How do we estimate survival? External validation of a tool for survival estimation in patients with metastatic bone disease-decision analysis and comparison of three international patient populations

How do we estimate survival? External validation of a tool for survival estimation in patients with metastatic bone disease-decision analysis and comparison of three international patient population

Trattamento del tumore a cellule giganti (TCG) del radio distale: studio multicentrico

Il radio distale \ue8 la terza sede pi\uf9 frequentemente coinvolta dal tumore a cellule giganti dell\u2019osso (TCG), tumore istologicamente benigno che nella sua forma aggressiva ha un comportamento destruente e infiltrante le parti molli. La tecnica chirurgica di scelta nel Grado 3 e in selezionati casi di Grado 2 \ue8 la resezione en-bloc della lesione. La ricostruzione del radio distale \ue8 tutt\u2019ora una sfida per le difficolt\ue0 legate alla scarsa copertura dei tessuti molli, ai rapporti di contiguit\ue0 con le strutture vascolo nervose e ai rapporti articolari con le ossa del carpo. Diverse tecniche chirurgiche ricostruttive sono state utilizzate: artrodesi con autograft o allograft, traslocazione ulnare, innesto autologo vascolarizzato o libero di perone prossimale, sostituzione protesica, artroplastica osteoarticolare con allograft di radio distale. Questo studio vuole valutare i risultati del trattamento del TCG del radio distale confrontando le diverse tecniche ricostruttive in termini di complicanze locali e risultati funzionali

Rehabilitation of a patient receiving a large-resection hip prosthesis because of a phosphaturic mesenchymal tumor

Tumor-induced osteomalacia is an osteomalacic syndrome caused by a mesenchymal origin’s tumor. The diagnostic procedure takes time and extensive investigations because of the characteristics of these tumors usually small dimensioned, slowly growing, non-invasive and therefore hard to locate. The differential diagnosis is determined by a bone biopsy. Tumor’s surgical removal is the treatment of choice that leads up to a complete regression of the oncogenic malacic syndrome. In the clinical course of these patients we can often see multiple episodes of pathological fractures, peri-prosthesis fractures or prosthesis mobilizations, due to the malacic picture: surgical procedures are often widely demolitive and requires mega-prosthetic implant. The rehabilitative procedure used to take care of these patients, is described in the following case report and based on the collaboration between surgical and rehabilitative teams. Rehabili - tative pathway after hip mega-prosthesis does not find references in medical literature: the outcomes analyzed in this case report demonstrate the efficacy of the rehabilitative procedure applied

Efficacy of trabectedin in advanced soft tissue sarcoma: Beyond lipo- and leiomyosarcoma

OBJECTIVE: Trabectedin is effective in leiomyosarcoma and liposarcoma, especially the myxoid variant, related to the presence of the FUS-CHOP transcript. We evaluated the efficacy of trabectedin in specific subgroups of patients with soft tissue sarcomas (STS). METHODS: Seventy-two patients with advanced anthracycline-pretreated STS, who received trabectedin at a dose of 1.5 mg/m(2) every 3 weeks by continuous 24-hour infusion, were retrospectively analyzed. Best response rate according to Response Evaluation Criteria In Solid Tumors (RECIST) criteria and severe adverse events (AEs) according to National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE v4.02) were evaluated. Secondary endpoints included progression-free survival and overall survival (OS). RESULTS: Median age was 48 (range, 20–75) years, with a median Eastern Cooperative Oncology Group performance status of 0. The median number of previous chemotherapy regimens was 1 (range, 0–5). Median number of trabectedin cycles was 3 (range, 1–17). About 69/72 patients (95.8%) were evaluable for response: 9 patients (13%) achieved partial response and 26 (37.7%) stable disease. According to histotype, clinical benefit (partial response + stable disease) was reported in synovial sarcoma (n=5), retroperitoneal liposarcoma (n=10), myxoid liposarcoma (n=5), leiomyosarcoma (n=8), high-grade undifferentiated pleomorphic sarcoma (n=5), Ewing/peripheral primitive neuroectodermal tumor (n=1), and malignant peripheral nerve sheath tumor (n=1). Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%), neutropenia (n=7, 9.6%), liver toxicity (n=6, 8.3%), and fatigue (n=2, 2.8%). With a median follow-up time of 11 (range, 2–23) months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively. CONCLUSION: Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and leiomyosarcoma and suggests promise in synovial sarcomas and high-grade undifferentiated pleomorphic sarcoma

MRI radiomics-based machine learning for classification of deep-seated lipoma and atypical lipomatous tumor of the extremities

Purpose: To determine diagnostic performance of MRI radiomics-based machine learning for classification of deep-seated lipoma and atypical lipomatous tumor (ALT) of the extremities. Material and methods: This retrospective study was performed at three tertiary sarcoma centers and included 150 patients with surgically treated and histology-proven lesions. The training-validation cohort consisted of 114 patients from centers 1 and 2 (n = 64 lipoma, n = 50 ALT). The external test cohort consisted of 36 patients from center 3 (n = 24 lipoma, n = 12 ALT). 3D segmentation was manually performed on T1- and T2-weighted MRI. After extraction and selection of radiomic features, three machine learning classifiers were trained and validated using nested fivefold cross-validation. The best-performing classifier according to previous analysis was evaluated and compared to an experienced musculoskeletal radiologist in the external test cohort. Results: Eight features passed feature selection and were incorporated into the machine learning models. After training and validation (74% ROC-AUC), the best-performing classifier (Random Forest) showed 92% sensitivity and 33% specificity in the external test cohort with no statistical difference compared to the radiologist (p = 0.474). Conclusion: MRI radiomics-based machine learning may classify deep-seated lipoma and ALT of the extremities with high sensitivity and negative predictive value, thus potentially serving as a non-invasive screening tool to reduce unnecessary referral to tertiary tumor centers

LA DIAGNOSI ED IL TRATTAMENTO DEL CONDROSARCOMA

La linea guida affronta l’approccio alla diagnosi e al trattamento del condrosarcoma dello scheletro degli arti e del cingolo pelvico e scapolare. Non è incluso nel presente documento il trattamento del condrosarcoma della colonna vertebrale. È stata eseguita una ricerca bibliografica su PubMed, utilizzando le parole chiave chondrosarcoma, cartilaginous tumor, chondroid tumor, selezionando studi inerenti la diagnosi ed il trattamento del condrosarcoma dello scheletro pubblicati negli ultimi 15 anni. Sono stati considerati anche alcuni studi pubblicati in precedenza per la particolare rilevanza delle casistiche. Per l’introduzione descrittiva è stato utilizzato come riferimento il manuale del professor Mario Campanacci Bone and soft tissue tumors edito da Springer Verlag nel 1999. Sono state valutate infine le pubblicazioni relative all’esperienza nel trattamento del condrosarcoma degli Istituti internazionali più accreditati nella diagnosi e cura dei tumori dell’apparato muscolo-scheletrico. Il metodo di grading delle prove utilizzato per il Progetto Linee Guida SIOT è quello descritto nel manuale metodologico del Sistema Nazionale Linee Guida (www.snlg.it) dell’Istituto Superiore di Sanità. Le raccomandazioni vengono qualificate con un livello di evidenza (da I a IV) e di forza della raccomandazione (da A a E). Nel caso del presente documento tuttavia, a causa della difficoltà o dell’impossibilità nell’eseguire studi controllati randomizzati, la letteratura di supporto è costituita integralmente da studi retrospettivi tipo caso-controllo e studi osservazionali, in particolare provenienti da Istituzioni specializzate che effettuano sistematicamente la documentazione e l’archiviazione dei casi trattati (Box 1: Studi clinici). Questo documento è stato inviato ad un panel di esperti in ambito nazionale al fine di valutare la chiarezza, la rilevanza clinica e l’applicabilità delle raccomandazioni. I revisori esterni sono i rappresentanti dei principali Centri di Riferimento Nazionali per la diagnosi e la cura dei tumori dell’apparato muscolo-scheletrico ed esperti di metodologia di ricerca clinica del GLOBE

Management of long bone metastases: recommendations from the Italian Orthopaedic Society bone metastasis study group

The purpose of this article is to outline the current approach to patients affected by metastasis to the long bones and to present a clinical and surgical algorithm available for clinicians and for future research. A modern approach to patients affected by long bone metastasis in fact requires a multidisciplinary contest where oncologists, radiotherapists, surgeons and physical therapists cooperate with a shared vision, in order to provide the best possible integrated treatments available. The authors of this article constitute the Bone Metastasis Study Group of the Italian Orthopaedic Society (SIOT): a national group of orthopedic tumor surgeons who are dedicated to studying the approach, techniques and outcomes of surgery for metastatic tumours of the musculoskeletal system. © 2014 Informa UK, Lt