scholarly journals Medical Therapy of Giant Cell Tumor of Bone

2021 ◽  
Author(s):  
Raquel Lopes-Brás ◽  
Isabel Fernandes ◽  
Sandra Casimiro ◽  
Luís Costa

Giant cell tumor of bone (GCTB) is mostly a benign disease of the bone, although with high local recurrence rate and potential for metastatic spread, namely to the lungs. It is also a locally aggressive tumor, associated with severe morbidity and functional impairment due to bone destruction. Treatment is therefore required and should be offered at an early stage to allow complete resection, minimizing functional sequelae and local recurrence. Surgical resection is the mainstay of treatment, often followed by intralesional adjuvant therapy. GCTB has a particular biology, in which RANKL represents a key factor in tumor pathogenesis, thus making this molecule a valuable therapeutic target. Monthly administration of denosumab, a fully human monoclonal antibody directed against RANKL, has been studied in several clinical trials and shown a high rate of local control with favorable safety profile. In this chapter, current medical management, ongoing studies, and future directions in GCTB will be discussed.

Cancers ◽  
2021 ◽  
Vol 13 (24) ◽  
pp. 6298
Author(s):  
Anna Parmeggiani ◽  
Marco Miceli ◽  
Costantino Errani ◽  
Giancarlo Facchini

Giant cell tumor of bone (GCTB) is classified as an intermediate malignant tumor due to its locally aggressive behavior, burdened by high local recurrence rate. GCTB accounts for about 4–5% of all primary bone tumors and typically arises in the metaphysis and epiphyses of the long tubular bones. Mutation of gene H3F3A is at the basis of GCTB etiopathogenesis, and its immunohistochemical expression is a valuable method for practical diagnosis, even if new biomarkers have been identified for early diagnosis and for potential tumor recurrence prediction. In the era of computer-aided diagnosis, imaging plays a key role in the assessment of GCTB for surgical planning, patients’ prognosis prediction and post treatment evaluation. Cystic changes, penetrating irregular margins and adjacent soft tissue invasion on preoperative Magnetic Resonance Imaging (MRI) have been associated with a higher rate of local recurrence. Distance from the tumor edge to the articular surface and thickness of unaffected cortical bone around the tumor should be evaluated on Computed Tomography (CT) as related to local recurrence. Main features associated with local recurrence after curettage are bone resorption around the graft or cement, soft tissue mass formation and expansile destruction of bone. A denosumab positive response is represented by a peripherical well-defined osteosclerosis around the lesion and intralesional ossification. Radiomics has proved to offer a valuable contribution in aiding GCTB pre-operative diagnosis through clinical-radiomics models based on CT scans and multiparametric MR imaging, possibly guiding the choice of a patient-tailored treatment. Moreover, radiomics models based on texture analysis demonstrated to be a promising alternative solution for the assessment of GCTB response to denosumab both on conventional radiography and CT since the quantitative variation of some radiomics features after therapy has been correlated with tumor response, suggesting they might facilitate disease monitoring during post-denosumab surveillance.


2021 ◽  
pp. 100366
Author(s):  
Lenian Zhou ◽  
Shanyi Lin ◽  
Hanqiang Jin ◽  
Zhaoyuan Zhang ◽  
Changqing Zhang ◽  
...  

Author(s):  
Kenta Hayashida ◽  
Yusuke Kawabata ◽  
Ikuma Kato ◽  
Takayuki Kamiishi ◽  
Kosuke Matsuo ◽  
...  

2005 ◽  
Vol &NA; (438) ◽  
pp. 116-122 ◽  
Author(s):  
Richard L McGough ◽  
Janie Rutledge ◽  
Valerae O Lewis ◽  
Patrick P Lin ◽  
Alan W Yasko

2021 ◽  
Author(s):  
taojun gong ◽  
Yi Luo ◽  
Yitian Wang ◽  
Chuanxi Zheng ◽  
Jianguo Fang ◽  
...  

Abstract Background: Giant cell tumor of bone (GCTB) is a rare benign but locally aggressive bone tumor. It has a high tendency for local recurrence, which may increase the occurrence of lung metastasis. Currently, the treatment of pulmonary metastases of GCTB is controversial. Denosumab is the preferred regimen for unresectable metastatic lesions, but there are no alternative treatment options when denosumab is resistant. So far, no case reports of metastatic GCTB treated with denosumab and apatinib have been published. Case presentation: This is a case report of a 26-year-old female who experienced right knee pain for over 6 months. Radiography and computed tomography revealed osteolytic bony destruction in the proximal right tibia. Using histological, radiological, and clinical techniques, a diagnosis of GCTB was achieved. Meanwhile, the immunohistochemical stain-identified the tumor cells were positive for vascular endothelial growth factor receptor 2 (VEGFR-2). After intralesional curettage of the primary tumor and wide resection of local recurrence surgeries, she developed recurrent hemoptysis. Chest computed tomography (CT) images showed multiple pulmonary nodules. She was administrated denosumab therapy but disease progression was confirmed after four months of treatment. She then received denosumab and apatinib therapy for 24 months, after a partial response was achieved.Conclusions: We depict a case of multiple pulmonary metastases of GCTB successfully controlled by denosumab and apatinib therapy. VEGFR-2 may be an effective therapeutic target for GCTB with pulmonary metastasis when denosumab is ineffective.


2019 ◽  
Vol Volume 11 ◽  
pp. 669-680 ◽  
Author(s):  
Mingmin Shi ◽  
Lei Chen ◽  
Yangxin Wang ◽  
Wei Wang ◽  
Yujie Zhang ◽  
...  

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10022-10022
Author(s):  
Emanuela Palmerini ◽  
Stefano Pengo ◽  
Robert G. Maki ◽  
Eric L. Staals ◽  
Angela Cioffi ◽  
...  

10022 Background: Tenosynovial giant cell tumor (TGCT) is a rare, usually benign neoplasm of synovium and tendon sheath. TGCT is classified as localized or diffuse according to the extent of synovial involvement. Surgery is the primary treatment, but the recurrence rate is high, with possible multiple recurrences, joint function deterioration and decline in quality of life. Recent data suggest a role for TKIs in advanced disease. In order to identify prognostic factors for recurrence, a retrospective pooled analysis was carried out in three institutions (Istituto Ortopedico Rizzoli, Bologna, Italy; Istituto Nazionale Tumori, Milano, Italy; Memorial Sloan Kettering Cancer Center, New York, USA). Methods: Clinical charts and pathology reports of patients (pts) treated in the period 1998-2008 were examined. Results: The study included 313 pts, 177 F and 136 M; median age: 36 years (range: 11-89 years). Most (64%) pts had tumors in the knee (15% ankle, 11% hip, 10% other). Tumor size was: <2 cm in 24% of pts, 2-5 cm in 44%, >5 cm in 32%. A diffuse pattern was reported in 69% of pts. The resection status was available in 289 pts: 51% had R0 surgery, 28% R1 and 21% R2. No metastases were documented. Local recurrence was reported in 76 pts (median time to recurrence: 15.7 months). With a median follow-up of 4.2 years, 5-year local recurrence-free survival (LRFS) was 66% (95% CI: 59 - 73). Size (< 2 cm 80% vs. 2-5 cm 67% vs. >5 cm 62%, p=0.04), gender (F 73% vs. M 56%, p=0.02), type (localized 78% vs. diffuse 61%, p=0.02), and resection status (R0 76% vs. R1 55%, vs. R2 57%, p=0.002) influenced 5-year LRFS, whereas age, tumor location and bone involvement did not. The 5-year 2nd LRFS was 43% (95% CI: 28 - 59). Multiple (2 to 5) local recurrences were observed in 39% of relapsed patients. Conclusions: The study confirms TGCT propensity to multiple local recurrences. Diffuse type, suboptimal surgery, male gender and larger tumors increase the recurrence risk. In order to improve the probability of local control, studies addressing the role of TKIs could be considered in subsets of patients.


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