Primary Giant Cell Tumor of the Axis: a Case Report
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Abstract Background: Primary giant cell tumor of the axis is a rare. The authors reported a case of a primary giant cell tumor of the axis revealed by cervical pain, and discussed the diagnosis and treatment of giant cell tumor.Case presentation: The patient presented to our clinic with neck pain and unstable gait. X-ray, computed tomography and magnetic resonance imaging showed osteolysis of the body and vertebral arch of the axis. Histologic evaluation gave a conclusion of a giant tumor, grade II. Spondylectomy of the axis was performed by the transoral approach. The local recurrence of the tumor was found 3 months later and the patient refused further therapy.Conclusion: Primary giant cell tumor of the axis is a rare type tumor with poor prognosis. Definitive diagnosis should be based on histopathological morphology and surgical treatment should be performed as soon as possible.Keywords:
Neck pain
Presentation (obstetrics)
Giant Cell Tumors
Case presentation
Primary tumor
Localized tenosynovial giant cell tumor (GCT) or giant cell tumor of tendon sheath (GCTTS), is a benign nodular lesion that arises from the synovium of the tendon sheath of the hands and foot. GCTTS is characterized by the presence of multinucleated giant cells and proliferation of synovial-like mononuclear cells. A clinical diagnosis of GCTTS is kept as a differential when a firm, nodular mass shows decreased signal intensity on both T1-and T2-weighted MR imaging. Treatment is usually marginal excision of the mass.It is a retrospective study, observed in the past 3 years at a tertiary care hospital. Those cases were included in the study in which histopathological confirmation was available or if clinico-radiological features were confirmatory of the diagnosis of GCTTS when correlated with cytological features.There was a total of 24 cases, out of which 16 were females and 8 males. The tumor was located in the upper limb in 21 cases and in 3 cases the tumor was present in the lower limb. In the upper limb, 18 cases were on the right side and three cases were on the left side. In the lower limb, 1 case was present on the left and 2 on the right side. The cytomorphology consisted of mononuclear stromal cells, multinucleated giant cells, and hemosiderin-laden macrophages in variable numbers.It is important to accurately diagnose and categorize giant cell-containing lesions because their prognosis depends on the exact categorization of the tumor.
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In three cases of a giant cell tumor-like lesion of bone, the histological characteristics of the lesions were a fibrogenic stroma, capillaries, thick-walled blood vessels, multinucleated giant cells, osteoid tissue and bone trabeculae. It is believed that tumors of that kind are another giant cell variant that should be separated from the true giant cell tumor if for no other reason than the favorable response to conservative surgical therapy.
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THE PRESENCE of multinucleated giant cells in a variety of bone lesions has led to inclusion of certain neoplastic, inflammatory, developmental, and metabolic disorders of bone under the diagnosis of giant-cell tumor or giant-cell tumor variant. In 1940, Jaffe, Lichtenstein, and Portis (1) offered a specific definition of giant-cell tumor which has gained wide, though not universal, acceptance. They considered it a distinctive neoplasm apparently arising in the nonosteoblastic connective tissue, composed of a vascularized network of spindle-shaped or ovoid stromal cells interspersed with multinuclear giant cells (which they regarded as syncytial stromal cells). In subsequent publications Jaffe and Lichtenstein (2–6) identified as distinct clinical and pathologic entities many of the lesions formerly classed as giant-cell tumor variants. Accurate identification of lesions in this category is of considerable practical importance. Most of the socalled giant-cell tumor variants are fundamentally benign lesions offering an excellent prognosis, while the true giantcell tumor is a distinctly more formidable neoplasm. Lichtenstein (7) has stated that approximately half of the proved giant-cell tumors will respond favorably to proper management, approximately a third will prove more aggressive and will recur after treatment, and the remainder ultimately will be frankly malignant. Changing concepts of the pathology of bone tumors have prompted Dahlin and associates to undertake a comprehensive review of the bone lesions encountered at the Mayo Clinic. Tissues obtained at surgical operation have been subjected to detailed gross and microscopic study, and the lesions have been reclassified in accordance with current concepts. Approximately 5 per cent of the lesions in which material was available for their review were giant-cell tumors. One practical result of a critical review in the light of changing concepts can be shown by the experience of the Mayo Clinic. In 1932, Kirklin and Moore (8) reported on the roentgenographic appearance of 86 pathologically confirmed giantcell tumors seen over a twenty-year period. On review, however, only 42 lesions encountered during that period met the revised criteria for the pathologic diagnosis of giant-cell tumor. Some of the lesions originally diagnosed as giant-cell tumor have now been reclassified pathologically as aneurysmal bone cyst, benign chondroblastoma, fibroma, benign giant-cell reparative granuloma, unicameral bone cyst, osteogenic sarcoma, fibrosarcoma, and fibrous dysplasia. The Cases Studied Williams, Dahlin, and Ghormley (9) have recently reported on the clinical and pathologic features of the giant-cell tumors observed at the Mayo Clinic. Their report was based on 101 cases seen from 1905 through December 1953. Since that report 1 of the lesions included in the series has been reclassified as an aneurysmal bone cyst and 4 new cases have been added.
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The article by Kaban et al1 appearing in this month's issue ofPediatrics is of interest because it describes, to the best of my knowledge, the first completely successful medical treatment of a primary giant cell tumor of the bone with the angiogenesis inhibitor interferon α-2a. Previously, a 37-year-old woman with a benign giant cell tumor of the leg that spread to the lungs demonstrated a significant decrease in pulmonary metastases and stabilization of disease following treatment with interferon α-2a.2 Giant cell tumors of the bone have 3 major cell types: 1) proliferating mononuclear cells thought to be the neoplastic element of the tumor, 2) nonproliferating mononuclear cells, and 3) multinucleated giant cell that are fused mononuclear cells.3 The incidence of giant cell tumors among primary bone neoplasms is 4% to 5%, with most occurring around the knee and <1% in the skull. Because 95% are benign, the incidence of these tumors among benign bone tumors rises to 18%.4,5 More than 50 cases of pulmonary metastases associated with benign giant cell tumors have been described.6 Aneurysmal bone cysts are associated with almost 15% of giant cell tumors, as was the case in this present study. With rare exceptions, giant cell tumors occur in skeletally mature individuals, with about 75% occurring in patients between 15 and 40 years of age. It has been estimated that only 1.7% of giant cell tumors occur in skeletally immature individuals.4The treatment for giant cell tumors is problematic. Approximately 40% to 70% of these lesions recur after marginal resection (curettage), although this rate can be decreased to about 25% if the lesion has a low histologic stage. Less than 10% of these tumors recur after wide resection; a procedure often limited because of the location of the lesion and/or the desire to preserve limb function.4 Conventional anticancer chemotherapy has occasionally been reported to be effective,5 while radiation therapy is associated with a recurrence rate of about 50%. The problem with radiotherapy is that it not only carries the usual risk of radiation-induced sarcoma but also the risk of radiation-induced giant cell tumor-specific sarcomas. This later risk has been estimated to be from 7% to 33%, although modern radiation techniques are said to decrease this risk.4Because of the rather limited success of current therapeutic modalities, the apparent success of interferon α-2a therapy is a welcome discovery. Many more typical patients, (ie older with extremity lesions), will have to be successfully treated before one can say with any surety that this inhibitor of angiogenesis is effective.7 After attending a lecture at which Dr Folkman discussed the case described in this month's issue, we gave interferon α-2a to a 17-year-old female with a nonosseous, pelvic, giant cell tumor that could not be removed surgically and was completely resistant to radiotherapy and embolization. A significant reduction in tumor size was observed and the patient regained some of the function that was compromised as a result of compression and local infiltration.In addition to those tumors discussed by Kaban et al that are responsive to interferon, interferon has also been shown to be effective in immunoblastic lymphadenopathy8 and, along with interleukin 2, renal cell carcinoma.9 Administration of interferon has been associated with fever, fatigue, anorexia, myelosuppression, and central nervous system and gastrointestinal toxicity.10 Cardiovascular complications have been observed and they include rhythm abnormalities, myocarditis, cardiomyopathy, myocardial infarction, and hypotension. Some of these cardiac problems may be attributable, in part, to the inhibitory effects of interferon on neovascularization which, in turn, may lead to an increase in the thickness of the endothelial processes of myocardial capillary walls.11The web site for the National Cancer Institute lists more than 20 phase I-III trials, each evaluating a different angiogenesis inhibitor, that are currently open for patients with various types of cancer. If only some of these studies are successful, we will have added to our armamentarium antitumor agents that, either alone or in combination with current antineoplastic agents, are less toxic and more tumor-specific than our present therapeutic regimens. Furthermore, because these agents have a unique mechanism of action, they may prove effective when other, more conventional, therapy fails. The study of angiogenesis inhibitors is of intense interest and promise.
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Introduction:Giant cell lesions of bone include true giant cell tumors & numerous benign osteoclasts and pseudo-anaplastic-appearing giant cells containing variants.Many times it is difficult to differentiate between true giant cell tumor and other tumor like conditions.Aims:To study histopathology of various giant cell rich tumor and tumor like conditions of bone.Methods and Material: Retrospective analysis of 50 cases of giant-cell rich lesions of bone diagnosed and treated at Smt. N.H.L. Municipal Medical College Ahmedabad, Gujarat during 1st January 2002 to 1st January 2003 included in the study. Patients’ clinical, radiological details, histopathological examination were studied using structured proforma. The cases were classified in different categories according to age groups, types of tumour, benign versus malignant category.Results: The most common giant cell containing benign tumor is giant cell tumor (19 cases) followed by Aneurysmal bone cyst (5), Most of the giant cell containing tumors of bone are found in younger age group and are located in epiphysis The common giant cell containing malignant tumor is osteogenic sarcoma (7 cases) followed by Talangiectatic O.S (01). The majority of cases found in age between 15 - 28 years and most common sites are epiphysis of long bones.Conclusion:The most common giant cell rich benign bone tumor is giant cell tumor and most common giant cell rich malignant bone tumor is osteosarcoma commonly occurs in younger age groups in the epiphysis region long bonesDOI:10.21276/APALM.1524
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Epiphysis
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Abstract. A histological comparison has been made of the giant cells in 10 cases which had been diagnosed as central giant cell granuloma of the jaws and 10 cases diagnosed as giant cell tumor of long bone. The numbers of nuclei in each of 50 giant cells were counted in the 20 lesions. The giant cells were measured in order to compare their sizes in the respective lesions. It was found that the giant cells of the jaw lesions contained significantly fewer nuclei than those of the lesions in other bones. Secondly it was found that cells in the lesions of long bone have significantly larger dimensions than those in the jaw lesions. There was, however, an area of overlap. It was concluded that these data supported the view that giant cell tumor and giant cell granuloma are distinct entities. Some giant cell lesions of long bones are, however, morphologically indistinguishable from giant cell lesions of the jaws; and conversely giant cell lesions of the jaws are indistinguishable from some giant cell lesions of the long bones. It seems possible, therefore, that some jaw lesions are giant cell tumors and that some giant cell lesions outside the jaws are giant cell granulomas.
Central giant-cell granuloma
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Giant Cell Tumors
Epulis
Central giant-cell granuloma
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The distinction between giant cell tumors and giant cell granulomas is challenging, as both entities have overlapping diagnostic criteria, especially in oral locations. The two entities have similar clinical and radiological presentations, but they differ in their prognoses.
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