Osteosarcoma Anatomic and Histologic Variants

Department of Pathology, University of Alabama at Birmingham and the Birmingham Veterans Affairs Medical Center 35233, USA.
American Journal of Clinical Pathology (Impact Factor: 3.01). 05/2006; 125(4):555-81. DOI: 10.1309/UC6K-QHLD-9LV2-KENN
Source: PubMed

ABSTRACT Osteosarcoma is the most common primary tumor of bone, yet its absolute incidence among malignant tumors is low. Within its strict histologic definition, osteosarcoma comprises a family of lesions with considerable diversity in histologic features and grade. Its prognosis is dependent not only on these parameters, but also on its anatomic site. It may occur inside the bones (in the intramedullary or intracortical compartment), on the surfaces of bones, and in extraosseous sites. Information of diagnostic or prognostic significance has not been elucidated from studies of its cytogenetics. This review summarizes the anatomic and histologic variations of osteosarcoma and offers a schema for its subclassification.

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Available from: Gene P Siegal, Aug 31, 2015
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    • "The majority of patients with newly diagnosed OS suffer from localized disease and up to 70% survive with state-of-the-art treatment, which comprises local surgical control of the primary tumor combined with neoadjuvant multidrug chemotherapy [2]. Unfortunately, 15–30% of OS patients present with metastases at diagnosis and their 5 year survival rate is only approximately 20% regardless of therapy [3]. Thus, the pathogenesis of OS requires further research to develop more effective treatment of metastatic disease. "
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    ABSTRACT: ΔNp63, a splice variant of p63, is overexpressed and exhibits oncogenic activity in many cancers including pancreatic and breast cancer and promotes cell survival by inhibiting apoptosis. Despite its role in tumorigenesis, mechanistic activity of ΔNp63 mediated oncogenic function in osteosarcoma is poorly understood. The expression levels of p63 isoforms in osteosarcoma cell lines were identified using quantitative techniques. Expression profiling using microarray, siRNA mediated loss-of-function, and chromatin immunoprecipitation assays were employed to identify novel ΔNp63α targets in p63-null osteosarcoma SaOS-2 cells that were engineered to express ΔNp63α. The phenotype of SaOS-2-ΔNp63α cells was assessed using wound-healing, colony formation, and proliferation assays. The comparative expression analyses identified ΔNp63α as the predominant p63 isoform expressed by invasive OS cell lines. Phenotypic analyses of SaOS-2-ΔNp63α cells in vitro indicate that ΔNp63α imparted tumorigenic attributes upon tumor cells. Further, we show that in osteosarcoma cells ΔNp63α directly regulated the transcription factor GLI2, which is a component of the hedgehog signaling pathway, and that functional interactions between ΔNp63α and GLI2 confer oncogenic properties upon OS cells. Here, we report that GLI2 is the novel target gene of ΔNp63α and that ΔNp63α-GLI2 crosstalk in osteosarcoma cells is a necessary event in osteosarcoma progression. Defining the exact mechanisms involved in this interaction that mediate the pathogenesis of osteosarcoma promises to identify targets for drug therapy.
    BMC Cancer 08/2014; 14(1):559. DOI:10.1186/1471-2407-14-559 · 3.32 Impact Factor
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    • "Osteosarcoma characteristically manifests with rapid intra-osseous growth and extra-osseous extension in the metaphysis of long bones [1]. The typical biological characteristics of osteosarcoma are that of an osteolytic mass, often with areas of osteoid or cartilaginous matrix deposition, cortical thickening or destruction, and a periosteal reaction in areas of cortical transgression [2]–[4]. "
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    ABSTRACT: Radiographic imaging plays a crucial role in the diagnosis of osteosarcoma. Currently, computed-tomography (CT) is used to measure tumor-induced osteolysis as a marker for tumor growth by monitoring the bone fractional volume. As most tumors primarily induce osteolysis, lower bone fractional volume has been found to correlate with tumor aggressiveness. However, osteosarcoma is an exception as it induces osteolysis and produces mineralized osteoid simultaneously. Given that competent bone is highly anisotropic (systematic variance in its architectural order renders its physical properties dependent on direction of load) and that tumor induced osteolysis and osteogenesis are structurally disorganized relative to competent bone, we hypothesized that μCT-derived measures of anisotropy could be used to qualitatively and quantitatively detect osteosarcoma provoked deviations in bone, both osteolysis and osteogenesis, in vivo. We tested this hypothesis in a murine model of osteosarcoma cells orthotopically injected into the tibia. We demonstrate that, in addition to bone fractional volume, μCT-derived measure of anisotropy is a complete and accurate method to monitor osteosarcoma-induced osteolysis. Additionally, we found that unlike bone fractional volume, anisotropy could also detect tumor-induced osteogenesis. These findings suggest that monitoring tumor-induced changes in the structural property isotropy of the invaded bone may represent a novel means of diagnosing primary and metastatic bone tumors.
    PLoS ONE 06/2014; 9(6):e97381. DOI:10.1371/journal.pone.0097381 · 3.23 Impact Factor
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    • "Osteosarcoma is the most common primary malignant tumor of bone. In the craniofacial region, osteosarcoma mostly affects jaw bones, with approximately 6 percent of all [15]. The majority of patients are older than 30 years of age. "
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    ABSTRACT: Introduction Osteoblastoma is an uncommon benign bone tumor that accounts for 1 percent of all primary bone tumors. About 30 to 40 percent of all osteoblastoma cases involve the spine. Osteoblastoma involving the nasal cavity is rare, with only 11 reported cases in the English-language literature, while only four cases of turbinate osteoblastoma have been described. Case presentation We report an unusual case of middle turbinate osteoblastoma associated with right-sided nasal obstruction and severe headache in a 14-year-old Caucasian girl. The tumor involved the right middle turbinate, complete anterior and incomplete posterior ethmoidal cells, and the frontal sinus ostium. Cribriform lamina was, in the most part, consumed by the tumor growth, while the skull base was mostly of normal bone structure. Conclusions To the best of our knowledge, this is the first case of middle turbinate osteoblastoma with intracranial spread. Surgical treatment is the only therapeutic option for osteoblastoma.
    Journal of Medical Case Reports 05/2014; 8(1):161. DOI:10.1186/1752-1947-8-161
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