Osteopoikilosis: Pain as a Presenting Symptom in Three Family Members

Osteopoikilosis is a rare asymptomatic sclerosing bony dysplasia of benign origin. It is usually found incidentally on radiological examinations. Familial occurrence indicates a genetic milieu with autosomal dominant pattern. Here, we present a case report of a young woman suffering from pelvic pain due to osteopoikilosis (OPK). The same disorder was later found in her son and daughter

Ten-year follow-up of a patient with metastatic Ewing's sarcoma of the pelvis.

PATIENT: We report a 32-year-old women with a pelvic Ewing's sarcoma, who developed skeletal metastases within 20 months of diagnosis but following treatment remains disease-free at 10 years. DISCUSSION: Ewing's sarcoma is a highly malignant tumour of bone. Presentation of a pelvic tumour over the age of 30 years is extremely rare and associated with a poor prognosis. Early recurrence is also associated with an extremely grave prognosis. In our patient there was little or no response to salvage chemotherapy, but, against the odds, remission was induced with pamidronate and palliative radiotherapy to some but not all sites of disease.This remission has been maintained without additional therapy for a further 5 years

Non-Metastatic Pelvic Ewing's Sarcoma: Oncologic outcomes and evaluation of prognostic factors

We are reporting our experience on patients with pelvic Ewing's Sarcoma treated in our unit. We retrospectively reviewed a series of patients with non-metastatic pelvic Ewing's sarcoma treated between 1977 and 2009. Patients were classifed into three groups according to the local treatment received: Group 1. radiotherapy-chemo; Group 2. surgery-chemo and Group 3. radiotherapy-surgery-chemo. Recurrence free and overall survival rates were calculated using the Kaplan-Meier method. In?uence of various factors (age at diagnosis, gender, tumour site and size, chemotherapy response, surgical margins and type of treatment) on survival was assessed with a logistic regression model. A total of 85 patients were treated with a mean follow-up of 65.8 months and mean tumour volume of 435ml. The 5-year survival for all patients was 40.7% decreased to 36.2% at 10 years. A signifcant prognostic factor identifed was chemotherapy response only. There was a trend for improved survival and local control rates for patients who had chemotherapy and surgery and the results were apparent for all tumours irrespective of size but not statistically signifcant. Currently, the optimal management of pelvic Ewing's sarcoma is controversial but our study shows a trend for improved survival for patients treated with chemotherapy and surgery. © 2016, Acta Orthopedica Belgica

Translation and preliminary validation of the english version of the DUX questionnaire for lower extremity bone tumor patients (Bt-DUX): A disease-specific measure for quality of life

Optimising joint reconstruction management in arthritis and bone tumour patient

Survival after recurrent osteosarcoma: data from 3 European Osteosarcoma Intergroup (EOI) randomized controlled trials

BACKGROUND Recurrence after osteosarcoma usually leads to death; thus prognostic factors for survival are of great importance. METHODS Between 1983 and 2002, the European Osteosarcoma Intergroup accrued 1067 patients to 3 randomized controlled trials of pre- and post-operative chemotherapy for patients with resectable non-metastatic high-grade osteosarcoma of the extremity. Control treatment in all trials was doxorubicin 75 mg/m² and cisplatin 100mg/m². The comparators were additional high-dose methotrexate (BO02), T10-based multi-drug regimen (BO03) and G-CSF intensified-DC (BO06). Post-recurrence survival (PRS) was investigated on combined data with standard survival analysis methods. RESULTS Median recurrence-free survival was 31 months; 8 recurrences were reported more than 5 years after the diagnosis. In 564 patients with a recurrence (median 13 months post-randomisation), there was no difference in post-relapse survival between treatment arms. Patients whose disease recurred within 2 years after randomization had a worse prognosis than those recurring after 2 years. Patients with good initial histological response to pre-operative chemotherapy had a better overall survival after recurrence than poor responders. Local relapse was more often reported after limb-saving procedures (2 versus 8%; amputation versus limb-saving), independent of the primary tumour site. Site of first recurrence (local 20%, lung 62%, "other" 19%) affected survival, as patients recurring with non-lung distant metastases only or any combination of local relapse, lung metastases and non-lung metastases (=group "other") had significantly worse overall survival (local 39%, lung 19%, "other" 9% at 5 years). CONCLUSIONS These data describing a large series of patients with recurrent extremity osteosarcoma confirm the relationship between early recurrence and poor survival. There was better PRS in patients after good histological response to pre-operative chemotherapy, or with local-only recurrence

Survival from high-grade localised extremity osteosarcoma: combined results and prognostic factors from three European Osteosarcoma Intergroup randomised controlled trials

Optimising joint reconstruction management in arthritis and bone tumour patient