The majority of these tumors develop in patients aged. 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. H3f3a mutation detection can be used as a diagnostic tool for the distinction of giant cell tumor of bone from other giant cell containing tumors. Giant cell tumours gct are benign noncancerous tumours that develop in the bone. It usually develops near a joint at the end of the bone. The vast majority of giant cell tumors occurs in extremity sites and is treated by surgery alone. Giant cell tumor of bone is a locally aggressive bone tumor. Patients with local recurrence are more likely to develop pulmonary. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor of bone gctb.
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. 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. Giant cell tumor is a relatively uncommon but painful tumor of bone, which can metastasize to the lungs. Lately, denosumab is being utilized in the treatment of certain gctbs. Giant cell tumors are benign bone tumors that can grow aggressively and destroy bone close to a joint. Imaging of giant cell tumor and giant cell reparative. It is immensely important to initially correctly diagnose giant cell tumors. Giant cell tumor is a one of the most common primary bone lesions in the distal phalanx. 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. Giant cell tumor is one of the more common benign bone tumors. Giant cell tumour of the bone bone cancer research trust. 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. Giant cell tumor of bone in patients 55 years and older. Giant cell tumours are common, comprising 1823% of benign bone neoplasms and 49.
Giant cell tumor of the temporal bone with direct invasion. 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. 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.
Historically, gctb have been treated primarily with surgery. The incidence of giant cell tumors of the bones of the hand and foot seen. After a second ctguided biopsy an histological diagnosis of recurrence of giant cell tumor of bone was made. Sarcomatous transformation is rare and typically occurs with a history of recurrences and radiation treatment. Giant cell tumor of bone, curettage, cementation, cauterization. Giant cell tumor gct of bone is a generally benign, but often locally aggressive, neoplasm of bone, with a propensity for recurrence. 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. Orthopedics educational objectives as a result of reading this article, physicians should be able to. Giant cell tumor of bone gct is a rare, aggressive noncancerous tumor.
Giant cell tumors of the bone with pulmonary metastasis. Pdf giant cell tumor gct of bone is generally a benign tumor composed of mononuclear stromal cells and characteristic multinucleated. Feb 01, 2020 a rare bone sarcoma characterized by a usually benign spaceoccupying lesion, which is nevertheless locally aggressive and massively damaging to surrounding bone tissue. Giant cell tumor of bone is a primary bone neoplasm occurring most frequently in young adults, with a slight female predominance. 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 tumour of bone gctb is a primary intermediate bone tumour with a local aggressive behaviour and a rare tendency to metastasise. Pdf giant cell tumor of bone an overview researchgate. Mononuclear stromal cells are the physiologically active and diagnostic cell type.
Intensity modulated radiotherapy imrt in benign giant cell. The distribution according to sex and age of the patient and site of the tumor was similar to the distributions in. Giant cell tumor is a benign but locally aggressive bone neoplasm which uncommonly involves the skull. Tumor surgery tumor education bone tumors types of bone. 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. Giant cell tumor of bone gct represents approximately 5% of primary bone tumors and most commonly occurs in skeletally mature individuals between the ages of 20 and 40 years.
Softtissue recurrence of giant cell tumor of bone after irradiation and excision. Giantcell tumor of bone is a rare, locally aggressive tumor that typically occurs in the bones of skeletally mature young adults in. The following list of medications are in some way related to, or used in the treatment of this condition. 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. List of giant cell tumor of bone medications 2 compared. Roentgen therapy for a giant cell tumor of the sphenoid bone. Giant cell tumor of bone gctb is mostly a benign tumor, but associated with recurrences and metastasis. Local recurrence of giant cell tumor of bone after intralesional treatment with and without adjuvant therapy. Definition of bone, giant cell tumor of medicinenet. 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 giant cell tumour of bone. Although they are benign, gct can grow fast and damage the affected bone and spread to the soft tissue around it.
Pdf a recurrent giant cell tumor of bone treated with denosumab. The purpose of this study was to evaluate the correlation between the surgical stage and the oncological or functional results of the treatment of giant cell tumor of bone at kyushu university hospital. Only bones, and rarely in the parietal or frontal bones. Denosumab, an inhibitor of the rank ligand involved in bone resorption in gct, is increasingly used in treatment of recurrent or unresectable giant. They mostly occur in the long bones found in the arms and legs. 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. While benign noncancerous, giant cell tumors of bone can grow quickly. Giant cell tumor gct of bone is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent. Pdf giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. This benign mesenchymal tumor has characteristic multinuclear giant cells. 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.
Two cases of sarcoma arising in giant cell tumor of bone. To discuss the treatment and outcomes for giant cell tumor gct of bone. Giant cell tumors gcts of the bone are locally progressive and destructive borderline malignant neoplasms, which comprise 5% of primary bone tumors and 20% of benign tumors. For orthopedists expert at recognizing bone and soft tissue tumors, the diagnosis of gct is relatively easy. Radiation therapy for giant cell tumors of bone request pdf. Vascular invasion outside the boundary of the tumor can be seen. Giant cell tumor of the bones of the hand and foot biscaglia 2000. A strange giant cell tumor european journal of radiology. Giant cell tumor is one of the more common benign bone. Giant cell tumor of the temporal bone a case report bmc. Giant cell tumor of bone gctb comprises up to 20 % of benign bone tumors in the us. 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. Giant cell tumor of bone list of high impact articles.
Giantcell tumor of the bone gctob, is a relatively uncommon tumor of the bone. Giantcell tumor of bone gctb is a rare tumor typically affecting the bones of skeletally mature young adults, with peak incidence in the third and fourth decades of life. Pdf giant cell tumor of bone revisited researchgate. Gct mostly affects people between the ages of 20 and 30 years old. Giant cell tumors are common, comprising 1823% of benign bone neoplasms and 49. A giant cell tumor gct of the bone is a benign but locally aggressive bone tumor. Gctb, mainly occurs at the metaepiphysis of the long bones, but also in difficult sites such as pelvis and spine, preferably in patients aged between 30 and 50 years with an estimated incidence of 1.
Giant cell tumor of bone radiology reference article. The petrous portion of the temporal bone forms a rare location for this tumor. Giant cell tumor gct of bone is a benign but aggressive bone tumor originating from mesenchymal cells. Pulmonary metastasis of giant cell tumor of bones world. Recognize the biology that drives giant cell tumor of bone. The majority are treated by aggressive curettage or resection. Denosumab in patients with giantcell tumour of bone. Giant cell tumor of bone is typified by massive infiltration of a bland neoplastic stroma by osteoclasts and monocyte progenitors. Pdf giant cell tumor of bone dror robinson academia. Of 327 patients who had a giantcell tumor of bone and were seen at the istituto rizzoli, 293 were t. It generally occurs in adults between the ages of 20 and 40 years. Giant cell tumor of bone with pulmonary and lymph node metastases.
Giant cell tumor of bone gct is a benign locally aggressive neoplasm composed of mononuclear cells admixed with innumerable osteoclasttype giant cells. Primary giant cell tumor of soft tissues similar to bone giant cell tumor. A giant cell tumor is one that is made up of a large number of benign noncancerous cells that form an aggressive tumor. 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. Malignancy in giant cell tumor is uncommon and occurs in about 2% of all c. 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. Wittig, i am writing at a loss to find adequate words to thank you for all that you have done for me. Anzawa u, hanaoka h, shiraishi t, morioka h, morii t, toyama y. Although it is considered to be a benign lesion, there are still incidences of pulmonary metastasis. Others have used the term dedifferentiated giant cell tumors to refer to these lesions.
Giant cell tumor gct of bone is one type of giant cell rich lesion 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. Case report giant cell tumor of the maxilla in an 8 year. Figure 2 tibia showing a lytic expansile lesion with septae consistent with aneurysmal bone cyst. Giant cell tumor of bone is very rarely seen in children or in adults older than 65 years of age. Typically they occur close to the knee, wrist, ankle or hip. Primary giant cell tumor of soft tissues similar to bone. Giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. Gctb are typically locally aggressive, but metastasize to the lung in 5 % of cases. Giant cell tumor gct of bone was first described by sir astley cooper in 1818, 1. Giant cell tumors gcts are usually found in the epiphysis of the long bones, and represent 5% of all bone tumors. First recognized in 1818, it was not until 1940 that gctb was formally distinguished from other tumors of bone, such as aneurysmal bone cyst, chondroblastoma, and nonossifying fibroma. Giant cell tumor is one of the more common benign bone tumors, occurring in adults ages 20 40 years.
Sep 03, 2019 present da, bertoni f, springfield d, braylan r, enneking wf. Though benign tumor, it is locally aggressive and has malignant potential they have significant bone destruction,local recurrence and occasional metastasis 3. Giant cell tumor orthopaedicsone articles orthopaedicsone. 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 rare neoplasms, representing less than 5% of all bone tumors. At kyushu university hospital, 55 patients with giant cell tumor of bone were treated between 1963 and 1990. It causes pain, restricts movement, and is usually cancerous. It can occur in any bone and can incur significant morbidity, due to a tendency to occur in the bones surrounding the wrist and knee, as well as the axial skeleton. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor of bone.
The current study aimed at evaluating the nature of the neoplastic cells and the mechanisms underlying the. For giant cell tumor of bone, the accepted approach of treatment is intralesional curettage with or without adjuvant therapy, leaving en bloc resection as an alternative in recalcitrant or. 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. A subset of the mutations can be easily detected using a g34w mutation specific antibody. It is characterized by the presence of multinucleated giant cells osteoclastlike cells. Giant cell tumour of bone in the denosumab era sciencedirect. 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. Among them, aneurysmal bone cysts abcs and giant cell tumors gcts are the most frequently observed in the spine.
Pdf malignancy in giant cell tumor of bone eric staals. Giant cell tumor of bone gctb accounts for 5% of primary skeletal tumors. Two hundred and fourteen patients with benign giant cell tumor of bone gctb, treated from 1980 to 2007 at the department of orthopedics of the university of muenster germany, were analyzed in a retrospective study. It usually develops in long bones but can occur in unusual locations. A tumor of bone characterized by massive destruction of the end epiphysis of a long bone. This pathway is a key signaling pathway of bone remodeling that plays a critical role in differentiation of precursors into multinucleated osteoclasts, and activation of osteoclasts. Primary malignancy in giant cell tumor pmgct is rare. A case of recurrent giant cell tumor of bone with malignant. As a result of reading this article, physicians should be able to. Musculoskeletal imaging 197 giant cell tumor of bone. It is probable that the tumor in this case study was malignant from the start, was incompletely resected and therefore not a recurrence. Giant cell tumours of the bone make up 45% of all primary tumours which start in the bone. The tumor is composed of giant multinucleated cells osteoclastlike cells, mononuclear macrophages, and mononuclear stromal cells which secrete promyeloid and pro.
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. Giant cell tumor of the bone gctob, is a relatively uncommon tumor of the bone. Giant cell tumor of bone express p63 modern pathology. 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 some reports show a higher number of giant cell tumour of the bone cases in female patients than. It accounts for 410% of all bone tumors and typically affects the metaepiphyseal region of long bones of young adults. Giant cell tumor gct is classified as a benign bone tumor, and it is frequently identified at the epiphysis of long bones and relatively rare in the temporal bone.
Giant cell tumor of bone an overview abstract giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. Malignant transformation occurs in a small percentage of cases, usually following radiation therapy. Most gcts are located in the epiphyseal regions of long bones. The full text of this article is available in pdf format. Historically, the lesion has been referred to by numerous terms, including myeloid sarcoma, tumor of myeloplaxus, osteoblastoclastoma, and osteoclastoma, 2, 5 gct is a relatively common skeletal tumor, accounting for 4%9. Metastasis, with identical morphology to the primary tumor, occurs in a few percent. The site most commonly struck by this tumor is the knee the far end of the femur and the near end of the tibia. 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. Rare malignant transformation of benign lesion may be observed with or without use of adjuvant radiotherapy. Giant cell tumors of the bone are found predominantly in the epiphysis and metaphysis of. Introduction it is one of the most common bone tumors encountered. 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. They almost invariably 9799% occur when the growth plate has closed and are therefore typically seen in early adulthood. However, a small percentage occurs in pelvis, spine or skull bones, where complete resection is challenging.
Giantcell tumor of bone an overview sciencedirect topics. Giant cell tumor of bone genetic and rare diseases. You were so generous to give up your time on a weekend to operate on my femur. The location of a giant cell tumor is often in the knee, but can also involve the bones of the arms and the legs, or the flat bones such as the breastbone or pelvis. Calcitonin use in giant cell bone tumors emconsulte. This report presents the case of a 39yearold female with pain in her left shoulder for one month. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. They are always found at the end of the bone next to the joint. 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. Abstract background giant cell tumor of the small bones of the hand and foot.
Giant cell tumour of bone radiology reference article. Giant cell tumor of bone childrens hospital of philadelphia. The authors report a case of a large giant cell tumor involving the petrous and squamous portions of the temporal bone in a 26 year old male patient. Giant cell tumor of bone gctb is a relatively rare, benign, but locally aggressive osteolytic skeletal neoplasm of young adults. Giant cell lesions are benign, nonodontogenic, relatively uncommon tumors of the oral cavity, developing peripherally in soft tissues gingiva or centrally in bone. Malignancy in giantcell tumor is uncommon and occurs in about 2% of all cases. Identify atrisk populations for giant cell tumor of bone. Less than 5% of patients with benign giant cell tumor develop metastases, usually to lungs.
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