional spondylectomy (n = 20), and curettage(n = 16). A total of 16 CT and MRI features were analyzed. A univariate analysis showed no significant difference in age, sex, treatment, multi-vertebral body involvement, location, boundary, expansile mass, residual bone crest, paravertebral soft tissue mass, CT value, and MRI signal on T1-weighted imaging (WI), T2-WI, and T2-WI fat suppression (FS) sequences (P > 0.05). The largest lesion diameter [(4.68 ± 1.79) vs (5.92 ± 2.17) cm, t = 2.287, P = 0.026] and the vertebral compression fracture (51% vs 82%, χ2  = 5.005, P = 0.025) were significantly different between the non-recurrence and recurrence groups. Logistic regression analysis showed that both largest lesion diameter (odds ratio [OR], 1.584; 95% confidence interval [CI], 1.108-2.264; P = 0.012) and compression fracture (OR, 8.073; 95%CI, 1.481-11.003; P = 0.016) were independent predictors of postoperative recurrence. When we set the cutoff value for the largest lesion diameter at 4.2 cm, the sensitivity and specificity for distinguishing the recurrence and non-recurrence of GCTB were 94.1% and 42.2%, respectively, and the area under the curve (AUC) was 0.671. The combined model achieved a sensitivity, specificity and accuracy of 47.1%, 97.8% and 83.9%, respectively.ConclusionsIn spinal GCTB, maximum lesion diameter and the vertebral compression fracture are associated with tumor recurrence after surgery, which may provide helpful information for planning personalized treatment.

Introduction: Giant cell tumours of the soft tissue (GCT-ST) are usually found in the superficial and deep soft tissues of the extremities but have been described in the pancreas, lung, thyroid gland, urothelial tract, skin, larynx, heart and very rarely, in the breast. At present, according to the World Health Organization's classification of soft tissue tumors, GCT-ST is categorized as an intermediate grade (rarely metastasizing) fibrohistiocytic tumour. GCT of the breast is extremely rare and to date, only eleven cases have been reported. We report a case of GCT of the breast, which was clinically suspected as a malignant tumor and discuss the different treatment modalities with the importance of close follow-up of the same after a thorough review of the literature. Case Presentation: We report a case of a 45-year-old woman who noticed a tender lump in her left breast. A malignant tumour was suspected on clinical examination and imaging. Histological evaluation revealed a tumour composed of a mixture of round and oval mononuclear cells with minimal atypia and uniformly distributed multinucleated osteoclast-like giant cells (OGCs) with a stroma rich in blood vessels. IHC was done in which the OGCs stained positively for CD68 and CD45, mononuclear stromal cells were positive for vimentin whereas the tumour was negative for breast markers Progesterone Receptor (PR), Estrogen Receptor (ER), GATA 3, epithelial marker EMA, S-100 and Desmin; hence the definitive diagnosis of GCT of the breast was made. Discussion: GCT of the breast, due to its rareness and the malignant-mimicking clinical presentation, causes difficulty in diagnosis. Other giant cell-rich lesions including breast cancer with OGCs, pleomorphic leiomyosarcoma, osteosarcoma, undifferentiated pleomorphic sarcoma and metastatic GCT-B are to be considered in the differential diagnosis. Conclusion: GCT of the breast is an extremely rare tumour and pretends a breast malignant tumours. For the correct diagnosis of this rare tumour, combining the results of histological and immunohistochemical analyses helps in ruling out differential diagnosis.

Thanapon Chobpenthai,1 Thanate Poosiripinyo,2 Chawin Warakul1 1Princess Srisavangavadhana College of Medicine, Chulabhorn Royal Academy, Bangkok, Thailand; 2Department of Orthopaedics, Khon Kaen Hospital, Khon Kaen, ThailandCorrespondence: Thanapon Chobpenthai, Princess Srisavangavadhana College of Medicine, Chulabhorn Royal Academy, 906 Kamphaengphet 6 Road, Talat Bang Khen, Lak Si, Bangkok, 10210, Thailand, Tel +662-576-6000, Fax +662-576-6904, Email thanapon.cho@cra.ac.thAbstract: The distal radius is rarely affected by either primary or metastatic bone cancers. The most frequent tumors of the distal radius are giant cell tumors, which are benign tumors with the propensity to invade. En bloc excision of giant cell tumors of the distal radius achieves a low recurrence rate but compromises the wrist joint, necessitates a significant reconstruction, and has functional consequences. Reconstruction after en bloc resection of a distal radius bone tumor is challenging. Furthermore, orthopedic oncologists disagree on treating such long bone anomalies most effectively. The present article summarizes the various biological and non-biological reconstruction techniques performed after en bloc resection of a distal radius tumor, discusses the advantages and disadvantages of each reconstruction strategy, and summarizes several case studies and case reports.Keywords: benign bone tumor, giant cell tumor, en bloc resection, reconstruction technique, distal radius tumor

Giant cell tumor of the soft tissue (GCTST) is a neoplasm with low malignant potential and typically affects the trunk and extremities. Herein, we present a case of a palpable right neck mass diagnosed as a GCTST of the carotid sheath in a 38-year-old woman. A review of the imaging characteristics as well as of the differential diagnoses of primary neoplasms of the carotid space is presented.