Diagnostic utility of molecular investigation in extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma is characterized by the reciprocal chromosomal translocation t(9;22) and the resultant fused gene EWS RNA-binding protein 1 and nuclear receptor subfamily 4, group A, member 3 (EWSR1-NR4A3). A second cytogenetic rearrangement t(9;17) involves the genes NR4A3 and TAF 15 RNA polymerase II, TATA box binding protein (TBP)-associated factor (TAF15). Less frequent fusion transcript variants of the NR4A3 gene, transcription factor 12 (TCF12)-NR4A3 and TRK-fused gene (TFG)-NR4A3, are associated with t(9;15) and t(9;3) respectively.

Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.

Background: Angiosarcoma of bone is a rare high-grade malignant vascular tumor. The literature regarding treatment and outcome of patients with this tumor is limited.We performed a 2 institutional retrospective study to analyze treatment and survival of patients with angiosarcoma of bone.

The Combination of Sorafenib and Everolimus Abrogates mTORC1 and mTORC2 upregulation in osteosarcoma preclinical models.

Purpose: The multikinase inhibitor sorafenib displays antitumor activity in preclinical models of osteosarcoma. However, in sorafenib-treated patients with metastatic-relapsed osteosarcoma, disease stabilization and tumor shrinkage were short-lived and drug resistance occurred. We explored the sorafenib treatment escape mechanisms to overcome their drawbacks.

Sustained autocrine induction and impaired negative feedback of osteoclastogenesis in CD14(+) cells of giant cell tumor of bone.

Giant cell tumor (GCT) of bone is a histologically benign osteolytic tumor featuring prominent osteoclast-like giant cells, mononuclear osteoclast precursors, and spindle-shaped stromal cells (SCs). Thus far, most studies have identified SCs as truly transformed elements that are responsible for sustained giant cell formation via receptor activator of NF-κB ligand (RANKL) paracrine induction. However, we have previously shown that SCs are hyperplastic, rather than neoplastic, and able to induce giant cell formation similar to that of normal mesenchymal SCs; we hypothesized that other cell subsets of GCTs might be primarily relevant for the pathogenesis.

Osteosarcoma of the pelvis: a monoinstitutional experience in patients younger than 41 years.

Aims And Background: Information is scarce on systemic treatment of pelvic osteosarcoma because most chemotherapy protocols for osteosarcoma include patients with extremity tumors and aged up to 30-40 years.

Methods: Data on patients <41 years of age with high-grade pelvic osteosarcoma were prospectively collected. Patients received two chemotherapy protocols consisting of methotrexate, cisplatin, doxorubicin (MAP) and standard-dose or high-dose ifosfamide.

Solitary fibrous tumors of the soft tissues: imaging features with histopathologic correlations.

Purpose: To describe the imaging features of soft tissue solitary fibrous tumors, with histopathological correlations and clinical outcome.

Material And Methods: Twenty-seven patients with histologically proven SFTs were retrospectively evaluated. Imaging studies included six radiographs, five U/S studies, eighteen CT scans, fourteen MRI exams, and one angiography.

Osteosarcoma of the hands and feet: a distinct clinico-pathological subgroup.

Osteosarcomas of hands or feet are rare, and seemingly these cases differ in presentation and behavior compared to those in usual locations. The clinico-pathological presentation of patients with osteosarcomas of the hand or foot was studied and compared with published cases. Forty osteosarcomas were identified among 4,221 cases, representing 0.

Solitary bone lesions: which ones to worry about?

The question is not classic: which signs suggest a possible malignancy when faced with a solitary bone lesion? Usually radiologists try to identify the leave me alone lesions, for which nothing is needed. Here we consider the suspicious lesions. Clinical and radiological indicators are proposed, leading to a probability.

Malignant fibrous histiocytoma and fibrosarcoma of bone: a re-assessment in the light of currently employed morphological, immunohistochemical and molecular approaches.

Malignant fibrous histiocytoma (MFH) and fibrosarcoma (FS) of bone are rare malignant tumours and contentious entities. Sixty seven cases labelled as bone MFH (57) and bone FS (10) were retrieved from five bone tumour referral centres and reviewed to determine whether recent advances allowed for reclassification and identification of histological subgroups with distinct clinical behaviour. A panel of immunostains was applied: smooth muscle actin, desmin, h-caldesmon, cytokeratin AE1-AE3, CD31, CD34, CD68, CD163, CD45, S100 and epithelial membrane antigen.

Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features.

The classification of vascular bone tumors remains challenging, with considerable morphological overlap spanning across benign to malignant categories. The vast majority of both benign and malignant vascular tumors are readily diagnosed based on their characteristic histological features, such as the formation of vascular spaces and the expression of endothelial markers. However, some vascular tumors have atypical histological features, such as a solid growth pattern, epithelioid change, or spindle cell morphology, which complicates their diagnosis.

Epithelial cell adhesion molecules and epithelial mesenchymal transition (EMT) markers in Ewing's sarcoma family of tumors (ESFTs). Do they offer any prognostic significance?

Epithelial marker and adhesion molecule expression has been reported in Ewing's sarcoma family of tumors (ESFTs), although the prognostic significance has not been assessed systematically. We performed immunohistochemical analysis of epithelial cell adhesion molecule and epithelial mesenchymal transition markers on 415 genetically confirmed ESFTs. Survival analyses were performed in 217 patients.

Diagnosis and treatment of benign notochordal cell tumors of the spine: report of 3 cases and literature review.

Study Design: Clinical case series.

Objective: To describe the clinical, radiological, and histological presentation of a series of patients presenting with benign notochordal tumors, and review the existing literature on the topic.

Summary Of Background Data: During the past decade, several authors have reported a spine tumor with benign clinical characteristics and histological resemblance to notochordal tissue.

Imaging of hibernomas: A retrospective study on twelve cases.

Background: To analyze the imaging features of hibernomas on computed tomography (CT) and magnetic resonance (MRI).

Methods: Twelve hibernomas were retrospectively assessed with CT and MR imaging and compared to the histology of the specimen

Results: Nine females and three males with a mean age of 30 years were included. Ten tumors occurred in the thigh and two affected the subcutis of the periscapular and buttock regions.

Parosteal osteosarcoma mimicking osteochondroma: A radio-histologic approach on two cases.

Background: Parosteal osteosarcoma is a well-differentiated variant of osteosarcoma that affects the surface of the bone. The imaging pattern is very typical. We report two cases mimicking an osteochondroma, radiologically and histologically and propose an explanation.

Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans.

Background: Dermatofibrosarcoma protuberans (DFSP) is a relatively common soft-tissue tumor. A more aggressive appearing fibrosarcoma may arise in DFSP, changing its biological behavior. CD34 and apolipoprotein-D are highly expressed in DFSP, but their prognostic significance is uncertain.

Neoadjuvant chemotherapy with methotrexate, cisplatin, and doxorubicin with or without ifosfamide in nonmetastatic osteosarcoma of the extremity: an Italian sarcoma group trial ISG/OS-1.

Purpose: We compared two chemotherapy regimens that included methotrexate (MTX), cisplatin (CDP), and doxorubicin (ADM) with or without ifosfamide (IFO) in patients with nonmetastatic osteosarcoma of the extremity.

Patients And Methods: Patients age ≤ 40 years randomly received regimens with the same cumulative doses of drugs (ADM 420 mg/m(2), MTX 120 g/m(2), CDP 600 mg/m(2), and IFO 30 g/m(2)) but with different durations (arm A, 44 weeks; arm B, 34 weeks). IFO was given postoperatively when pathologic response to MTX-CDP-ADM was poor (arm A) or given in the primary phase of chemotherapy with MTX-CDP-ADM (arm B).

Enchondroma vs. chondrosarcoma: a simple, easy-to-use, new magnetic resonance sign.

Introduction: There is no clear radiologic or pathologic agreement on the differences between enchondroma and conventional chondrosarcoma, which has huge therapeutic consequences. Microscopically, an enchondroma is composed of "islands of intramedullary hyaline cartilage surrounded by marrow fat", and a chondrosarcoma a "diffuse cartilaginous replacement (invasion) of the marrow which leads to complete 'trapping' of host lamellar bone trabeculae." The marrow around islands of cartilage should be detectable on magnetic resonance imaging (MR).

Atypical lipomatous tumors/well-differentiated liposarcomas: clinical outcome of 67 patients.

Atypical lipomatous tumors/well-differentiated liposarcomas are low-grade malignant mesenchymal neoplasms with high propensity to local recurrence and potential to dedifferentiate to higher grades over time. However, the published risks of local recurrence and dedifferentiation vary, and no unified treatment and follow-up plan has been accepted. We performed a study to evaluate the long-term clinical behavior and proper treatment and follow-up strategy for these tumors.

YY1 overexpression is associated with poor prognosis and metastasis-free survival in patients suffering osteosarcoma.

Background: The polycomb transcription factor Yin Yang 1 (YY1) overexpression can be causally implicated in experimental tumor growth and metastasization. To date, there is no clinical evidence of YY1 involvement in outcome of patients with osteosarcoma. Prognosis of osteosarcoma is still severe and only few patients survive beyond five years.

Receptor tyrosine kinase pathway analysis sheds light on similarities between clear-cell sarcoma and metastatic melanoma.

To highlight possible similarities and differences in receptor tyrosine kinase (RTK) and downstream signalling activation profiles between clear-cell sarcomas (CCS) and metastatic melanomas (MM), frozen, and paired-matched fixed samples of six CCS with EWSR1 rearrangement (EWSR1+), five CCS without EWSR1 rearrangement (EWSR1-), and seven MM were investigated by means of biochemical, immunohistochemical, FISH, molecular analyses, and immunofluorescence confocal microscopy. Fixed samples of a further 10 CCS and 14 MM were investigated by means of sequencing for BRAF, NRAS, and KRAS mutations and FISH analyses for the gain of chromosomes 22 and 8. RTK analysis of all CCS/MM samples showed activation of short-form (sf) recepteur d'origine nantais (RON) RTK and of PDGFRB, MET, and HER3.

miR-34a predicts survival of Ewing's sarcoma patients and directly influences cell chemo-sensitivity and malignancy.

Identification of factors to detect chemotherapy-resistant tumours at diagnosis is a first priority for risk-adapted therapy in the oncology of children and young adults, where more individualized, effective, and less toxic treatments are highly desirable. In this study, we analysed the miRNAs discriminating Ewing's sarcoma (EWS) patients with different clinical outcomes in order to identify new indicators of prognosis. miRNA expression was investigated in 49 primary EWSs by using the Agilent human miRNA microarray v.

Epithelial marker expression does not rule out a diagnosis of Ewing's sarcoma family of tumours.

Epithelial marker expression has been reported in Ewing's sarcoma family of tumors (ESFT). However, cytokeratin (CK), epithelial membrane antigen (EMA), and carcino embryonic antigen (CEA) prevalence has not been assessed thoroughly in a large series of genetically confirmed ESFT. The aim of the present study is to confirm the presence of epithelial markers in a large group of ESFT tested genetically for any of their specific gene fusions and the differential diagnosis with other small round cell tumors.

Compressive behaviour of child and adult cortical bone.

In this study, cortical bone tissue from children was investigated. It is extremely difficult to obtain human child tissue. Therefore, the only possibility was to use bone tissue, free from any lesion, collected from young bone cancer patients.

Aggressive fibromatosis of the neck treated with a combination of chemotherapy and indomethacin.

Aggressive fibromatosis (desmoid tumor) of the neck is rare. When feasible, surgery is the best treatment option. However, complete excision with negative margins is not possible in most cases because of the involvement of vascular and nervous structures.

Periosteal osteosarcoma: a single-institution experience.

Background: Periosteal osteosarcoma is a rare variant of osteosarcoma. Wide surgical removal is the mainstay of treatment, but controversy remains about the role of chemotherapy. The objective of this study was to review and analyze the clinical and treatment-related factors that influence the survival of patients with periosteal osteosarcoma who received treatment in a single institution.