Clinical activity of mTOR inhibition in combination with cyclophosphamide in the treatment of recurrent unresectable chondrosarcomas.

Objective: Chondrosarcomas (CS) represent a heterogeneous group of rare sarcomas, poorly responsive to chemotherapy or radiotherapy. When local therapies in recurrent or metastatic disease are exhausted, chemotherapy plays a marginal role. Different molecular pathways have been shown to be activated in CS.

Biological reconstruction of bone defects: the role of the free fibula flap.

This review describes the kinds of skeletal bone defects in bones which develop through enchondral ossification. It focuses on the biological reconstruction of those defects according to the two main subtypes, intercalary and osteoarticular. We list the causes of bone defects and outline the different types and configurations that result from them.

Expandable endoprosthesis for limb-sparing surgery in children: long-term results.

Purpose: Most children today with bone sarcomas undergo limb-sparing surgery. When treating children younger than 12 years of age, the result is significant limb length discrepancy (LLD). One of the solutions is the use of an expandable endoprosthesis.

Pre-clinical and clinical significance of heparanase in Ewing's sarcoma.

Heparanase is an endoglycosidase that specifically cleaves heparan sulphate side chains of heparan sulphate proteoglycans, activity that is strongly implicated in cell migration and invasion associated with tumour metastasis, angiogenesis and inflammation. Heparanase up-regulation was documented in an increasing number of human carcinomas, correlating with reduced post-operative survival rate and enhanced tumour angiogenesis. Expression and significance of heparanase in human sarcomas has not been so far reported.

Communication skills, working environment and burnout among oncology nurses.

Purpose: To examine the association between communication self-efficacy, working environment perceptions and burnout in an Israeli sample of oncology nurses.

Methods: A non-randomized convenience sample of nurses (n=39) was recruited from six oncology units in a major tertiary medical center in Israel. Measurements included a socio-demographic survey, the Maslach Burnout Inventory, a communication skills self-efficacy inventory and the Working Environment Scale.

Radiation therapy for palliation of sarcoma metastases: a unique and uniform hypofractionation experience.

Radiotherapy (RT) is our preferred modality for local palliation of metastatic soft tissue sarcoma (STS). A short and intense course of RT is usually needed for rapid palliation and local control of metastatic disease. Seventeen patients at a median age of 61 had symptomatic metastatic sarcoma and required rapid palliation.

Aberrant methylation and reduced expression of RASSF1A in Ewing sarcoma.

Background: Ewing sarcoma (ES) is the second most common solid bone and soft tissue malignancy in children and young adults with low cure rates indicating the need to identify further prognostic markers. The importance of methylation in the inactivation of key tumor suppressor genes including RASSF1A has begun to be appreciated in context of cancer development, prognosis and therapy. However there is lack of similar broad based studies in ES.

Expression and significance of EGFR in malignant peripheral nerve sheath tumor.

Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma. Epidermal growth factor receptor (EGFR) may play a putative role in its pathogenesis, and be targeted for therapeutic purposes. The study was aimed at investigating the expression and prognostic influence of EGFR in MPNST.

The co-occurrence of breast cancer and soft tissue sarcoma in a single cohort series.

Background: The incidence of breast cancer (BC) and soft tissue sarcoma (STS) in the Israeli general population is 97/10 women and 1.5/10 persons. It is expected that 1.

Subanaesthetic ketamine spares postoperative morphine and controls pain better than standard morphine does alone in orthopaedic-oncological patients.

Background: Postoperative pain in patients with bone and soft tissue cancer is different from that of other surgical patients due to the severity of the pain generated during surgery and because many of them have already been in pain preoperatively. The search for optimal intravenous pharmacologic management for this population is an ongoing one. We conducted a 10-month prospective, randomised, double blind study to compare the effects of a standard morphine dose to a 35%-lower dose plus a subanaesthetic dose of ketamine for postoperative pain control in patients undergoing bone and soft tissue cancer surgery under standardised general anaesthesia.

Unacceptable complications following intra-articular injection of yttrium 90 in the ankle joint for diffuse pigmented villonodular synovitis.

Background: Simple resection of diffuse pigmented villonodular synovitis of the ankle joint is associated with local recurrence rates as high as 50%. Thus, adjuvant treatment modalities, such as radiation or intra-articular isotope injection, are sometimes used after tumor resection. Our initial and highly satisfactory experience with the injection of radioactive yttrium 90 to treat pigmented villonodular synovitis of the ankle joint eroded with time so much so that we discontinued its use in the ankle and believe that it is important to alert our colleagues to the complications that we observed.

Short telomeres: a novel potential predictor of relapse in Ewing sarcoma.

Purpose: Despite advances in therapy, >50% of patients with Ewing sarcoma will relapse. The current prognostic factors are not optimal for risk prediction. Studies have shown that telomere length could predict outcome in different malignancies.

The complexity of management of pregnancy-associated malignant soft tissue and bone tumors.

Objective: The incidence of musculoskeletal tumors during pregnancy is very low. The aim of this study was to summarize our experience in treating a large cohort of pregnant patients diagnosed with these rare tumors.

Methods: Women diagnosed with musculoskeletal tumors during pregnancy or immediately after delivery were identified retrospectively in our database between 1996 and 2006.

FGFR1 over-expression in primary rhabdomyosarcoma tumors is associated with hypomethylation of a 5' CpG island and abnormal expression of the AKT1, NOG, and BMP4 genes.

Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma likely results from abnormal proliferation and differentiation during skeletal myogenesis. Multiple genetic alterations are associated with the three RMS histopathological subtypes, embryonal, alveolar, and pleomorphic adult variant. Recently, we reported the novel amplification of the FGFR1 gene in a RMS tumor.

Large tumors of the axilla: limb-sparing resection versus amputation in 27 patients.

Tumors of the axilla are rare and pose a surgical challenge because they are usually large at presentation and in close proximity to the major neurovascular bundle of the upper extremity. The use of detailed preoperative evaluation studies and extensile surgical exposure for these tumors enabled us to determine tumor resectability and proceed with a safe resection or perform an amputation when required. We retrospectively reviewed 27 patients who underwent resection of an axillary tumor from 1989 to 2004 and analyzed their presenting symptoms, results of preoperative studies, type of surgery, and functional outcome.

Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumors.

Rhabdomyosarcoma, the most common pediatric soft tissue sarcoma, likely results from deregulation of the skeletal myogenesis program. Although associations between PAX3, PAX7, FOXO1A, and RMS tumorigenesis are well recognized, the entire spectrum of genetic factors underlying RMS development and progression is unclear. Using a combined approach of spectral karyotyping, array-based comparative genomic hybridization (CGH), and expression analysis, we examined 10 primary RMS tumors, including embryonal, alveolar, and the rare adult pleomorphic variant, to explore the involvement of different genes and genetic pathways in RMS tumorigenesis.

Free fibula flap elbow-joint hemiarthroplasty reconstruction for chronic osteomyelitis of the distal humerus.

The vascularized fibula flap has become a major tool in upper limb reconstruction. Free fibula flap reconstructions of the humeral part of the shoulder and the radial part of the wrist joints are well-documented, but reports of elbow joint reconstruction are rare. The authors report a 53-year-old patient with chronic osteomyelitis of the distal humerus that was unsuccessfully treated by many local surgical debridements and long-term systemic antibiotics.

Vacuum-assisted wound closure after resection of musculoskeletal tumors.

Unlabelled: Resection of musculoskeletal tumors may result in large soft tissue defects that cannot be closed primarily and require prolonged dressing changes and complex surgical interventions for wound coverage. We retrospectively reviewed 23 patients with such defects treated with a vacuum-assisted wound closure system and compared the outcome of these patients with a control group. The study group included 15 women and eight men who had their wounds located at the back (two), pelvic girdle (11), thigh (eight), and leg (two).

Limb sparing approach: adjuvant radiation therapy in adults with intermediate or high-grade limb soft tissue sarcoma.

Background: Limb soft tissue sarcomas (STS) are currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT).

Patients And Methods: Between October 1994 and October 2002, 133 adult patients with intermediate or high-grade limb STS were approached by LSS+RT.

Results: RT related toxicity was manageable, with a low rate of severe effects.

A single-team experience of limb sparing approach in adults with high-grade malignant fibrous histiocytoma.

Malignant fibrous histiocytoma (MFH) is the most common subtype of soft-tissue sarcoma (STS). When located in a limb, MFH, is currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT). During 8 years, 42 adult patients with high-grade limb MFH were approached by LSS and RT.

Function after resection of humeral metastases: analysis of 59 consecutive patients.

Unlabelled: Metastatic bone disease of the humerus may require surgery for treatment of an impending or existing pathologic fracture or for alleviating disabling pain. Prompt restoration of function is a main goal of surgery, although published results do not reveal if that goal is being met. We retrospectively reviewed range of motion and function of 59 patients operated on from 1986-2003 for those indications.

Prediction of high risk Ewing's sarcoma by gene expression profiling.

Ewing's sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. Currently accepted clinical prognostic factors fail to classify ES patients' risk to relapse at diagnosis. We aimed to find a new strategy to distinguish between poor and good prognosis ES patients already at diagnosis.

Free fibula long bone reconstruction in orthopedic oncology: a surgical algorithm for reconstructive options.

The fibula free flap became popular in orthopedic oncology for limb-sparing long bone tumor resection. It is particularly suitable for intercalary or resection arthrodesis options. In the present series, a surgical reconstruction algorithm was used, enabling each patient to receive a personalized technique.