Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Giant cell tumor of bone is a rare but aggressive benign tumor that arises at the end of long tubular bones. The tumor rarely metastasizes; however, we report a case in which a giant cell tumor of bone presented with progressive pulmonary metastases. There has been no clear pathologic evidence of the definitive cause or route of metastasis. In our case, the primary tumor site was located in the left femur with pathological evidence of blood vessel invasion. The histological and pathological features of this entity are discussed in this letter to the editor.
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PMID:Giant-cell tumor of bone with pathological evidence of blood vessel invasion. 2863 39

Giant cell tumor of bone (GCT), which frequently occurs in the patients' spine, is relatively prevalent in Chinese population. A group of GCT invades into vessels and appears to be circulating tumor cells (CTCs) responsible for the distal metastasis of the primary tumor. So far the cell surface markers of GCT have not been determined. In the current study, we aimed to identify a novel CTC marker with higher specificity in GCT. TRAIL-R1+ cells were purified from GCT cell lines. The TRAIL-R1+ cells were compared with total GCT cells for tumor sphere formation, chemo-resistance, tumor formation in nude mice, and frequency of developing distal metastases. We found that TRAIL-R1+ GCT cells appeared to be highly enriched for CTCs in GCT. Compared to total GCT cells, TRAIL-R1+ GCT cells generated significantly more tumor spheres in culture, were higher chemo-resistant, and had a higher frequency of being detected in the circulation after subcutaneous transplantation as well as development of distal metastases. Thus, we conclude that TRAIL-R1+ may be a novel CTC marker in GCT. Selective elimination of TRAIL-R1+ GCT cells may improve the current GCT therapy.
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PMID:TRAIL-R1 as a novel surface marker for circulating giant cell tumor of bone. 2888 98

Giant cell tumor of bone is a benign albeit aggressive tumor commonly affecting the bones of the knee. Patients with these tumors present with pain, swelling, and inability to bear weight on the involved extremity. These destructive tumors typically arise in the metaphyseal region of the long bones in individuals in the second, third, and fourth generations of life. Histologically, the multinucleated giant cells are the hallmark of the lesion, easily recognized on histological review, which recently have become therapeutic targets for medical management of the disease. For decades, surgical management has been the primary treatment for giant cell tumor of the bone. Some tumors can be treated with excision and filling of the osseous void with bone cement or allograft. This is an effective treatment option with a low to moderate risk of local recurrence while preserving limb function. For more destructive tumors, wide excision and reconstruction with prosthetic, structural allograft or combined allograft prosthetic components are utilized. Advances in medical management of the disease have also demonstrated promise as an effective treatment; however, its use has usually been limited to the treatment of metastatic disease, recurrent disease or when advanced local disease would require surgical treatment felt to be overly morbid.
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PMID:Giant Cell Tumor of Bone: Review of Current Literature, Evaluation, and Treatment Options. 3044 24

Giant cell tumor of bone is a histologically benign but locally aggressive osteolytic lesion, capable of spreading 'benign' metastases mainly to the lungs. Since its description as a separate entity, surgery has been the mainstay of treatment. Recently, target therapy has been introduced using denosumab - an inhibitor antibody for the receptor activator of nuclear factor kappa B ligand with dramatic eff ect on the natural course of the tumor. Herein, we report a rare case of an aggressive stage 3 giant cell tumor of bone of the proximal fibula that was successfully downstaged using neoadjuvant denosumab treatment and then treated by surgical excision followed by adjuvant target therapy. The clinical characteristics and treatment modalities of giant cell tumor of bone, the indications for target therapy, therapy response, and histological changes are also briefly discussed.
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PMID:Neoadjuvant and Adjuvant Treatment with Denosumab in Aggressive Giant-cell Tumor of Bone in the Proximal Fibula: a Case Report. 3118 71

Giant cell tumor of bone is a benign tumor with an aggressive behavior. Its typical subarticular location and high recurrence risk can be associated with significant morbidity. Although benign, it can rarely metastasize especially to the lungs. Also, it can be multicentric in less than 1% of patients. Late malignant transformation, although rare, can occur with a very poor prognosis. This series reports on these unusual and challenging features and management considerations of giant cell tumor of bone. This retrospective study included review of the medical records of patients with a confirmed histopathological diagnosis of giant cell tumor of bone. A total of 25 patients (16 females and 9 males) with a mean age of 34.5 years were included; 22 had primary tumors, while 3 were referred with recurrent tumors. Pain was the most common presenting symptom. Most patients had grade III tumors. Tumors around the knee were the most common. Multicentric tumors were detected in three patients. Twenty-three patients (20 primary giant cell tumor of bone and 3 with recurrence) received treatment. Most patients (15/23) were treated with intralesional curettage with or without adjuvants. Seven patients had wide excision. Recurrence was seen in 45% (9/20) of primary giant cell tumor of bone especially with difficult anatomical locations. Most recurrences occurred more than 4 years after treatment. Pulmonary nodules were detected in four patients; two of them showed resolution during follow-up. One patient developed secondary sarcoma transformation with a fatal outcome. Giant cell tumor of bone was more common in females. Long bones were more affected, especially around the knee. Intralesional curettage was the most frequently used treatment. Recurrence was associated with inadequate tumor resection (especially in difficult anatomical location), younger age, male gender, and advanced local tumor grade. Denosumab can be used in the treatment of pulmonary metastasis, multicentric and recurrent giant cell tumor of bone. Due to late recurrence and malignant transformation, a prolonged follow-up is warranted.
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PMID:Giant cell tumor of bone: Unusual features of a rare tumor. 3159 8

Giant cell tumor of bone (GCT) is a benign locally aggressive neoplasm composed of mononuclear cells admixed with innumerable osteoclast-type giant cells. H3F3A gene mutations producing mutant histone protein product H3.3 have been identified in 96% of GCT; mutant H3.3 is reliably demonstrated by immunohistochemistry. GCT may contain woven bone and rarely, neoplastic cartilage nodules which causes diagnostic challenges with aggressive neoplasms such as osteosarcoma. We describe the features of GCT with cartilage matrix and report the next-generation sequencing findings in a subset of tumors. Seventeen cases of GCT with cartilage matrix form the cohort: 7 males and 10 females, 13 to 55 (mean: 25) years old. Tumors involved the fibula (6), femur (6), and patella, tibia, humerus, S1, and scapula (1 case each). Tumors were radiolucent, circumscribed, lytic, and expansile. All contained classic GCT, foci of cartilage matrix, and trabeculae of woven bone. Immunohistochemistry showed diffuse staining for H3.3 in 9/9 cases and 1 case was positive for S100 and SOX9 in the cartilage areas. Next-generation sequencing showed a mutation in the H3F3A gene in 6/6 cases. On follow-up, 2 patients who underwent resection showed no disease after 12, and 7 months, respectively. Three patients had recurrences 10, 12, and 27 months after curettage; there were no metastases. GCT with cartilage matrix is uncommon. The cartilage matrix is associated with woven bone suggesting the neoplastic cells may differentiate into chondrocyte-like and osteoblast-like cells. Recognition of this neoplasm is important to prevent misdiagnosis and overtreatment of affected patients.
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PMID:Giant Cell Tumor of Bone With Cartilage Matrix: A Clinicopathologic Study of 17 Cases. 3241 16


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