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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Osteosarcoma is the most common primary malignant mesenchymal tumor, accounting for approximately 20% of sarcomas, with 5% incidence in the jaws. They present various clinical and histological aspects as well as variable disease prognosis and outcome. About 50% of all osteosarcomas are osteoblastic, 25% fibroblastic, 25% chondroblastic. Metastasis of osteosarcoma in the oral cavity is rare, and very few cases have been described so far in the literature. This article presents a metastatic case of chondroblastic osteosarcoma in the mandibular right-attached gingiva arising from 4(th) rib. This case report further suggests that chondroblastic osteosarcoma has poor prognosis.
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PMID:Oral metastasis of chondroblastic osteosarcoma. 2329 3

Distinguishing chondrosarcoma from chondroblastic osteosarcoma can be difficult and highly subjective, especially on a small biopsy specimen. This distinction is critical in determining the most accurate prognosis and appropriate treatment modality, as adjuvant chemotherapy with surgery is standard treatment for osteosarcoma, whereas chondrosarcoma is generally treated by surgical excision alone. Cartilaginous neoplasms have recently been shown to frequently (56%) harbor gene mutations in the metabolic enzymes isocitrate dehydrogenase 1 (IDH1) and IDH2 (IDH1>IDH2), whereas other mesenchymal tumors lack these genetic aberrations. We investigated whether the presence of IDH1/2 mutations can be used to distinguish chondrosarcoma from chondroblastic osteosarcoma. Tumors including 25 predominantly high-grade chondrosarcomas and 65 osteosarcomas (44 chondroblastic osteosarcomas and 21 mixed osteosarcomas with a chondroblastic component) were evaluated, and a total of 59 cases (66%) were suitable for genotyping. Mutational analysis was performed using a multiplexed polymerase chain reaction genotyping platform to query for hotspot mutations in the genes IDH1 at codon R132. IDH1-negative cases underwent Sanger sequencing of IDH2 exon 4. No osteosarcomas (0/36) and 61% of chondrosarcomas (14/23) harbored a somatic mutation in IDH1/2, with the majority (86%) of mutations found in the IDH1 gene. IDH1/2 mutation analysis appears to be a promising biomarker for the distinction of chondrosarcoma from chondroblastic osteosarcoma. A positive result strongly favors the diagnosis of chondrosarcoma over chondroblastic osteosarcoma. The presence of IDH1/2 mutations can also help confirm the diagnosis of dedifferentiated chondrosarcoma when the tumor displays osteosarcomatous differentiation.
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PMID:Molecular distinction of chondrosarcoma from chondroblastic osteosarcoma through IDH1/2 mutations. 2359 60

Chondrosarcoma represents approximately 11% of all primary malignant bone tumors. It is the second most common sarcoma arising in bone after osteosarcoma. Chondrosarcomas of the head and neck are rare and may involve the sinonasal tract, jaws, larynx or skull base. Depending on the anatomical location, the tumor can produce a variety of symptoms. Computed tomography and magnetic resonance imaging are the preferred imaging modalities. The histology of conventional chondrosarcoma is relatively straightforward; major challenges are the distinction between grade I chondrosarcomas and chondromas, and the differential diagnosis with chondroblastic osteosarcoma and chondroid chordoma. Surgery alone or followed by adjuvant radiotherapy is the treatment of choice. Radiotherapy alone has also been reported to be effective and can be considered if mutilating radical surgery is the only curative alternative. The 5-year survival for chondrosarcoma reaches 80%; distant metastases and/or local recurrences significantly worsen prognosis. The present review aims to summarize the current state of information about the biology, diagnosis and management of these rare tumors.
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PMID:Chondrosarcomas of the head and neck. 2421 3

Periosteal osteosarcoma is an extremely rare chondroblastic osteosarcoma in the flat bone. There were authors reporting of two cases of periosteal osteosarcoma in the highly unusual sites. One of them arose from the rib, in a 17-year-old male, which appeared as a hypodense juxtacortical mass with periosteal reaction on CT. The other one arose from the scapula, in a 17-year-old female, which showed the intermediate signal intensity (SI) on T1-weighted image (WI), heterogeneous high SI on T2WI, and rim-enhancement on contrast-enhanced T1WI with cortical destruction on MRI.
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PMID:Periosteal osteosarcoma arising from the rib and scapula: imaging features in two cases. 2484 42

Primary osteosarcoma arising from the ribs is very rare. An unusual case of a primary chondroblastic osteosarcoma of the rib in a 30-year-old woman is described here. The patient underwent wide excision of the tumor along with the involved rib, followed by chemotherapy. Although clinically unsuspected in this unusual site, pathology confirmed a completely resected chondroblastic osteosarcoma.
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PMID:Unusual case of chondroblastic osteosarcoma of the rib in an adult. 2488 43

Osteosarcoma (OS) is the most common bone cancer in children and young adults. The etiology of osteosarcoma is currently unknown. Besides the predominant osteoblasts, the presence of cartilage forming chondrocytes within its tumor tissues suggests a role of chondrogenesis in osteosarcoma development. Runx2 is a master transcription factor both for osteoblast differentiation and for chondrocyte maturation. Interestingly, RUNX2 has been shown to directly interact with p53 and Rb1, two genes essential for osteosarcoma development in mice. However the in vivo relevance of Runx2 during osteosarcoma progression has not been elucidated. We have recently shown that targeting Runx2 expression in hypertrophic chondrocytes delays chondrocyte maturation. It has also been shown that osteoblast-specific deletion of p53 and Rb1 genes developed osteosarcoma in mice. Here, we report our recent research findings using these osteosarcoma mouse models as well as human osteosarcoma tissues. We have detected high-level RUNX2 expression in human osteoblastic osteosarcoma, while chondroblastic osteosarcoma is predominant with chondroid matrix. To minimize the effect of strain difference, we have backcrossed osterix-Cre mice onto congenic FVB/N genetic background. We also detected low-GC content (36%) in sequence around the floxed Rb1 gene and demonstrated that addition of BSA into the reaction system increases the efficiency of PCR genotyping of floxed Rb1 gene. Finally, we successfully generated multiple osteosarcoma mouse models with or without Runx2 transgenic background. These mice showed heterogeneous osteosarcoma phenotypes and marker gene expression. Characterization of these mice will facilitate understanding the role of Runx2 in osteosarcoma pathogenesis and possibly, for osteosarcoma treatment.
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PMID:Research findings working with the p53 and Rb1 targeted osteosarcoma mouse model. 2495 78

Radiation-related osteosarcomas are well described malignant mesenchymal neoplasms, yet their pathogenesis is not fully understood. They are generally classified into either skeletal osteosarcomas, or their and rare soft tissue counterpart. The occurrence of osteosarcoma in the urinary bladder (UB) following radiotherapy is exceedingly rare. To the best of our knowledge, only two cases of radiation-related urinary bladder osteosarcoma have been published; we herein describe another case of an 85-year-old man who developed post radiotherapy chondroblastic osteosarcoma of the urinary bladder four years following initial surgical resection and radiotherapy for bladder urothelial carcinoma. We believe that this is the first case of radiation-related chondroblastic osteosarcoma arising in the urinary bladder. In addition, we review the literature and explore the possible histogenesis of this rare neoplasm.
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PMID:Urinary bladder radiotherapy-related chondroblastic osteosarcoma: Rare case report and review of literature. 2512 1

Chondroblastic osteosarcoma (COS), a subgroup of intramedullary osteosarcoma (OS), is the most common osteosarcoma that occurs in adolescents and early adulthood. The COS has similar clinical and radiological features to those of conventional OS. We present a case of 20-year-old male patient with the chief complaint of pain and swelling in the left zygomatic region. The computed tomography (CT) and three-dimensional (3D) CT face showed erosion, calcific foci, sunray type of spicules suggestive of OS. On fine-needle aspiration cytology (FNAC) examination, initial diagnosis was malignant chondroid lesion, with differential diagnosis of mesenchymal chrondrosarcoma, COS on incisional biopsy and finally COS on excisional biopsy. The patient underwent radical resection of left zygomatic arch, followed by chemotherapy. Although clinically unsuspected in this unusual site, histopathology along with immunohistochemistry (IHC) results confirmed the COS. Because zygomatic location of COS is very rare, this report aimed to discuss clinical, radiographic, histopathologic, IHC findings and diagnostic pitfalls of COS in light of the literature.
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PMID:Chondroblastic osteosarcoma of the left zygomatic bone: Rare case report and review of the literature. 2532 13

The purpose of this paper is to report a case of chondroblastic osteosarcoma in the region of the maxilla, with 5 months of evolution. The term osteosarcoma refers to a heterogeneous group of malignancies with bone formation or mesenchymal tissue with histopathological evidence of osteogenic differentiation. The pattern of chondroblastic osteosarcoma represents 25% of all reported cases of this neoplasm. Its histopathological diagnosis is based on the predominance of a chondroid matrix formed in the midst of neoplastic cells. A woman patient, 27-year old, melanoderm, presented on extraoral exam with facial asymmetry caused by the a swelling in the premaxillary region with upper lip protrusion. Intraoral exam showed a maxillary tumefaction with involvement of the vestibular and palatine regions. The computerized tomography (CT) analysis exhibited a radiolucent mass with dispersed areas of radiopacity, with poorly defined and indistinct peripheral edges. The patient was subjected to incisional biopsy and histopathological examination showed the presence of a malignant neoplasm of mesenchymal origin characterized by the presence of irregular bone trabeculae dispersed among mildly atypical chondroblastic cells. The World Health Organization (WHO) recognizes several variants that differ in location, clinical behavior and degree of cellular atypia. The conventional or classical osteosarcoma is the most frequent variant, which develops within the medullary bone. This report illustrates the rapid evolution of one of the histological variants of osteosarcoma.
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PMID:Chondroblastic osteosarcoma. 2594 8

Osteosarcoma, which is most common in non-blood systemic tumors, accounts for 20% of primary bone malignancies. Primary osteosarcoma usually occurs in young individuals aged 10-20 years, while secondary osteosarcoma is more common in the elderly. It had been reported that secondary osteosarcoma may be associated with osteofibrous dysplasia, bone infarction, chondrosarcoma or osteogenesis imperfecta. However, osteosarcoma secondary to fibrosarcoma has rarely been reported. The current study presents the rare case of a female with chondroblastic osteosarcoma secondary to fibrosarcoma. The patient was relatively young and suffered from more than one type of cancer. The course of the disease lasted for >20 years. This case study provides general knowledge of osteosarcoma, and discusses its clinical presentation, diagnosis, treatment and prognosis.
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PMID:Chondroblastic osteosarcoma secondary to fibrosarcoma: A case report and literature review. 2678 71


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