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Objective: To present pathologic, clinical, and treatment findings for giant cell tumors (GCTs) of sphenoid bone and clivus., Methods: We describe the optimal treatment algorithm in patients with a histopathologic diagnosis of bone GCT by presenting the effects of denosumab treatment in both pediatric and adult patients with GCT undergoing endoscopic transnasal surgery. Clinicopathologic correlation is crucial for the differential diagnosis of GCT and the choice of treatment modality., Conclusion: GCT of bone is a local aggressive tumor that accounts for about 3%-7% of all bone tumors. GCTs located in the cranium are extremely uncommon neoplasms. There are no defined guidelines for the treatment of GCTs in skull base. Following surgical resection of the tumor, the addition of denosumab treatments to radiotherapy has a significant role in preventing the recurrence of GCT and in promoting regression of residual tumor size.

Background: Bone tumors can cause severe pain and poor quality of life due to recurrence and non-achievement of complete remission after surgery, chemotherapy, or radiotherapy. Radiofrequency ablation (RFA) can be considered for minimally invasive treatment of bone tumors that are difficult to radically excise. In this study, RFA was performed for bone tumors that were difficult to radically excise and did not respond to surgery, chemotherapy, or radiotherapy due to their large sizes and/or locations. The purpose of this study was to retrospectively analyze the clinical characteristics and survival rates of bone tumors after RFA and provide one more treatment option for the future., Methods: There were 43 patients with bone tumors who underwent percutaneous RFA at our hospital from April 2007 to October 2017. The median age of the patients was 59 years (range, 31-75 years), and the median follow-up duration was 67.2 months (range, 10.2-130.5 months). Of the 43 patients, 26 were male and 17 were female. Thirty-four cases were metastatic bone tumors, 5 were chordomas, 3 were osteosarcomas, and 1 was a giant cell tumor. Pain and functional ability of the patients were evaluated using a visual analog scale (VAS) and the Musculoskeletal Tumor Society (MSTS) functional scoring system, respectively. Scores were recorded preoperatively, 1 week postoperatively, and 4 weeks postoperatively. The 1-year, 2-year, and 5-year survival rates were evaluated using the Kaplan-Meier method., Results: The mean VAS score was 8.21 preoperatively. The mean VAS score at 1 week, 4 weeks, 12 weeks, and 24 weeks postoperatively were 3.91, 3.67, 3.31, and 3.12, respectively. The mean preoperative MSTS score was 64.0% (range, 32%-87%). The mean postoperative MSTS score was 71.0% (range, 40%-90%). The 1-year, 2-year, and 5-year survival rates were 95.3%, 69.8%, and 30.2%, respectively., Conclusions: As per our study findings, RFA was effective in reducing pain and improving functional ability of patients with bone tumors that were difficult to radically excise., Competing Interests: CONFLICT OF INTEREST: No potential conflict of interest relevant to this article was reported., (Copyright © 2021 by The Korean Orthopaedic Association.)

A 21-year-old man consulted our hospital for treatment of a spinal giant cell tumor (GCT) of Enneking stage III. Lower lumbar-spine tumors and severe spinal canal stenosis are associated with high risk for surgical mor-bidity. Stability was temporarily secured with a percutaneous pedicle screw fixation in combination with deno-sumab, which shrank the tumor. Total en bloc spondylectomy was then performed 6 months after initiation of denosumab, and the patient was followed for 3 years. There was no local recurrence, and bony fusion was obtained. Minimally invasive surgery and denosumab allowed safer and easier treatment of a collapsing lower lumbar extra-compartmental GCT., Competing Interests: No potential conflict of interest relevant to this article was reported.

Background: Denosumab is a human monoclonal antibody that has been used successfully in the treatment of giant cell tumors of bone. These tumors are rare and, in principle, benign, but they are highly aggressive, locally advanced, osteolytic bone tumors that can metastasize to the lungs. Denosumab is an effective treatment when these tumors cannot be surgically removed or when surgical resection is likely to lead to severe morbidity (eg, loss of limbs or joints). The aim of this systematic review and meta-analysis was to investigate patients with giant cell tumors of bone who experienced tumor progression during treatment with denosumab and to compare them with patients who experienced reduction of their giant cell tumors of bone during treatment with denosumab., Methods: Embase, Cochrane Library, and MEDLINE/PubMed databases were searched for trials submitted by January 7, 2020, that reported the efficacy and safety of denosumab in patients with giant cell tumors of bone., Results: Sixty studies were reviewed, involving a total of 1074 patients who had giant cell tumors of bone and were treated with denosumab. Of the 60 studies, 58% of the patients were from case series studies, 39% from open-label phase II studies, and 3% from case reports. The response rate for denosumab as a treatment for giant cell tumors of bone was 97.5%, with statistical significance ( P < .0001). Pain in the limbs was statistically the most common adverse event for denosumab treatment in case series studies ( P < .0001). No treatment-related deaths occurred in the reviewed studies., Conclusion: Cumulative evidence supports the addition of surgery to optimal medical therapy with denosumab to reduce tumor size, clinical symptoms, and mortality among patients with giant cell tumors of bone.

Next-generation sequencing has revealed mutations in several bone-related lesions and was recently used to uncover the genetic basis of giant cell lesions of the jaws (GCLJ). Consistent with their benign nature, GCLJ show a low tumor mutation burden. They also harbor somatic, heterozygous, mutually exclusive mutations in TRPV4, KRAS, or FGFR1. These signature mutations occur only in a subset of lesional cells, suggesting the existence of a 'landscaping effect', with mutant cells inducing abnormal accumulation of non-mutant cells that form the tumor mass. Osteoclast-rich lesions with histological similarities to GCLJ can occur in the jaws sporadically or in association with genetically inherited syndromes. Based on recent results, the pathogenesis of a subgroup of sporadic GCLJ seems closely related to non-ossifying fibroma of long bones, with both lesions sharing MAPK pathway-activating mutations. In this review, we extrapolate from these recent findings to contextualize GCLJ genetics and we highlight the therapeutic implications of this new information. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd., (© 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.)

Giant cell tumor of the tendon sheath (GCTTS) is a benign tumor with a high recurrence rate of up to 50%. The lesion may appear anywhere in the synovium; the nature of this tumor is still controversial, however recent data shows that tenosynovial giant cell tumors (TGCTs) could be clonal neoplastic tumors. Most lesions of GCTTS produce one or more discrete nodules, while the radiological features can include soft-tissue masses with or without bone destruction. In an effort to advance in the understanding of GCTTS pathogenesis, we decided broadly investigate the immunophenotypic profile, of a TGCT in a 69-year-old female patient.

Cryotherapy, also called Cryoablation (CA), is a technique that provides a local treatment to various pathological conditions. In Musculoskeletal tumours management, Cryoablation is well accepted and validated as a treatment in palliative cures for metastatic patients. Recently, CA has been proposed also as an alternative to radiofrequency ablation in osteoid osteoma and other benign tumour treatment with promising results. Cryotherapy with argon ice-balls as local adjuvant in open surgery is a tool that can provide enlargement of surgical margins if used properly. There is still not enough evidence supporting use of cryotherapy as local adjuvant in Musculoskeletal open surgery as the series cited above are very small and there is no comparative RCT between local adjuvant therapies including CA. One-hundred-and-eighty-three patients were treated with Cryoablation from 2000 and 2018 in the Musculoskeletal Tumours Surgery Unit of Careggi (Florence) and the University 2nd Clinic of Pisa. In our study group, 38 patients (26.6%) were affected by bone metastasis, 16 patients (11.1%) by aneurismal bone cysts or angiomas, 22 patients (15.4%) by low-grade malignant musculoskeletal tumours, 2 patients (1,4%) by fibromatosis, 63 patients (44.1%) by benign musculoskeletal tumours (principally Giant Cell Tumours-GCT) and 2 patients (1.4%) by Osteosarcomas. In 125 cases (87.4%), CA has been used as an adjuvant therapy, in 12 cases (8.4%) as a percutaneous ablation therapy and in 6 cases (4.2%) as adjuvant to remove tumoral lesions 'en bloc' or as a 'poor technique' for its sterilizing effect on previously resected bones. Mean follow-up was 10 years. Twenty-three patients (16%) were classified as Alive with Disease (AWD) due to local recurrence or tumour progression (14 metastases, 5 low-grade malignant bone tumours, 4 Giant Cell Tumours). Eight patients died due to the disease (6 metastases, 2 osteosarcomas), while 1 died from leukaemia. One-hundred-and-eleven patients (78%) were classified as Continues Disease Free (CDF). All patients reported decrease in pain-related symptoms after surgery and all surgeons reported better control of blood loss. Three cases (2%) of local skin necrosis or wound dehiscence were reported. No local recurrences were reported after fibromatosis ablation. Our results confirm that CA could be considered as a safe and effective technique to treat various conditions as adjuvant and palliative therapy. In particular, in open surgery, cryotherapy as an adjuvant treatment could lead to very low rates of recurrence in locally aggressive tumours like Giant Cell Tumours. These results could be generalized but a better understanding about indications and outcomes can be reached studying CA in specific populations with comparation to other adjuvant techniques.

Objectives: Although surviving bone resection/limb salvage surgery treatment is the beginning of the journey towards recovery, the importance of providing patients with post-operative psychological support is often overlooked by health professionals. Hence, patients typically are left to their own devices in terms of ensuring their mental health and well-being. Methodological Approach and Participants: This qualitative analysis of seven long-term sarcoma survivors' reflective journal entries provides insights into the different phases of distress, resilience building, resilient growth and advice-giving that they moved through during their survivorship journey., Interpretation: Our findings identify the fragility of patient resilience and highlight areas for future research.

Rationale: Both serial arterial embolization (SAE) and denosumab have been proved to be effective in treatment for giant cell tumor (GCT). There is potential synergic effect of combining two methods. The purpose of current study is to justify a new treatment strategy of combination of SAE and denosumab as neoadjuvant or stand-alone treatment for large sacropelvic giant cell tumor., Patient Concerns: Pelvic and sacral GCTs tend to be very large size and vascular. The concerns of surgical treatment were invasiveness of extensive surgery, intraoperative hemorrhage, nerve function jeopardized and local recurrence. However, SAE alone may not be adequate for complete removal of the tumor., Diagnoses: All the three cases were proved to be GCT by core-needle biopsy. Post-treatment pathological change was confirmed by further biopsy., Interventions: The patient in Case 1 diagnosed of large recurrent sacral GCT received 6 times of endovascular embolizations with 2-month interval and started on denosumab simultaneously after first session of embolization. The second case was a 22-year-old female presented with a massive iliosacral tumor. SAE was performed for 3 sessions and the denosumab was started simultaneously. The patients was on treatment for half year. Both patients experienced a dramatic decrease in symptoms and concomitant improvement in function after the first embolization and weekly injection of denosumab. Tumor removal was performed on patient in case 2. The last case was a pelvic GCT and the patient received SAE and denosumab for half year. The tumor was then removed with purpose of complete cure., Outcomes: The first patient was still on denosumab with stable tumor. The other two patients were both free of recurrence after surgical removal of the tumors. No denosumab was used postoperatively., Lessons: We reported the first three cases treated by combination of SAE and denosumab in the literature and aim to raise an alternative method for large GCT at challenging anatomical locations, for which surgery would carry significant risk. SAE and denosumab can synergically promote sclerosis and result in significant decrease in pain. It is reasonable to consider using SAE combined with denosumab neoadjuvantly to reduce the extensiveness and morbidity of surgery, however further investigation is warranted.

A 34-year-old man with a 1-week history of diplopia was referred to the authors' hospital. Neurological examination revealed left abducens nerve palsy. Computed tomography showed a lesion in the left sphenoid sinus involving the medial wall of the left internal carotid artery (ICA) and osteolytic change at the clivus bordering the lesion. Magnetic resonance imaging demonstrated an extensive soft-tissue mass occupying the left sphenoid sinus. Surgical intervention by the endoscopic transnasal method allowed most of the lesion to be removed. Only the portion attached to the medial wall of the ICA was not removed. Postoperatively, the lesion was diagnosed as a giant cell tumor (GCT) and the patient received 120 mg of subcutaneous denosumab every 4 weeks, with additional doses on Days 8 and 15 during the first month of therapy. MRI a week after starting denosumab revealed shrinkage of the initially fast-growing residual tumor. The patient was discharged upon completion of the third denosumab administration. GCT is an aggressive stromal tumor developing mainly in young adults. Complete resection is recommended for GCT in the literature. However, size and location of the CGT often limit this approach. Various adjuvant treatments for skull base GCTs have been reported, including radiation and chemotherapy. However, the roles of adjuvant therapies have yet to be clearly defined. Denosumab, a monoclonal antibody, was recently approved for GCT in several countries. Denosumab may permit less invasive treatments for patients with GCTs while avoiding deleterious outcomes, and may also limit disease progression and recurrence.

Background: Giant cell tumor of the spine has high rate of postoperative recurrence. There are not much published studies on repeatedly recurrent GCTS (RRGCTS). Also, there are controversies as to the prognostic factors and treatment options for RRGCTS., Methods and Materials: A retrospective survival analysis between 2000 and September 2014 was performed on the 74 times of in-hospital treatment of the 21 patients. Recurrence-free survival was defined as the time between the date of surgery and the date of recurrence. Factors with P values <0.05 in univariate analysis were subjected to multivariate analysis by means of proportional hazard analysis., Result: A total of 21 patients comprising ten males and 11 females with a mean age of 29.7 years (range 15-49) were included, with a total of 74 times of surgery performed. Eighteen patients had no evidence of disease. Univariate and multivariate analysis suggested that total spondylectomy and bisphosphonate therapy were independent prognostic factors for better RFS in RRGCTS patients., Conclusion: Patients of RRGCTS are suitable for further surgical treatment with relatively good healing process and restoration of nervous function. Early diagnosis of recurrence may be associated with better prognosis. Total spondylectomy in combination with bisphosphonate therapy could reduce postoperative recurrence rate.

Background: Isolated limb perfusion (ILP) is an established and effective treatment for advanced melanoma and soft tissue sarcomas of the extremities with a high overall response rate. The aim of this study was to describe our experience of ILP for more rare types of tumours., Methods: Patients with Merkel cell carcinoma (MCC) (n = 4), squamous cell carcinoma (SCC) (n = 2), B-cell lymphoma (n = 1), desmoid tumours (n = 3), pigmented villonodular synovitis (PVNS) (n = 1) and giant cell tumour (n = 1) were treated with ILP and analysed retrospectively., Results: The four patients with in-transit MCC had three complete responses (CR) and one partial response (PR); the two patients with SCC had one CR and one stable disease (SD); the patients with desmoid tumours had two PR and one SD. A CR was also observed for the patient with a giant cell tumour, but the patient with PVNS had a SD. The patient with cutaneous metastases of B-cell lymphoma showed a CR, however with rapid systemic progression. Local toxicity according to Wieberdink was grade II in 10 patients (83%) and grade III in two patients (17%)., Conclusions: These results show that ILP can be used as a treatment option also for more rare disease entities when other treatments have failed.

Giant cell tumors of the bone are benign but locally aggressive, and they rarely metastasize to the lungs. The purpose of this study was to retrospectively review the clinical presentation, long-term outcomes, and treatment of pulmonary metastasis of these tumors. Between 1991 and 2004, a total of 168 patients with giant cell tumors of the bone were treated at the authors' institution, 7 of whom developed lung metastasis. Four of the 7 patients were men, and mean age of these patients at initial surgery was 40 years (range, 19-56 years). All patients underwent wide excision and reconstruction or curettage and bone grafting for the bony lesions. Lung metastases were detected at a mean of 44 months after the treatment of bone lesions. Five patients had multiple metastases, and 2 had solitary pulmonary metastases. Six of these patients underwent delayed treatment, locally aggressive, or multiple recurrent and surgical procedures. All of the aforementioned procedures had similar risk factors to those previously reported in the literature. One patient had multiple giant cell tumors of the bone. At last follow-up, 2 patients had died due to complications from the pulmonary metastases or chemotherapy. One patient underwent a metastasectomy 4 years after treatment due to the progression of pulmonary metastasis. The remaining 4 patients were alive and healthy after chemotherapy or conservative treatment. Therefore, early detection, adequate treatment of the primary bone lesion, conservative treatment of lung metastases, and regular long-term follow-up are recommended., (Copyright 2016, SLACK Incorporated.)

Giant cell tumor of the larynx (GCTL) is a rare entity; only 34 cases have been reported in the literature. We report a case of GCTL in a 46 year-old male presenting clinical, radiographic, histological and therapeutic features. Previously reported cases are also reviewed.

Resently, we treated a 69-year-old patient with squamous cell carcinoma of lung with osteoclast-like giant cells which were similar with the giant cell tumor of bone. The clinical and pathological characteristics of this case were presented, and the literatures were reviewed. The tumor differed histologically from the pleomorphic carcinoma, which occurs most commonly in the lung and showed diverse pleomorphic manifestation with benign looking osteoclast-like multinucleated cells and bizarre giant cells. In addition, undifferentiated carcinoma with a sarcoma-like appearance containing small areas of papillary adenocarcinoma was evident in the tumor. Histological features and immunohistochemical staining could be helpful in differential diagnosis.

Benign lesions comprise a majority of soft tissue tumors. It has been estimated that their incidence outnumbers that of malignant tumors by a factor of at least 100 [1]. While history and physical examination can start the diagnostic process, imaging including the use of magnetic resonance imaging can be more helpful. Biopsy of these tumors is sometimes necessary and can be performed in a number of ways, often in conjunction with definitive treatment. Specific diagnostic and treatment strategies for a number of the more commonly encountered benign soft tissue tumors including lipomas, pigmented villonodular synovitis and hemangiomas are reviewed. An algorithm for the management of benign soft tissue tumors is discussed.

Giant cell tumors (GCTs) are generally benign, locally aggressive lesions mostly located in the metaphysis of long bones. GCTs of the skull are rare and the majority of the cases have been presented as case reports. The authors retrospectively reported 18 patients with GCTs of the skull at a single institution from April 1994 to February 2012 and summarized the clinical, radiological, pathological characteristics and management of the disease. Meanwhile, a systematic review of 94 case reports of GCTs of the skull was performed. Headache and symptoms related to the involvement of intracranial nerves were the most common symptoms. Over 90 % of the tumors originated from sphenoid and temporal bones. On MRI, very low signal on T2-weighted images were found highly indicative of GCTs of the temporal bone. Univariate analysis revealed that extent of tumor resection and post-operative radiation therapy (RT) were prognostic factors significantly influencing the survival of the patients. We concluded that complete tumor resection is the optimal goal in treating this disease and adjuvant RT should be given once tumor residual is inevitable.

Giant cell tumors deriving from synovium are classified into a localized (GCT of tendon sheath; GCT-TS) and diffuse form (diffuse-type GCT, Dt-GCT). We propose a multidisciplinary management based upon a systematic review and authors' opinion. Open excision for GCT-TS and open synovectomy (plus excision) for Dt-GCT is advised to reduce the relatively high recurrence risk. External beam radiotherapy should be considered in severe cases, as Dt-GCT commonly extends extra-articular., (Copyright © 2012 Wiley Periodicals, Inc.)

Pigmented villonodular synovitis (PVNS) is a rare benign disease of the synovium of joints and tendon sheaths, which may be locally aggressive. We present 18 patients with diffuse-type PVNS of the foot and ankle followed for a mean of 5.1 years (2 to 11.8). There were seven men and 11 women, with a mean age of 42 years (18 to 73). A total of 13 patients underwent open or arthroscopic synovectomy, without post-operative radiotherapy. One had surgery at the referring unit before presentation with residual tibiotalar PVNS. The four patients who were managed non-operatively remain symptomatically controlled and under clinical and radiological surveillance. At final follow-up the mean Musculoskeletal Tumour Society score was 93.8% (95% confidence interval (CI) 85 to 100), the mean Toronto Extremity Salvage Score was 92 (95% CI 82 to 100) and the mean American Academy of Orthopaedic Surgeons foot and ankle score was 89 (95% CI 79 to 100). The lesion in the patient with residual PVNS resolved radiologically without further intervention six years after surgery. Targeted synovectomy without adjuvant radiotherapy can result in excellent outcomes, without recurrence. Asymptomatic patients can be successfully managed non-operatively. This is the first series to report clinical outcome scores for patients with diffuse-type PVNS of the foot and ankle.

Giant cell tumours (GCT) of the synovium and tendon sheath can be classified into two forms: localised (giant cell tumour of the tendon sheath, or nodular tenosynovitis) and diffuse (diffuse-type giant cell tumour or pigmented villonodular synovitis). The former principally affects the small joints. It presents as a solitary slow-growing tumour with a characteristic appearance on MRI and is treated by surgical excision. There is a significant risk of multiple recurrences with aggressive diffuse disease. A multidisciplinary approach with dedicated MRI, histological assessment and planned surgery with either adjuvant radiotherapy or systemic targeted therapy is required to improve outcomes in recurrent and refractory diffuse-type GCT. Although arthroscopic synovectomy through several portals has been advocated as an alternative to arthrotomy, there is a significant risk of inadequate excision and recurrence, particularly in the posterior compartment of the knee. For local disease partial arthroscopic synovectomy may be sufficient, at the risk of recurrence. For both local and diffuse intra-articular disease open surgery is advised for recurrent disease. Marginal excision with focal disease will suffice, not dissimilar to the treatment of GCT of tendon sheath. For recurrent and extra-articular soft-tissue disease adjuvant therapy, including intra-articular radioactive colloid or moderate-dose external beam radiotherapy, should be considered.

Background: Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by osteomalacia, which occurs as a result of excess renal phosphate excretion caused by fibroblast growth factor-23 secreted by mesenchymal tumors. This entity is rare in head and neck cancers. We report a rare case of oncogenic osteomalacia in a patient with an anterior skull base giant cell tumor., Methods and Results: A 34-year-old woman presented with a 5-year history of progressive weakness in both lower limbs and the trunk. Hypophosphatemia and hypocalcemia had been noted by a local physician, but her symptoms persisted despite receiving calcium and vitamin D supplements. A recent onset of epistaxis and nasal blockage led to referral to the head and neck services. Nasal endoscopy revealed a left nasal cavity mass. Further evaluation with imaging studies revealed a mass in the nasal cavity with intracranial extension. Biopsy of the lesion suggested a neurogenic tumor. A putative diagnosis of anterior skull base neurogenic tumor with paraneoplastic hypophosphatemia was made. After the biochemical parameters were corrected, the patient underwent craniofacial resection. The final histopathologic study suggested the lesion as a "giant cell tumor." During the postoperative period the patient's biochemical and clinical symptoms improved dramatically, allowing her to regain normal mobility., Conclusions: Clinicians and pathologists must be aware of the clinical symptoms, laboratory abnormalities, and pathologic features of oncogenic osteomalacia, which may be caused by tumors in the head and neck and thus make an exhaustive effort to diagnose the same., (Copyright © 2010 Wiley Periodicals, Inc.)

Purpose of Review: To review recent developments in the molecular pathogenesis of tenosynovial giant cell tumor (TGCT) or pigmented villonodular synovitis (PVNS) and its therapeutic implications., Recent Findings: TGCT or PVNS is a benign clonal neoplastic proliferation arising from the synovium characterized by a minor population of intratumoral cells that harbor a recurrent translocation. These cells overexpress CSF1, resulting in recruitment of CSF1R-bearing macrophages that are polyclonal and make up the bulk of the tumor. Inhibition of CSF1R using small molecule inhibitors such as imatinib, nilotinib or sunitinib can result in clinical, radiological and functional improvement in the affected joint., Summary: Currently, surgery remains the treatment of choice for patients with TGCT/PVNS. Localized TGCT/PVNS is managed by marginal excision. Recurrences occur in 8-20% of patients and are easily managed by re-excision. Diffuse TGCT/PVNS tends to recur more often (33-50%) and has a much more aggressive clinical course. Patients are often symptomatic and require multiple surgical procedures during their lifetime. For patients with unresectable disease or multiple recurrences, systemic therapy using CSF1R inhibitors may help delay or avoid surgical procedures and improve functional outcomes.

Purpose: To describe a case of bilateral eyelid-confined giant cell angiofibroma (GCAF) in a patient with a slowly progressive bilateral eyelid swelling., Methods: A 40-year-old man with a 5-year history of slowly progressive bilateral eyelid swelling, severe functional impairment, and bilateral cosmetic deformity was studied. An extensive ophthalmologic evaluation, laboratory examinations, and orbital magnetic resonance imaging were carried out., Results: Clinical examination showed nonpitting lymphedema affecting both upper and lower eyelids, with orange peel skin. Orbital magnetic resonance imaging revealed diffuse thickening of the preseptal structures in the eyelids without extension to the orbit. Histologic specimen revealed the presence of spindle and multinucleated giant cells surrounding pseudovascular spaces strongly positive to CD34 and vimentin. A diagnosis of GCAF was made and radiation therapy was performed 3 weeks after surgical debulking with partial recovery of visual and anatomic function., Conclusions: Giant cell angiofibroma involving the eyelid is rare and can represent a diagnostic and therapeutic challenge to the ophthalmologist.

Recurrent giant cell tumors refractory to various treatment modalities are challenging dilemmas for the most experienced practitioner. We report a case of a multiply recurrent aggressive giant cell tumor diagnosed at 10 months of age with extensive involvement of the maxillae and mandibles unresponsive to multiple therapeutic modalities. The various treatments attempted in this patient including conventional therapies as well as the original use of bevacizumab (Avastin) are described.

Giant cell tumour (GCT) of bone is an uncommon primary bone neoplasm typically occurring at the epiphyses of long bones in young adults. They are osteolytic neoplasms with approximate local recurrence rates of 25%, and 2% of patients develop pulmonary metastases. These tumours appear very rarely in the skull, with those few reported cases arising predominantly in the sphenoid and occasionally the temporal bones. They demonstrate benign histological features, but are locally aggressive and surgical excision is the treatment of choice. It is widely believed that giant cell tumours should be distinguished from other giant cell lesions, importantly central giant cell reparative granulomata (CGCG) which are thought to have a lower recurrence rate and for which no cases of malignant transformation or metastases have been reported. Investigators have noted that giant cell lesions in the skull bones may be unique and that GCT and CGCG may be part of a spectrum of a single disease process. We present a case of a giant cell tumour of the temporal bone which illustrates and re-emphasises this concept and review the literature on these lesions.

Opinion Statement: Primary spinal neoplasms are rare tumors that can lead to significant morbidity secondary to local bone destruction and invasion into adjacent neurological and vascular structures. These tumors represent a clinical challenge to even the most experienced physicians and require a multidisciplinary approach to ensure optimal patient outcomes. This review will discuss the most common primary bone tumors and focus on recent surgical, medical, and radiation treatment advances.

Pre-operative endovascular embolization of spinal giant cell tumors (GCTs) has been an effective strategy to reduce blood loss during surgical resection. Traditionally, spinal GCTs have been embolized with polyvinyl acetate (PVA) particles. We present the pre-operative embolization of a recurrent cervical GCT with N-butyl 2-cyanoacrylate (NBCA) rather than PVA. The patient was a 17-year-old female who, 3 months prior, had undergone a surgical resection of a cervical GCT without pre-operative embolization. She returned with tumor recurrence in the approximate location. Resection was recommended, and pre-operative embolization was requested. The tumor was embolized with NBCA. Post-embolization angiography demonstrated significantly decreased tumor "blush" and a significant reduction of the vascular supply. This is the first reported use of NBCA for the pre-operative embolization of a cervical GCT. The benefits of NBCA over PVA particles include superior penetration, permanent tumor embolization and lower exposure to radiation due to shorter procedure time.

Transarterial embolization should be considered in the treatment algorithm of primary or secondary bone tumors. Specific benefit is present where there is a high risk of bleeding at surgery, where there is spinal involvement and neural encroachment, where active bleeding is present, or in awkward surgical locations where prolonged surgery is anticipated.

Background: Malignant diffuse-type tenosynovial giant cell tumor (D-TSGCT), an unusual sarcoma with concurrent or previous benign D-TSGCTs, poses challenges to diagnosis and prognostication., Methods: We described the radiologic, clinicopathologic, and immunophenotypical findings of 5 primary and 2 metachronous malignant D-TSGCTs and reviewed published cases to better delineate their morphologic spectrum and behavior. Twenty-four benign D-TSGCTs were also statically compared to analyze the diagnostic values of various variables., Results: The 7 malignant cases affected 4 females and 3 males aged 45 to 78 (mean, 60.9) years, which included 1 intraarticular and 6 extra-articular lesions. These tumors were 5 to 17 cm (mean, 9.4) and located within or near the large joints of extremities. Magnetic resonance imaging revealed expansile or infiltrative masses with frequent lobulation and heterogeneous signals. Histologically, areas of benign D-TSGCTs blended abruptly or gradually with frank sarcomas composed of pleomorphic, spindle, or enlarged oval cells, forming malignant fibrous histiocytomalike (n = 4), fibrosarcomatous (n = 1), myxosarcomatous (n = 1), or giant cell tumorlike (n = 1) patterns. One patient experienced recurrences twice, and another 3 developed metastases to the lymph nodes (n = 2), lung (n = 1), or vertebrae (n = 1), with 1 dying from disseminated diseases. An older age (P = 0.003), a larger size (P = 0.036), tumor necrosis (P < 0.001), atypical mitoses (P < 0.001), and Ki-67 overexpression (P < 0.001) appeared preferentially in malignant lesions, but these parameters had overlap between few benign and malignant tumors., Conclusions: Malignant D-TSGCTs are a distinct sarcoma with considerable morphologic variability, metastatic propensity, and lethality. Altered architecture with anaplastic cells represents an important distinguishing feature, while abnormalities of other parameters should not be directly equated with malignancy.

Giant cell tumors are benign tumors commonly found in the long bones. Rarely, they may occur in the larynx, and patients with such tumors may present with hoarseness and anterior neck swelling. Since Wessely reported the first case of laryngeal giant cell tumor in 1940, 30 cases have been identified. Herein, we present a case of a 31-year-old man with giant cell tumor of the larynx successfully treated via the hemilaryngectomy approach.

Study Design: Level III retrospective case series with historical controls., Objective: To evaluate the mid- to long-term outcomes of serial arterial embolization as a primary treatment modality for large sacral giant-cell tumors (SGCT)., Summary of Background Data: Giant-cell tumors are potentially aggressive benign tumors that can cause significant morbidity and may occasionally prove lethal. Large GCTs in the sacrum present a significant challenge, and treatment methods, including surgical resection and radiation, are associated with morbid complications and high recurrence rates. This report presents the mid- to long-term follow-up results of our cases of SGCT treated with serial arterial embolization., Methods: Nine consecutive patients with biopsy-proven SGCTs received initial primary treatment with serial arterial embolization between 1984 and 2006. All patients underwent angiography and selective arterial embolization at the time of diagnosis, followed by repeat embolization every 6 weeks until no new vessels were noted, and then at 6 and 18 months following stabilization of the lesion. Patients were closely monitored with MRI and/or CT every 6 months for 5 years and annually thereafter. Functional outcomes were measured using the 1993 Musculoskeletal Tumor Society Rating Scale (MSTS93)., Results: The mean duration of follow-up in this series was 8.96 years (median, 7.8 years; range, 3.8-21.2 years). No progression was noted in 7 of the 9 cases. Two cases experienced tumor progression of less than 1 cm early in the treatment course and continued to remain asymptomatic. Adjuvant radiation therapy provided local control in 1 of these cases, while radiation and chemotherapy failed in the other case with ultimate mortality. All patients demonstrated substantial pain relief. Cross-sectional MSTS93 scores were obtained in the 8 surviving patients at their most recent follow-up visit with a mean score of 29/30., Conclusions: Serial arterial embolization is a useful primary treatment modality for large SGCTs given the favorable long-term results and potential morbidity of alternative treatments.

Osteoclast-like giant cell tumors (OGCT) are rare abdominal tumors, which mainly occur in the pancreas. The neoplasms are composed of two distinct cell populations and frequently show an inhomogenous appearance with cystic structures. However, due to the rarity of these tumors, only very limited clinical data are available. Imaging features and sonographic appearance have hardly been characterized. Here we report on two cases of osteoclast-like giant cell tumors, one located within the pancreas, the other within the liver, in which OGCTs are extremely rare. Both patients were investigated by contrast sonography, which demonstrated a complex, partly cystic and strongly vascularized tumor within the head of the pancreas in the first patient and a large, hypervascularized neoplasm with calcifications within the liver in the second patient. The liver OGCT responded well to a combination of carboplatin, etoposide and paclitaxel. With a combination of surgical resection, radiofrequency ablation and chemotherapy, the patient's survival is currently more than 15 mo, making him the longest survivor with an OGCT of the liver to date.

Nail abnormalities are varied and numerous. They include warts, keratoacanthomas, onychomatricomas, fibrokeratomas, osteochondromas, tumors (ie, glomus, giant cell, Koenen's, and others), and Bowen's disease. Although the gravity of these conditions may vary, prompt diagnosis and treatment is of the utmost importance. This article discusses the most common defects associated with the nail unit and its surrounding tissue, as well as the differential diagnosis and treatment of these conditions.

Purpose: Treatment with curettage, cauterization, and methylmethacrylate of aggressive benign bone lesions is a method now widely accepted in most orthopedic oncology centers. However, one of the controversies regarding this technique is the possible complications that may arise from the use of methylmethacrylate, which has caused some authors to remove it 2 years after the surgery and replace it with bone graft. The objective of this paper is to present a functional and radiographic evaluation of 214 patients presenting with aggressive benign bone lesions treated with curettage, cauterization, and methylmethacrylate from 1974 to 1998, with some of them having 24 years of follow-up. These patients were clinically and radiographically evaluated for the incidence of late osteoarthrosis, range of motion, and pain in the involved joint., Method: This study comprised 214 patients, with an average follow-up duration of 10.6 years (range: 2 to 24 years. All cases involved aggressive benign lesions. The patients were evaluated according to the Musculoskeletal Tumor Society Score (MSTS) functional evaluation system, and the complications are described., Results: The MSTS functional evaluation was excellent in 166 cases (78%), good in 26 (12%), fair in 11 (5%), and poor in 11 (5%). The following complications were observed: late osteoarthrosis, 25 cases (12%); infection, 12 (6%); pathologic fracture, 11 (5%); and local recurrence, 19 (9%)., Conclusion: Based on clinical assessment, no significant deleterious effects directly related to the use of methylmethacrylate were observed. The functional evaluation performed in 1998 (up to 24 years of follow-up) did not show significant change when compared to the evaluation performed in 1985.

We report on our experience in the treatment of intracranial germinomas (18 pure germinomas and two germinomas with syncytiotrophoblastic giant cells) according to a strategy of radiotherapy doses and fields reduction after a neoadjuvant chemotherapy (Cisplatin-vinblastine and bleomycin combination). Radiation therapy was delivered after the completion of the third and last course of chemotherapy. For the solitary germinoma the target volume was the gross tumour volume. In the five multifocal germinoma patients the whole ventricle volume was irradiated. For the single disseminated germinoma patient we treated the whole central nervous system. The cumulative doses were 30 Gy for the pure germinomas. For the STGCs, a cumulative dose of 35 Gy was used. The median follow-up was 55 months (range 12-120). 18 patients were alive without recurrence of disease. In the two patients with STGCs the death took place 16 and 35 months after diagnosis.

Cauda equina syndrome (CES) is defined as the constellation of symptoms that includes low-back pain, sciatica, saddle anesthesia, decreased rectal tone and perineal reflexes, bowel and bladder dysfunction, and variable amounts of lower-extremity weakness. There are several causes of this syndrome including trauma, central disc protrusion, hemorrhage, and neoplastic invasion. In this manuscript the authors reviewed CES in the setting of both primary and secondary neoplasms. They examined the various primary tumor types in this region as well as those representative of metastatic spread. Both surgical and nonsurgical management in this setting were studied.

Neoplasms that are composed focally, predominantly, or exclusively of osteoclast-like giant cells admixed with variably pleomorphic mononuclear cells have been described in a wide variety of organs. In this report, we describe the case of a 76-year-old woman with an 8-cm tumor that appeared to be localized to the ovary, that was composed predominantly of diffusely distributed, bland-appearing osteoclast-like giant cells admixed with pleomorphic mononuclear cells, and that was not associated with an ovarian cystic neoplasm. Hemorrhage, large zones of necrosis, and a high mitotic index were the other characteristics of the tumor. Immunohistochemically, the mononuclear cells were strongly positive for vimentin and proliferating cell nuclear antigen and were negative for keratin AE 1/3, CAM 5.2, cytokeratin 7, epithelial membrane antigen, beta-human chorionic gonadotropin, desmin, smooth muscle actin, p53, leukocyte common antigen, S-100, inhibin, alpha-1-antichymotrypsin, and CD68. The osteoclast-like giant cells displayed immunoreactivity for CD68, vimentin, alpha-1-antichymotrypsin, and leukocyte common antigen only. Ultrastructurally, rare intercellular junctions were present between mononuclear cells, suggestive of an epithelial histogenesis. Less than a dozen ovarian lesions with the "giant cell" designation have been described, and most of these cases are thought to be analogous to the "sarcoma-like" nodules or other such lesions that have a well-known association with ovarian cystic neoplasms. Our case, in contrast, did not have an easily identifiable epithelial component and demonstrated both an infiltrative border and vascular invasion. This is, to the authors' knowledge, the first detailed clinicopathologic description of such a case as an ovarian lesion.

The giant cell tumor is considered to be a locally aggressive benign tumor and has a low tendency toward distant metastasis. They are primarily present in the long bone and are rare in the skull. The osteoclast-like giant cells do not demonstrate mitotic activity and contain large numbers of nuclei. Herein, we described a case of giant cell tumor in the temporal lobe. Heterogenous enhancement is shown on CT imaging in solid areas of tumor. The appearance intermediate to high signal intensity on T1-weighted and very low signal intensity on T2-weighted MR imaging were due to the presence of hemosiderin from prior hemorrhage.

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

Treatment of giant cell tumors (GCT) especially in the vertebrae remains controversial. With multidisciplinary treatments, their results are still insufficient. Moreover, GCT shows the potential for malignant transformation and metastasis, additional options such as adjuvant medication must be considered. We report favorable results in three consecutive cases diagnosed with GCT of the spine which were treated with radiotherapy and bisphosphonate (BP) as a new treatment option, and present a review of the literature and a comparison with these case reports.

The authors report a case of giant cell tumor originating in the sphenoid bone. A 29-year-old woman presented with headache and diplopia. Bilateral ophthalmoplegia developed and MRI showed rapid growth of the tumor in spite of transnasal removal and conventional radiation therapy. The second transnasal surgery was performed 3 weeks after completion of radiotherapy. The symptoms were relieved except for right abducens palsy. Chemotherapy with cisplatin and etoposide was started after the second operation. The patient regained full ocular movement several months after the operation. Clinical remission has continued for four years. We conclude that the combination of repeated operations in the initial phase of rapid tumor growth, irradiation and chemotherapy is important to achieve tumor control.

Among 56 cases of a giant cell tumor of bone (GCT) and 52 cases of chondrosarcoma (CSA) in our series, four patients were discovered to have a tumor in the pelvic bone that grew in size during pregnancy. These four rare cases are described here. They include three cases of a GCT in the sacrum and one case of a CSA in the innominate bone. The dextran-coated charcoal assay and immunohistochemical techniques demonstrated the independence of these tumors from hormonal regulation despite the growth stimulated during pregnancy. It was concluded that the delay in detection of these tumors in the pelvis was just related to the opportunity afforded for unexpected growth during pregnancy. Surgical management was difficult due to the delay in tumor detection. The initial complaints such as pain, discomfort, or numbness around the pelvis were misinterpreted as symptoms of pregnancy. It should be kept in mind that during pregnancy, any pain or numbness in the pelvic region could be the direct result of a tumor in the pelvic bone.

Little has been written about the specific timing and elements Involved in the follow-up of orthopedic oncology patients. The experience with bone tumor treatment at the First Clinic of the Istituto Rizzoli started 30 years ago and more than 15,000 patients have been treated to date. The increment of limb salvage surgery is linked with the improvements in imaging and surgical skills and with the availability of new reconstructive techniques. For this purpose it is mandatory to carry out meticulous monitoring of the patients. A large number of patients are still followed up to evaluate the results in terms of oncological and functional status. Based on this experience we developed a time schedule to better respond to the need for adequate patient evaluation.