Involvement of the bones of the foot and ankle is rare, and there have been a limited. Abstract giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and. The characteristic histological appearance of gct displays a high number of osteoclast. Giant cell tumor of bone radiology reference article. We searched pubmed and ovid when the study was being planned in 200506, with the terms giant cell tumor of bone, giant cell tumor, bone neoplasms, bone cancer, osteoclastoma. Giant cell tumor of bone 1 authorstream presentation.
They mostly occur in the long bones found in the arms and legs. Giant cell tumor gct of bone is generally a benign tumor composed of mononuclear stromal cells and characteristic multinucleated giant cells that exhibit. Giant cell tumors of the bone with pulmonary metastasis. Malignancy in giant cell tumor of bone wiley online library.
Giant cell tumour of the bone bone cancer research trust. Although the tumor was clinically considered a ganglion initially, the microscopic features were identical to those found in classic gct of bone. Active untreated lesions are associated with significant focal bone lysis and skeletal complications, including pathologic fracture, pain, limitation of joint motion, nerve compression, and mechanical destabilization of adjacent joints or the axial spine depending on. Report of clinical characteristics and linkage analysis of a large pedigree 15 january 20 journal of bone and mineral research, vol. Although it is considered to be a benign lesion, there are still incidences of pulmonary metastasis. Local recurrence of giant cell tumor of bone after intralesional treatment with and without adjuvant therapy. Giant cell tumor of bone gct is a locally osteolytic tumor with variable aggressiveness. Involvement of the bones of the foot and ankle is rare, and there have been a limited number. Epidemiology of benign giant cell tumor of bone in the. Although gct is classified as a benign lesion, few patients develop progressive lung metastases with poor outcomes 25, 76, 77. Whether that tumor arises in the epiphysis or distal metaphysis is a matter of controversy, but giant cell. 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. Giant cell tumor gct is a benign but locally aggressive neoplasm comprising approximately 5% of all primary bone.
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. Giant cell tumor is a type of benign tumor which has the characteristic of rapidly growing and a chance to metastasis. H3f3a gene mutations producing mutant histone protein product h3. Gct is a rare benign bone lesion most often found in the extremities of women in the third and fourth decades of life. Benign but locally aggressive primary bone neoplasm composed of mononuclear round to spindle cells with numerous evenly dispersed osteoclastlike giant cells. Giant cell tumors of bone, also known as osteoclastomas, are relatively common bone tumors and are usually benign. Patients with local recurrence are more likely to develop pulmonary. Giant cell tumours gct are benign noncancerous tumours that develop in the bone.
Primary giant cell tumor of soft tissues similar to bone. It is however locally aggressive and would typically affect. Although they are benign, gct can grow fast and damage the affected bone and spread to the soft tissue around it. Report of clinical characteristics and linkage analysis of a large pedigree 15 january 20 journal of bone and mineral. Giant cell tumor gct of bone was described by cooper and travers in 1818. Because giant cell tumors destroy bone, there is risk for pathologic fracture in the area of the tumor.
Giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. Giant cell tumor of bone slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Giant cell tumors are common, comprising 1823% of benign bone neoplasms and 49. 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. As a result of reading this article, physicians should be able to. 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. P rimary malignancy in giant cell tumor of bone pmgct is extremely unusual.
Pdf giant cell tumor of bone revisited researchgate. Giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent. Giant cell tumor is a one of the most common primary bone lesions in the distal phalanx. Multiple giant cell tumors and paget disease of bone. All giantcell tumors were considered at one time to be malignant lesions and were classified by the term giantcell sarcoma. In a previous publication i 1 suggested the use of the terms benign giant cell tumor and malignant giant cell sarcoma in an attempt to clarify the differentiation. It generally happens in adults between ages 20 and 40 when skeletal bone growth is complete. Apr 17, 2015 contents definition of giant cells origin classification of giant cell lesions giant cell granuloma tumor aneurysmal bone cyst traumatic bone cyst osteoid osteoma osteoblastoma cherubism brown tumor of hyperparathyroidism histiocytosis 3. Giant cell tumor of bone is a rare, aggressive noncancerous tumor. The nature of giant cell tumor of bone springerlink.
Light microscopy showed a lesion composed of a homogeneously mixed proliferation of spindle and polygonal mononucleated stromal cells and evenly distributed multinucleated, osteoclast. It accounts for 410% of all bone tumors and typically affects the metaepiphyseal region of long bones of young adults. However, for unknown reasons, this tumour occurs 2 to 3 times more often in southern india. Only bones, and rarely in the parietal or frontal bones. Malignancy in giantcell tumor is uncommon and occurs in about 2% of all cases. Identify atrisk populations for giant cell tumor of bone. Of 327 patients who had a giantcell 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 fortyfour years. Giant cell tumors gcts are usually found in the epiphysis of the long bones, and represent 5% of all bone tumors.
The multinucleated giant cells appear similar to osteoclasts, which led to the older term osteoclastoma 2. Pdf giant cell tumor of bone nancy mendenhall academia. Giant cell tumor occurring in familial pagets disease of bone. Giant cell tumors of the bone are found predominantly in the epiphysis and metaphysis of long bones in young adults. Giant cell tumors of bone gctbs are benign tumors occur mainly in young adults with potential for aggressive behavior and capacity to metastasize. Pdf giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize.
Review article giant cell tumor of bone international journal of. Giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and occasionally metastatic malignant bone tumor. Giant cell tumor of bone, local recurrence, p63, prognostic marker introduction giant cell tumor of bone gct is a primary bone tumor that occurs predominantly in young adults. Pdf giant cell tumor of bone an overview researchgate. There is compact spindle stroma lacking collagenization.
It is classified as being locally aggressive, but rarely metastasizing in the. Giant cell tumor gct of bone is a rare, histologically benign but locally aggressive neoplasm of the bone. Jan 28, 2016 a giant cell tumor gct of the bone is a benign but locally aggressive bone tumor. The lesion most frequently occurs in the epiphysis of long tubular bones of the knee region, predominantly affecting young adults after closure of the growth plate. Giant cell tumour central giant cell lesion of the maxilla. Of 327 patients who had a giantcell 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. It is characterized by the presence of multinucleated giant cells osteoclastlike cells.
Denosumab in patients with giantcell tumour of bone. To discuss the treatment and outcomes for giant cell tumor gct of bone. Benign giant cell tumor, showing the characteristic pattern of a loosely packed spindle cell stroma, a fig. Giant cell tumor is one of the more common benign bone. Giant cell tumor of bone gctb is a benign but locally aggressive bone tumor of young adults. Pulmonary metastasis of giant cell tumor of bones world. Giant cell tumor of soft tissue lacks the h3f3a mutations characteristic of giant cell tumor of bone suggesting that despite the histologic resemblance, these tumor types are not in fact related. Giant cell tumor of the bone gctob, is a relatively uncommon tumor of the bone.
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. Pdf malignancy in giant cell tumor of bone eric staals. Pulmonary metastasis of gctb may be affected by tumor grading and localization as well as the age, gender and overall health status of the patient. Metastases after gct of bone are rare, occurring in only 3% of patients the behavior of pulmonary metastases is unpredictable 5, 12, 38, 78 81. Giant cell tumor of bone gctb is rare, with 150 patients diagnosed each year in japan. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor of bone. Arbeitsgemeinschaft knochentumoren, becker wt, dohle j, et al. Most often, the tumors occur close to the knee jointat. Malignancy in giant cell tumor is rare giant cell tumor of bone definition. 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. In rare cases, pulmonary metastasis can be observed.
Giant cell tumor, or osteoclastoma, usually develops in patients 20 to 50 years old. We searched pubmed and ovid when the study was being planned in 200506, with the terms giant cell tumor of bone, giant cell tumor, bone neoplasms, bone cancer, osteoclastoma, chemotherapy, radiation therapy, embolization, and surgery for publications in the english language related to treatment of patients with giantcell tumour of bone. A giant cell tumor gct of the bone is a benign but locally aggressive bone tumor. Although it is considered to be a benign lesion, there are still incidences of pulmonary. Giant cell tumor gct of bone is one of the commonest benign bone tumors encountered by an orthopedic surgeon. 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. Giant cell tumor of bone gpn banff pathology course. Whether that tumor arises in the epiphysis or distal metaphysis is a matter of controversy, but giant cell tumors only occur after the epiphyseal plates have closed and a diagnosisof gct in a patient with open growth plates should be questioned. Malignancy in giant cell tumor is uncommon and occurs in about 2% of all cases.
Giant cell tumor accounts for 5 to 9 percent of all primary bony tumors. Recognize the biology that drives giant cell tumor of bone. The rib is an unusual site for giant cell tumors 4 which more commonly arise eccentrically in the epiphysis of long bones 4. In a previous publication i 1 suggested the use of the terms benign giantcell tumor and malignant giantcell sarcoma in an attempt to clarify the differentiation. Radiation therapy for giant cell tumors of bone request pdf. Case report giant cell tumor of the maxilla in an 8 year. Giant cell tumors are usually found in the long bones, most often the distal femur, proximal tibia, and distal radius. Giant cell tumor of bone with pulmonary metastases. It usually develops near a joint at the end of the bone. Giant cell tumor of bone and other giant cellrich lesions. Aug 20, 2014 giant cell tumor of bone gctb accounts for 5% of primary skeletal tumors. They are always found at the end of the bone next to the joint. Satb2 expression in locally aggressive giant cell tumor of. 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 fortyfour years.
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. Giant cell tumours of the bone make up 45% of all primary tumours which start in the bone. Giant cell tumor of bone gctb accounts for 5% of primary skeletal tumors. 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. Giant cell tumor of bone gct is a benign locally aggressive neoplasm composed of mononuclear cells admixed with innumerable osteoclasttype giant cells. Giant cell tumors of the bone are found predominantly in the epiphysis and metaphysis of long. It is characterized by the presence of multinucleated giant cells osteoclast like cells. Musculoskeletal imaging 197 giant cell tumor of bone. 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. The location of a giant cell tumor is often in the knee, but can also involve the bones of the arms and the legs. Not the same tumor as giant cell lesion of the small bones. Giant cell tumor of bone radiology reference article radiopaedia.
Gct mostly affects people between the ages of 20 and 30 years old. Abstract giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and occasionally metastatic malignant bone tumor. Typical roentgenographic appearance is a lytic lesion. Giantcell tumor of the bone gctob, is a relatively uncommon tumor of the bone. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. Case report giant cell tumor of the maxilla in an 8 year old boy. Giant cell tumor is a benign primary bone neoplasm which most often occurs in a periarticular location. Giant cell tumor of bone an overview abstract giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. They almost invariably 9799% occur when the growth plate has closed and are therefore typically seen in early adulthood. It accounts for 410% of all bone tumors and typically affects the metaepiphyseal region of long bones of young. Benign and malignant giantcell tumors of bone, diagnosis and result of treatment.