Background: Giant cell tumor of bone (GCTB) is a locally aggressive neoplasm with a high propensity for recurrence following intralesional curettage. The introduction of denosumab, a RANKL inhibitor, has shown potential in facilitating joint-sparing surgery. However, concerns exist regarding its impact on local recurrence rates.
View Article and Find Full Text PDFBackground: Fibrodysplasia ossificans progressiva (FOP) is an extremely rare disease characterized by malformation of the bilateral great toes and progressive heterotopic ossification. The clinical features of FOP occur due to dysfunction of the bone morphogenetic protein (BMP) signaling pathway induced by the mutant activin A type I receptor/activin-like kinase-2 (ACVR1/ALK2) which contributes to the clinical features in FOP. Dysregulation of the BMP signaling pathway causes the development of osteochondroma.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Basis Dis
January 2024
Introduction: Limb-salvage surgery has become the mainstream approaches for the treatment of sarcoma in the lower extremity. In cases where the sarcoma infiltrates the primary vessel, concurrent resection of the vessels and vascular reconstruction are required to ensure sufficient resection and preservation of limb function. The objective of this study is to assess the clinical outcomes of patients who underwent vascular reconstruction utilizing synthetic grafts for limb salvage, specifically in terms of postoperative complications and limb functional status.
View Article and Find Full Text PDFThe distal radius is an extremely rare site for epithelioid hemangioendothelioma. Joint preservation to maintain a good wrist joint function is rarely reported. The present study described a case of joint preservation surgery in the distal radius with an uncemented 3D-printed endoprosthesis and evaluated the endoprosthesis design and short-term outcomes.
View Article and Find Full Text PDFBackground: Patients who undergo massive femoral malignant tumor (MFMT) resection often exhibit shortened femoral metaphyseal juxta-articular segments. The use of a customized femoral endoprosthesis (CFE) with an intra-neck curved stem (INCS) has emerged as a viable reconstructive surgical strategy for these individuals. Relative to a cemented INCS, it remains unclear as to whether cementless INCS use is associated with improvements in functionality or reconstructive longevity.
View Article and Find Full Text PDFExtraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. In view of the indolent course throughout the prolonged natural history of EMC, it was considered as a low-grade soft-tissue sarcoma. However, recent studies have revealed a high recurrence and metastatic potential in EMC, and the invasiveness of EMC may progress during the protracted clinical course.
View Article and Find Full Text PDFIntroduction: Few patients presented with a distal tibial tumor that only invaded a small area of bone in the medial malleolus. There have been no previous cases in which only the medial or lateral malleolus was removed and reconstruction was complete. This article describes our attempt to reconstruct the medial malleolus (1/4 of the ankle joint) after resection of a distal tibial tumor with an uncemented three-dimensional (3D)-printed prosthesis.
View Article and Find Full Text PDFHemiarthroplasty is widely used for proximal femoral reconstruction after tumor resection. However, complications of hemiarthroplasty include infection, hip dislocation, and acetabular wear. This study aimed to: (1) evaluate the reliability and validity of a customized cementless intercalary endoprosthesis (CCIE) with an intra-neck curved stem (INCS) to reconstruct femoral diaphyseal defects with an ultrashort proximal femur (UPF); (2) assess the lower extremity function after reconstruction with this endoprosthesis; and (3) identify the postoperative complications associated with the use of this endoprosthesis.
View Article and Find Full Text PDFOsteosarcoma (OS) is rare cancer with bimodal age distribution with peaks observed in children and young adults. Typically, OS is treated with pre-surgery neoadjuvant therapy, surgical excision, and post-surgery chemotherapy. However, the efficacy of treatment on disease prognosis and objective response is not currently optimal, often resulting in drug resistance; in turn, highlighting the need to understand mechanisms driving resistance to therapy in OS patients.
View Article and Find Full Text PDFBackground: Osteosarcoma is the most common primary malignant bone tumor with a highly metastatic propensity in children and young adolescents. The majority of metastases develope in the lung, while metastases to the extrapulmonary locations have rarely been discussed, especially in skeletal muscle.
Case Presentation: We reported a young patient with pathologically diagnosed osteosarcoma of the right tibia who was initially treated with standard chemotherapy and complete surgical resection.
Epithelioid sarcoma (ES) is a rare soft tissue sarcoma (STS), with limited therapies available for metastatic disease. Here, we describe a case of a 30-year-old male with ES of the left knee and underwent surgery and radiation therapy for the primary disease. After 2 years, he had local recurrence and underwent extensive resection surgery; however, adjuvant chemotherapies were delayed due to recurrent wound infection.
View Article and Find Full Text PDFGiant 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 incidence of lung metastasis. Currently, an optimal treatment strategy has not been established because of the rarity of pulmonary metastatic GCTB.
View Article and Find Full Text PDFEwing sarcoma is the second most common malignant bone tumor in children, but it rarely originates from extra-skeletal sites. The commonly involved sites of soft tissue include paravertebral spaces, lower extremities, the pelvis, head, and neck, while primary extra-skeletal Ewing sarcoma (EES) located in the genitals is extremely rare. We report a young patient who presented to our hospital with a painful erection of the penis and limited motion of the left hip.
View Article and Find Full Text PDFPurpose: Desmoid fibromatosis (DF) is a locally aggressive connective-tissue tumor arising in deep soft tissues. Although multiple therapeutic modalities have been demonstrated effective for DF, there is no standard systemic treatment for progressive and recurrent DF. As a part of systemic treatment, tyrosine kinase inhibitors have shown promising activity against DF with tolerable toxicity profiles.
View Article and Find Full Text PDFOsteosarcoma (OS) is a malignant bone neoplasm, mostly occurring in pediatric patients. OS is characterized by a highly aggressive and metastatically active tumor. Chemotherapy followed by surgical excision is the treatment of choice but is often associated with both chemoresistance and relapse.
View Article and Find Full Text PDFPurpose: Desmoid fibromatosis (DF) is an aggressive fibroblastic neoplasm with a high propensity for local recurrence. Although multiple therapeutic modalities seem effective for DF, the standard systemic treatment for symptomatic and progressive DF remains controversial. As targeted therapy, tyrosine kinase inhibitors have been recently reported to contribute to the treatment of DF.
View Article and Find Full Text PDFBiochim Biophys Acta Rev Cancer
December 2020
Osteosarcoma is the most common primary malignant bone tumor, predominantly occurring in children and adolescents. Despite treated with surgery and neoadjuvant chemotherapy, osteosarcoma has a high potential of local recurrence and lung metastasis. Overall survival rates for osteosarcoma have plateaued in the past four decades, therefore, identification of novel targets and development of more effective treatment strategies are urgent.
View Article and Find Full Text PDFBackground: Gorham-Stout disease, also known as vanishing bone disease, idiopathic massive osteolysis, is a rare entity of unknown etiopathology. This disease is characterized by destruction of osseous matrix and proliferation of lymphatic vascular structures and associated with massive regional osteolysis. It has a variable clinical presentation and is commonly considered as a benign disease with a progressive tendency and an unpredictable prognosis.
View Article and Find Full Text PDFF-box proteins are essential components of the Skp-cullin-F-box complex (a type of E3 ubiquitin ligase), and participate in cell cycle and immune responses through the ubiquitin proteasome system. F-box protein 39 () belongs to the F-box family, which has been reported to be associated with cancer oncogenesis and progression. The present study aimed to investigate the role of in osteosarcoma (OS) cell proliferation and apoptosis .
View Article and Find Full Text PDFProgrammed cell death-ligands 1 (PD-L1) is a key immune checkpoint protein and a promising therapeutic target for malignancy tumor immunotherapy. The prognostic value of PD-L1 in patients with bone and soft tissue sarcoma remains controversial. Therefore, this meta-analysis is conducted to evaluate the associations of PD-L1 expression with overall survival (OS), progression-free survival (PFS), and clinicopathological characteristics of sarcomaA comprehensive literature search of PubMed, Web of Science, Embase, and Cochrane Library was conducted for relevant studies.
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