Giant cell tumor of bone at the proximal epiphysis of humerus in a skeletally immature patient: A case report

Ramírez-González, Manuel; Olivella, Gerardo; Ramírez, Norman; Soler-Salas, Antonio; Astacio, Eric; Bibiloni, Juan; Foy-Parilla, Christian

doi:10.1016/j.ijscr.2020.11.112

Cited by 2 publications

(1 citation statement)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MGCs are recruited and induced from mononuclear precursors of osteoclasts by the tumor stromal cells, which represent the neoplastic component of the tumor and often harbor a highly specific mutation in the histone variant H3.3 which is encoded by H3F3A [ 4 ]. GCTB occurs in young adults in epiphyseal–metaphyseal regions of the long bones, such as distal femur (26%), proximal tibia (20%) and distal radius (11%), while in iliac bone, spine and hand bones, cases are rarely reported [ 5 , 6 , 7 , 8 ]. The incidence of this bone sarcoma is estimated in almost 5% of all primary bone tumors, with a slight female predilection [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

A Rationale for the Activity of Bone Target Therapy and Tyrosine Kinase Inhibitor Combination in Giant Cell Tumor of Bone and Desmoplastic Fibroma: Translational Evidences

et al. 2022

View full text Add to dashboard Cite

Giant cell tumor of bone (GCTB) and desmoplastic fibroma (DF) are bone sarcomas with intermediate malignant behavior and unpredictable prognosis. These locally aggressive neoplasms exhibit a predilection for the long bone or mandible of young adults, causing a severe bone resorption. In particular, the tumor stromal cells of these lesions are responsible for the recruiting of multinucleated giant cells which ultimately lead to bone disruption. In this regard, the underlying pathological mechanism of osteoclastogenesis processes in GCTB and DF is still poorly understood. Although current therapeutic strategy involves surgery, radiotherapy and chemotherapy, the benefit of the latter is still debated. Thus, in order to shed light on these poorly investigated diseases, we focused on the molecular biology of GCTB and DF. The expression of bone-vicious-cycle- and neoangiogenesis-related genes was investigated. Moreover, combining patient-derived primary cultures with 2D and 3D culture platforms, we investigated the role of denosumab and levantinib in these diseases. The results showed the upregulation of RANK-L, RANK, OPN, CXCR4, RUNX2 and FLT1 and the downregulation of OPG and CXCL12 genes, underlining their involvement and promising role in these neoplasms. Furthermore, in vitro analyses provided evidence for suggesting the combination of denosumab and lenvatinib as a promising therapeutic strategy in GCTB and DF compared to monoregimen chemotherapy. Furthermore, in vivo zebrafish analyses corroborated the obtained data. Finally, the clinical observation of retrospectively enrolled patients confirmed the usefulness of the reported results. In conclusion, here we report for the first time a molecular and pharmacological investigation of GCTB and DF combining the use of translational and clinical data. Taken together, these results represent a starting point for further analyses aimed at improving GCTB and DF management.

show abstract