Giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. Giant cell tumor of bone accounts for 45% of primary bone tumors and 18. Giant cell tumour of bone in the denosumab era sciencedirect. Giantcell tumor of bone an overview sciencedirect topics. Vascular invasion outside the boundary of the tumor can be seen. It most frequently occurs in young adults between 20 and 40 years of age with a slight female predominance 25. Malignant transformation occurs in a small percentage of cases, usually following radiation therapy. The possible surgical procedures range from repeated intralesional curettage to wide resection. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor. Giantcell tumor of bone is a rare, locally aggressive tumor that typically occurs in the bones of skeletally mature young adults in. Giant cell tumor of bone primarily affects the young adult patient population. Malignancy in giant cell tumor is uncommon and occurs in about 2% of all cases. Pdf giant cell tumour of bone in the denosumab era researchgate. It is defined as a highgrade sarcoma originating in a giant cell tumor gct and seems to behave less aggressively than its secondary counterpart does.
Giant cell tumor of bone is a benign tumor with an aggressive behavior. Giant cell tumor health encyclopedia university of. Epidemiology of benign giant cell tumor of bone in the. The tumor is localized epimetaphyseal end of the bone, mainly in the long bones, especially around the knee in 50%, followed by the axial skeleton, especially the sacrum. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. Giant cell tumor gct of bone is one of the commonest benign bone tumors encountered by an orthopedic surgeon. Recognize the biology that drives giant cell tumor of bone. Although well defined in clinical, radiological and histological terms, detailed information on its biological development is still relatively incomplete. Giant cell tumor the spine hospital at the neurological institute. Giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent. Historically, gctb have been treated primarily with surgery. Anzawa u, hanaoka h, shiraishi t, morioka h, morii t, toyama y.
Introduction giant cell tumor gct of bone is a relatively common, locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly. Giant cell tumor of bone is very rarely seen in children or in adults older than 65 years of age. First recognized in 1818 1, it was not until 1940 that gctb was formally distinguished from other tumors of bone, such as aneurysmal bone cyst, chondroblastoma, and nonossifying fibroma 2. You were so generous to give up your time on a weekend to operate on my femur. Bortezomib inhibits giant cell tumor of bone through induction of. While a giant cell tumor can occur in any bone, it is most commonly located in the metaphysis or at the end epiphysis of the thigh bone femur or shin bone tibia.
However, in large series of patients, such as those of the mayo clinic dahlin, 1981 they constitute 5% of the total number of bone tumors and if grouped with the benign lesions, constitute 20%. The absence of clinical, radiographic, or pathologic features that are predictive of tumor behavior and patient outcome has resulted in recent attention to the pathobiology of giant cell tumor of bone. Radiation therapy for giant cell tumors of bone request pdf. We report the case of a patient with a giant cell tumor in the distal ulna.
It usually grows near a joint at the end of the bone. Gctb are typically locally aggressive, but metastasize to the lung in 5 % of cases. Two hundred and fourteen patients with histologically certified gctb have been treated at the authors department from 1980 to 2007. It generally occurs in adults between the ages of 20 and 40 years. Soft tissue neoplasms, giant cell tumors, head and neck neoplasms. The giant cell tumour of bone gct is a locally aggressive intraosseous neoplasm of obscure biological behaviour. Giant cell tumour gct of bone is classified by the world health organisation who as a benign but locally aggressive tumour that usually involves the end of a long bone. Most gcts are located in the epiphyseal regions of long bones.
Not only pain and swelling but also continuous pyrexia may suggest the diagnosis of a secondary. Giant cell a type of cell formed when individual cells merge. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. Giant cell tumor of bone with pulmonary and lymph node metastases. Two cases of sarcoma arising in giant cell tumor of bone. Soft tissue giant cell tumor of low malignant potential of the neck. Giant cell tumour of bone gctb is a locally aggressive rank ligand. Giant cell tumor of bone list of high impact articles. A tumor of bone characterized by massive destruction of the end epiphysis of a long bone. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor of bone. Less than 5% of patients with benign giant cell tumor develop metastases, usually to lungs.
We estimated the 2017 incidence of gct in china using a direct registrybased approach with available populationbased data. Giant cell tumor of bone gct is a rare, aggressive noncancerous tumor. Secondary malignant giant cell tumor of bone due to malignant. A case of recurrent giant cell tumor of bone with malignant. The fever might be attributable to the tumor releasing inflammatory cytokines. Giant cell tumors of bone are uncommon lesions in general orthopedic practice. Rare malignant transformation of benign lesion may be observed with or without use of adjuvant radiotherapy. Giant cell tumor of bone gctb accounts for 5% of primary skeletal tumors. Treatment options for recurrent giant cell tumors of bone.
Giant cell tumor of bone, curettage, cementation, cauterization. Tumor surgery tumor education bone tumors types of bone. Giant cell tumor of bone radiology reference article. When the morbidity of surgery would be excessive, radiation. Giant cell tumor is one of the more common benign bone tumors, occurring in adults ages 20 40 years. Secondary malignancy in giant cell tumor of bone arising after treatment of a benign giant cell tumor has a poor prognosis akin to other high grade sarcomas and much worse than primary malignancy in giant cell tumor of bone cancer 2003. A giant cell tumor of bone is a type of benign noncancerous tumor that typically occurs in young adults between the ages of 20 and 40. Giant cell tumors are common, comprising 1823% of benign bone neoplasms and 49. Giant cell tumor of bone gct is a benign, locally aggressive bone tumor. Pdf pulmonary metastasis of giant cell tumor of bones. Most often, the tumors occur close to the knee jointat the lower end of the thighbone femur or the upper end of the shinbone tibia. Initially, described by cooper and travers in 1818 as an aggressive and destructive lesion of long bones, then virchow first described the recurrence and possible degeneration into a malignant gct. A 17yearold colombian man presented with a painful swelling of the left wrist. Giant cell tumor is one of the more common benign bone tumors.
Giant cell tumour of bone gctb is a rare osteolytic tumour that is responsible for approximately 6 % of all primary bone tumours. Giant cell tumor orthopaedicsone articles orthopaedicsone. Mononuclear stromal cells are the physiologically active and diagnostic cell type. Pdf giant cell tumour of bone gctb is an intermediate locally aggressive primary bone tumour. Giant cell tumor of bone almost invariably occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak incidence between 20 and 30. Denosumab, an inhibitor of the rank ligand involved in bone resorption in gct, is increasingly used in treatment of recurrent or unresectable giant. Giant cell tumor gct of bone is a benign but aggressive bone tumor originating from mesenchymal cells. Giant cell tumor of bone is a locally aggressive bone tumor. Giant cell tumor of bone gctb is a relatively rare, benign, but locally aggressive osteolytic skeletal neoplasm of young adults.
Giant cell tumor of bone gctb is an aggressive osteolytic tumor that. Sarcomatous transformation is rare and typically occurs with a history of recurrences and radiation treatment. Quantifying the incidence of giant cell tumor gct of bone is challenging because it is a rare, histologically benign bone tumor for which populationlevel statistics are unavailable in most countries. Softtissue recurrence of giant cell tumor of bone after irradiation and excision. Giant cell tumor gct of bone is generally a benign tumor composed of mononuclear stromal cells and characteristic multinucleated giant cells that exhibit osteoclastic activity. Sep 22, 2010 giant cell tumor gct of bone is a locally destructive tumor that occurs predominantly in long bones of postpubertal adolescents and young adults, where it occurs in the epiphysis. A giant cell tumor gct of bone is a benign noncancerous bone. Similarities between giant cell tumor of bone, giant cell tumor of tendon sheath, and pigmented villonodular synovitis concerning ultrastructural cytochemical features of multinucleated giant cells and mononuclear stromal cells. It is characterized by the presence of multinucleated giant cells osteoclastlike cells. Giant cell tumour of the bone bone cancer research trust.
Several cases of long bone giant cell tumor have been reported in the literature. Although the recurrence rate of giant cell tumors of bone gctb is relatively high exact data on treatment options for the recurrent cases is lacking. Mar 12, 2015 genetically, 80% of individuals with giant cell tumor of bone exhibit the cytogenetic abnormality of teleomeric associations tas, whereas half of the cells in the tumor show the tas abnormality 1,31. Metastasis, with identical morphology to the primary tumor, occurs in a few percent. Although rarely lethal, benign bone tumors may be associated with a substantial disturbance of the local bony architecture that can be particularly troublesome in periarticular locations. Surgery is the primary mode of treatment, but gct has a tendency to recur locally. Giant cell tumor of bone gpn banff pathology course. Giant cell tumor of bone an overview abstract giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. It usually develops in long bones but can occur in unusual locations.
As a result of reading this article, physicians should be able to. You may not embed one of our images on your web page without a link back to our site. The site most commonly involved by this tumor is the knee, both the far end of the femur and the near end of the tibia. It most often grows in adults between ages 20 and 40 when skeletal bone growth is done. Although it is considered to be a benign lesion, there are still incidences of pulmonary metastasis. Definition of giant cell tumor of bone medicinenet. This is very unusual, with a reported incidence of 0. This benign mesenchymal tumor has characteristic multinuclear giant cells. I believe that the term giant cell sarcoma, when used in reference to benign giant cell tumor, has been partly responsible for the confusion that has existed in the classification and study of this particular group of bone lesions. Primary malignancy in giant cell tumor pmgct is rare. The reported incidence of gct in the oriental and asian population is higher than that in the caucasian population and may account for 20% of all skeletal neoplasms. However, for unknown reasons, this tumour occurs 2 to 3 times more often in southern india and china where giant cell tumours of the bone make up a much larger 20% of all primary bone tumour cases1,3. A shortterm in vivo model for giant cell tumor of bone. Giant cell tumor is a relatively uncommon but painful tumor of bone, which can metastasize to the lungs.
Pdf a shortterm in vivo model for giant cell tumor of bone. The majority are treated by aggressive curettage or resection. Bone tumors develop when cells within a bone divide uncontrollably, forming a lump or mass of abnormal tissue. Giant cell tumor gct of bone is a generally benign, but often locally aggressive, neoplasm of bone, with a propensity for recurrence. Identify atrisk populations for giant cell tumor of bone. Giant cell tumours of the bone make up 45% of all primary tumours which start in the bone. Wittig, i am writing at a loss to find adequate words to thank you for all that you have done for me. Giant cell tumor of the bone gctob, is a relatively uncommon tumor of the bone. Sep 03, 2019 present da, bertoni f, springfield d, braylan r, enneking wf. They almost invariably 9799% occur when the growth plate has closed and are therefore typically seen in early adulthood. Giant cell tumor of bone gctb is a primary bone tumor composed of two types of cells. After performing an instrumental examination, a diagnosis of distal ulna giant cell tumor was. Local recurrence of giant cell tumor of bone after intralesional treatment with and without adjuvant therapy.
The natural history of gct is progressive bone destruction leading to joint deformity and disability. Giant cell tumor gct of bone is one type of giant cell rich lesion of bone. Its typical subarticular location and high recurrence risk can be associated with significant morbidity. Giant cell tumor of bone is a rare, fastgrowing noncancer tumor. This report presents the case of a 39yearold female with pain in her left shoulder for one month. Giant cell tumor of bone gctb comprises up to 20 % of benign bone tumors in the us.
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