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      Treatment of Clival Giant Cell Tumor: A Case Report and Literature Review

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          Abstract

          Giant cell tumors (GCTs) are locally aggressive primary bone tumors of osteoclast-like cells. Most GCTs occur within the long bones, and primary GCTs involving the clivus are extremely rare. We present the case of an 18-year-old boy with binocular horizontal diplopia with an insidious onset who was found to have a hypointense enhancing mass involving the clivus and left side dorsum sellae on magnetic resonance images. The tumor was completely resected via an endoscopic endonasal transclival approach, and histopathologic examination via immunohistochemistry indicated a GCT. The patient’s left abducens nerve palsy improved slightly after surgery. Because of the rarity of GCTs, there is no consensus about the definitive treatment protocol. However, we suggest that gross total resection is the treatment of choice, and denosumab plays a critical role in patients with subtotal resection.

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          Most cited references37

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          Giant-cell tumor of bone.

          Of 327 patients who had a giant-cell tumor of bone and were seen at the Istituto Rizzoli, 293 were treated at the Institute, and 280 of these were followed for two to forty-four years. The distribution according to sex and age of the patient and site of the tumor was similar to the distributions in major reports of large series. The tumor usually involved the metaphysis and the epiphysis, but was occasionally limited to the metaphysis, and in only 2 per cent of the patients was it adjacent to an open growth plate. The tumor on occasion invaded the articular space, also involving the ligaments and the synovial membrane. Extension to an adjacent bone through the joint occurred in 5 per cent of the tumors. Our radiographic grading, which is roughly comparable with the staging system of Enneking et al., was Grade I in 4 per cent, II in 74 per cent, and III in 22 per cent of 266 patients before treatment. A pathological fracture was apparent on the first radiograph in 9 per cent of the patients. In the 280 patients with adequate follow-up, 331 surgical procedures were performed. The rate of local recurrence was 27 per cent in the 151 intralesional procedures, 8 per cent in the 122 marginal excisions, and zero in the fifty-eight wide or radical procedures. These results did not correlate with the radiographic grade of the lesion. Of the fifty-one local recurrences that were seen after treatment at our institution, 90 per cent appeared in the first three years after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)
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            Giant cell tumor of bone.

            To discuss the treatment and outcomes for giant cell tumor (GCT) of bone. Review of the pertinent literature. GCT is a rare benign bone lesion most often found in the extremities of women in the third and fourth decades of life. Surgery is the mainstay of treatment and usually consists of intralesional curettage; local control rates range from 80% to 90% after this procedure. Patients with extensive, recurrent, and/or biologically more aggressive tumors may require wide excision. A small subset of patients with incompletely resectable GCTs or with lesions that are surgically inaccessible may be treated with moderate-dose radiotherapy (45-50 Gy) and have a 65% to 80% likelihood of being locally controlled. The majority of patients with GCTs are effectively treated with intralesional curettage. Wide excision or radiotherapy is necessary to cure a relatively limited subset of patients with extensive, aggressive, and/or incompletely resectable GCTs.
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              Chordoma and chondrosarcoma: similar, but quite different, skull base tumors.

              Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis. The objective of the current study was to investigate the distinctions between these 3 entities. The data concerning 109 patients with chordoma, chondroid chordoma, and chondrosarcoma who were treated by a single surgeon with maximum surgical resection and frequently by adjunct proton beam radiotherapy between 1990 and 2006 were analyzed retrospectively. Pathologic distinction was established by cytokeratin and epithelial membrane antigen staining. Clinical, radiologic, pathologic, and cytogenetic studies were analyzed in relation to disease recurrence and death. The average follow-up was 48+/-37.5 months (range, 1-191 months). There were no reliable distinguishing clinical or radiologic features noted between the groups. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrence-free survival (P=.028 and P<.001, respectively), whereas patients with chondroid chordoma had a poor outcome similar to chordoma patients with regard to survival and recurrence-free survival (P=.337 and P=.906, respectively). Chordoma and chondrosarcoma differ with regard to their origin and histology, and differ markedly with regard to outcome. Chondroid chordomas behave in a manner that is clinically similar to chordomas, with the same prognosis. Both chordoma types demonstrate an aggressive clinical course and poor outcome after disease recurrence. The optimal treatment for all groups of patients involves radical surgical resection followed by high-dose radiotherapy in patients with chordomas. Radiotherapy may not be necessary in patients with low-grade chondrosarcoma. Copyright (c) 2007 American Cancer Society.
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                Author and article information

                Journal
                Brain Tumor Res Treat
                Brain Tumor Res Treat
                BTRT
                Brain Tumor Research and Treatment
                The Korean Brain Tumor Society; The Korean Society for Neuro-Oncology; The Korean Society for Pediatric Neuro-Oncology
                2288-2405
                2288-2413
                April 2024
                30 April 2024
                : 12
                : 2
                : 132-140
                Affiliations
                [1 ]Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
                [2 ]Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam, Korea.
                [3 ]Pituitary Center, Seoul National University Hospital, Seoul, Korea.
                Author notes
                Correspondence: Yong Hwy Kim. Department of Neurosurgery, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel: +82-2-2072-4062, Fax: +82-2-744-8459, kimhy96@ 123456snu.ac.kr
                Author information
                https://orcid.org/0009-0002-6727-2652
                https://orcid.org/0000-0003-2143-410X
                https://orcid.org/0000-0001-9009-4191
                Article
                10.14791/btrt.2024.0010
                11096631
                38742263
                06a6f4a1-d080-4756-b959-cfcef9b0c90d
                Copyright © 2024 The Korean Brain Tumor Society, The Korean Society for Neuro-Oncology, and The Korean Society for Pediatric Neuro-Oncology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 05 March 2024
                : 22 March 2024
                Funding
                Funded by: Seoul National University, CrossRef https://doi.org/10.13039/501100002551;
                Award ID: 800-20220266
                Funded by: Seoul National University Hospital, CrossRef https://doi.org/10.13039/501100004332;
                Award ID: 0520200030
                Categories
                Case Report

                cranial fossa, posterior,giant cell tumor of bone,h3-3a protein, human,denosumab

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