Giant cell tumor of the bones of the hand and foot biscaglia 2000. 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. The most common location of a giant cell tumour of the bone is around the knee joint where over 50% of giant cell tumours arise4. Giant cell tumours of the bone can also start in the pelvis, the spine, the ribs, the skull and the sacrum which is the base of. Giant cell tumor of bone gpn banff pathology course. 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. 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. Sep 27, 2012 multinucleated giant cell containing tumors and pseudotumors of bone represent a heterogeneous group of benign and malignant lesions.
Two cases of sarcoma arising in giant cell tumor of bone. They are always found at the end of the bone next to the joint. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. Literature documents a close association of secondary sarcomatous transformation in. 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.
Patients with local recurrence are more likely to develop pulmonary. Giant cell tumor of bone, bone, cat, multinucleated giant cells corresponding author. Giant cell tumors have a recognizable pattern on xray a large, almost transparent area in the bone where the tumor has destroyed bone cells. Giant cell tumor is one of the more common benign bone. The breakdown between sexes has ranged from even to a predilection toward females 51.
Challenges of denosumab in giant cell tumor of bone, and oth. Giant cell tumor gct is a benign, locally aggressive primary bone tumor. Rare malignant transformation of benign lesion may be observed with or without use of adjuvant radiotherapy. They almost invariably 9799% occur when the growth plate has closed and are therefore typically seen in early adulthood. Gct mostly affects people between the ages of 20 and 30 years old. Can p63 serve as a biomarker for giant cell tumor of bone. Muller da, beltrami g, scoccianti g, campanacci da, franchi a, capanna r. Giant cell tumor of bone with pulmonary and lymph node metastases. Giant cell tumor of the bones of the hand and foot. Giant cell tumor of bone gctb first described by cooper and travers in 1818 1. Malignancy in giant cell tumor is uncommon and occurs in about 2% of all cases.
The standard treatment for giant cell tumors typically includes surgery to remove as much of the tumor as possible and bone reconstruction, if needed. Sarcomatous transformation is rare and typically occurs with a history of recurrences and radiation treatment. Although it is considered to be a benign lesion, there are still incidences of pulmonary metastasis. Giant cell tumor of bone radiology reference article. Their local aggressiveness explained by later nelaton and malignant features showed by virchow 2,3. 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. The aim of this study was to analyze the features of lesions such as these in the files of the rizzoli orthopedic institute. 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 petrous portion of the temporal bone forms a rare location for this tumor. They mostly occur in the long bones found in the arms and legs. Giant cell tumor of bone presenting as left posteromedial. Giant cell tumor orthopaedicsone articles orthopaedicsone.
Giant cell tumours of the bone can also start in the pelvis, the spine, the ribs, the skull and the sacrum which is the base of the spine where it connects to the pelvis 2. 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. Tenosynovial giant cell tumor soft tissue giant cell tumor of low malignant potential. Its typical subarticular location and high recurrence risk can be associated with significant morbidity. Pdf giant cell tumor of bone an overview researchgate. Pulmonary metastasis of giant cell tumor of bones world. Although surgical treatment resulted in many cures of patients who had these tumors, i became wary in my prognosis for i found that occasionally there was recurrence of the. A host bony trabecula is embedded in a giant cell tumor proliferation. Giant cell tumor of the bone gctob, is a relatively uncommon tumor of the bone. Giant cell tumour of the bone bone cancer research trust. If the inline pdf is not rendering correctly, you can download the pdf file here. Giant cell tumor of the sphenoid bone cambridge core. Differential diagnosis can be challenging, particularly in instances of limited sampling. Usually polymorphous with siderophages, foam cells and lymphocytes.
If a tumor cannot be removed through surgery, newer drugs such as denosumab are being used. Giant cell tumor of the bone usually presents after a long course of pain caused by the tumor growth, which can also impact mechanical functioning, biermann said. Giant cell 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. Softtissue recurrence of giant cell tumor of bone after irradiation and excision. The majority are treated by aggressive curettage or resection.
Arbeitsgemeinschaft knochentumoren, becker wt, dohle j, et al. Only five patients were shown to have malignancy in a giant cell tumor at the time of diagnosis. The tumor is composed of giant multinucleated cells osteoclastlike cells, mononuclear macrophages, and mononuclear stromal cells which secrete promyeloid and pro. Not the same tumor as giant cell lesion of the small bones. Less than 5% of patients with benign giant cell tumor develop metastases, usually to lungs.
Aug 20, 2014 giant cell tumor of bone gctb accounts for 5% of primary skeletal tumors. Giant cell tumor of bone is a benign tumor with an aggressive behavior. Twentyfive of these 31 had previously been irradiated. The disease occurs more often in mature individuals, around the age of 30. Giant cell tumor gct of bone is one of the commonest benign bone tumors encountered by an orthopedic surgeon. The impression was that of a grade i benign giant cell tumor of bone. The rank pathway is often reported to be involved in the pathogenesis of giant cell tumor of bone.
Pdf giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. Cooper in 1818 first described giant cell tumors gct of the bone 1. 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. Of 327 patients who had a giantcell tumor of bone and were seen at the istituto rizzoli, 293 were t. 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. It appears most commonly at the ends of tubular long bones, usually in closed epiphyses around the knee and the. He presented with right side severe hearing loss and facial. Giant cell tumor of bone gctb is an uncommon benign primary bone tumor that mainly affects the long bones. Usually lacks siderophages, foam cells and lymphocytes. Giant cell tumor of bone an overview abstract giant cell tumors gct are benign tumors with potential for aggressive behavior and capacity to metastasize. 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. A giant cell tumor of bone is a type of benign noncancerous tumor that grows at the ends of the bodys long bones.
Pdf giant cell tumor gct of bone is generally a benign tumor composed of mononuclear stromal cells and characteristic multinucleated. Metastasis, with identical morphology to the primary tumor, occurs in a few percent. 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. Benign but locally aggressive primary bone neoplasm composed of mononuclear round to spindle cells with numerous evenly dispersed osteoclastlike giant cells. 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. Giant cell tumours gct are benign noncancerous tumours that develop in the bone. Differential diagnosis soft tissue giant cell tumor of low. A lthough giant cell tumors have been reported in different areas of the skull and paranasal sinuses, their occurrence in the. It is characterized by the presence of multinucleated giant cells osteoclastlike cells. Although they are benign, gct can grow fast and damage the affected bone and spread to the soft tissue around it. 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.1140 1415 1191 542 1360 884 571 511 1116 927 544 558 210 1349 1095 620 1291 980 1350 818 1051 988 946 691 1016 1010 319 1345 202 1071 1284