Desmoplastic fibroma of bone arising in the cortex of the proximal femur

Ishizaka, Takahiro; Susa, Michiro; Sato, Chiaki; Horiuchi, Keisuke; Koga, Ayano; Kumazawa, Fumihisa; Shimazaki, Hideyuki; Chiba, Kazuhiro

doi:10.1016/j.jos.2018.07.011

Cited by 3 publications

(3 citation statements)

References 16 publications

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“…The incidence of DF is estimated to be about 0.1% of all bone tumors, with a slight prevalence for males. The most common site of disease is the mandible (22%) and less frequently other bones such as femur (15%), pelvic bones (13%), radius (12%), and tibia (9%) [ 24 ]. Concerning etiology, no conclusive data are available, however, previous pathologic fractures are sometimes observed.…”

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

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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.

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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

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“…Recurrence following simple curettage has been reported to very high; in consequence, surgical wide excision is the current treatment of choice. 6 7 8 9 Evans et al 10 reported a recurrence rate after intralesional resection of 33%, while more aggressive techniques showed a 0% rate. They also stated that local recurrence was strongly associated with soft tissue extension.…”

Section: Discussionmentioning

confidence: 99%

“…A recorrência após curetagem simples foi relatada como muito alta; consequentemente, a excisão cirúrgica ampla é o tratamento atual de escolha. 6 7 8 9 Evans et al 10 relataram uma taxa de recorrência após a ressecção intralesional de 33%, enquanto as técnicas mais agressivas mostraram uma taxa de 0%. Eles também afirmaram que a recorrência local foi fortemente associada à extensão dos tecidos moles.…”

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Fibroma desmoplásico na tíbia proximal: Relato de caso

Pujol,

Castellanos,

Carrasco

et al. 2024

Rev Bras Ortop (Sao Paulo)

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ResumoO fibroma desmoplásico do osso é um tumor fibrogênico muito incomum, benigno, mas localmente agressivo. Este relato descreve o caso de uma paciente de 45 anos com um enorme fibroma desmoplásico na tíbia proximal. Foi realizado com sucesso um procedimento cirúrgico em dois tempos: ressecção ampla e reconstrução endoprotética. Os cirurgiões devem estar cientes da complexidade de seu tratamento nos casos localmente avançados e agressivos. Uma revisão abrangente da literatura também é fornecida.

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Intramedullary Fixation With a Short Nail in a Young Patient Presenting With a Pathological Proximal Femur Fracture

et al. 2021

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An 18-year-old man presented with a pathological fracture of the right proximal femur. Desmoplastic fibroma was diagnosed through histological studies. Surgical management involved extended intralesional curettage and fracture stabilization by open reduction with intramedullary nailing, using a short Gamma nail. At 42-month follow-up, the patient presented no limitations or recurrence. Internal fixation after prior intralesional curettage is a valid treatment strategy for pathological fractures in young patients. A short nail was chosen to prevent direct tumor cell seeding throughout the femur and future recurrence. Fracture consolidation was achieved because of the healing potential of a young patient.

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Desmoplastic fibroma of bone arising in the cortex of the proximal femur

Cited by 3 publications

References 16 publications

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

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

Fibroma desmoplásico na tíbia proximal: Relato de caso

Intramedullary Fixation With a Short Nail in a Young Patient Presenting With a Pathological Proximal Femur Fracture

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