Huntington procedure for the treatment of tibial nonunion in a 17-years old male: A case report.

INTRODUCTION: Bone is considered a tissue with good healing properties, and many bone defects can heal spontaneously under appropriate conditions. Extreme bone loss can hinder remodeling and regenerative processes, leading to bone nonunion. This condition negatively impacts the patient's quality of life with a severe socioeconomic burden. Many treatment options have been proposed, but none can be defined as a gold standard, mainly due to the variety of clinical presentation, bone loss, and quality. PRESENTATION OF CASE: We present a 15-year-old case of tibial nonunion following multiple traumas. The patient was treated non-surgically at the beginning, but the external fixator positioning was required due to a delay in the healing process. Following further trauma, the patient showed progressive anterolateral angulation, severe lateral procurvation, and a progressive worsening of the pseudoarthrosis. The severe bone loss and poor quality of the bone surrounding the defect required a special technique called Huntington procedure that consists in a vascularized bone autograft from the ipsilateral fibula to achieve mechanical and biological healing of the pseudoarthrosis. The patient recovered well and returned to full weight bearing without a mobility aid. DISCUSSION: We report this case of complex tibial nonunion and malalignment, developed after subsequent traumas. Due to the multiple complications, and the poor biology a Huntington procedure was required to provide mechanical stability and a biological boost to the bone defect. CONCLUSION: This case report shows a complicated case requiring several surgeries and treatment options and confirms the potential benefit of the Huntington procedure for treating a tibial severe bone loss.

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.

Multidisciplinary management of adolescents and young adults (AYA) sarcoma: A successful effort of an adult high-volume cancer center.

INTRODUCTION: The aim of this retrospective study was to investigate the clinicopathological characteristics of AYA sarcomas and their clinical outcomes at a high-volume single center. METHODS: Demographic, clinicopathological data on the diagnosis, treatment and follow-up of all sarcoma patients aged 16-39 years (ys) observed at our Institute between January 2010 and December 2021 were retrospectively collected, including diagnostic (TTD) and treatment delay(TTT), clinical outcomes (OS and PFS), and late-treatment effects. RESULTS: We identified 228 AYA patients, median age 30 years, 29% ≤ 25 years, 57% males, 88% soft tissue sarcomas (STS), and 12% bone sarcomas (BS). Among STSs, 13% were small round cell tumors (SRCT), 52% intermediate-high-grade, 24% low-grade STSs. Among BS, 32% were high-grade. Median TTD and TTT were 120 (0-8255) and 7 days (0-83), respectively. Surgery was performed in 83%, radiotherapy in 29%, and systemic therapy in 27%. Median follow-up was 72.9 months(1.6-145), 5-year and 10-year OS were 78.5% and 62%, respectively. Kaplan-Meyer analysis showed a significantly better 5-year OS and PFS for patients with >92 days of TTD (OS 85.7% vs. 66.7%, p = 0.001, PFS 50.2% vs. 24.9%, p = 0.009). According to age (≤25 years vs. > 25 years), 5-year OS was 69.8% versus 82.2%, respectively (p = 0.047). CONCLUSION: Our analysis confirmed previous data on sarcoma AYA patients followed in a referral center. Unexpectedly, diagnostic delay was not associated with poor OS and PFS. Patients <25 years showed a poorer prognosis due to the higher incidence of SRCT.

CT-guided radiofrequency ablation of painful intra-articular osteoid osteoma in children: Is there a concern for cartilage damage?

OBJECTIVE: To evaluate the safety and efficacy of computed tomography-guided percutaneous radiofrequency ablation (PRFA) in the management of uncommon and technically challenging intra-articular osteoid osteoma in children. MATERIAL AND METHODS: From December 2018 to September 2022, 16 children with intra-articular osteoid osteoma, including ten boys and six girls, were treated at two tertiary centers with percutaneous CT-guided RF ablation using a straight monopolar electrode. The procedures were carried out under general anesthesia. Post-procedural clinical outcomes and adverse events were assessed through clinical follow-up. RESULTS: Technical success was achieved in all of the participating patients. Clinical success with relief of symptomatology throughout the period of follow-up was achieved in 100% of the patients. No persistence or recurrence of pain occurred during the follow-up period. No immediate or delayed adverse effects were observed. CONCLUSION: PRFA is shown to be technically feasible. Clinical improvement can be achieved with a high rate of success in the treatment of children with difficult-to-treat intra-articular osteoid osteomas.

Treating secondary malignant neoplasms: A burden of childhood cancer survivors.

Each year approximately 35,000 children and adolescents are diagnosed with cancer in Europe. Five-year survival rates have improved and now reach 80% in most European countries, thanks to a combination of chemotherapy, radiotherapy, and surgery. To date, there are more than 44,000 Italians still living several years after being diagnosed with cancer in developmental age. The risk of premature morbidity and mortality for cancer survivors is well known and documented. Approximately 60% of survivors of cancer in childhood and adolescence have at least one chronic health condition in later life, and more than one in four develop severe or life-threatening disorders. Among the various long-term iatrogenic sequelae of cancer treatments, the most worrisome are second malignant neoplasms. We reported on our mono-institutional experiences of screening and treating secondary breast cancer, secondary thyroid cancer and secondary osteosarcoma. Recommendations on the surveillance needed for cancer survivors because of the risk of late effects of their disease or its treatment suggest that discussing the potential problems early on can be crucial to a patient's future health. These considerations and our consolidated experience strengthen our conviction that survivors of cancer in childhood and adolescence who develop second malignant neoplasms should be treated at highly-specialized centers. Multidisciplinary care requires close communications and high levels of up-to-date professional expertise. This challenging area of health care is also changing rapidly because cancer survivorship is a work in progress, but we cannot wait for definitive conclusions on many aspects because this will take decades, especially for pediatric patients.

Secondary osteosarcoma: a challenge indeed.

BACKGROUND: The risk of survivors developing a secondary bone sarcoma after being treated for pediatric cancers is well established. The aim of this study was to examine the clinical characteristics and outcomes of patients with secondary osteosarcoma (SOS). METHODS: The study concerns survivors of childhood and adolescence primary neoplasms (PN) treated with chemotherapy, with or without radiotherapy and surgery, subsequently diagnosed with SOS. RESULTS: We identified 26 patients (13 females, 13 males) who developed SOS a median 7.3 years after being diagnosed with a PN (5/7 of these patients tested for Li-Fraumeni and found positive for the syndrome). The sample's median age was 8.0 and 15.0 years when their PN and SOS were diagnosed, respectively. To treat their PN, 24 out of 26 patients had been given radiotherapy, and 19 had received chemotherapy including doxorubicin. A considerable number of SOS occurred at unfavorable sites (nine hip bone, six skull). All but one patient received chemotherapy with tailored schedules, omitting doxorubicin in 19 cases. Eighteen of the 26 patients underwent surgery. The 5- and 10-year overall survival and probabilities after the diagnosis of SOS (95% confidence interval) were 50% (32.7-76.5%) and 38.9% (22.4-67.4%); 5- and 10-year progression-free survival was 47% (29.9-73.7%) and 35.2% (19.3-64.4%), respectively. CONCLUSIONS: The survival rates after SOS are lower than in patients with primary osteosarcoma, but not negligible. It is therefore mandatory to discuss the best choice of treatment for such patients at a referral center, in terms of their chances of cure and quality of life.

Role of percutaneous CT-guided radiofrequency ablation in treatment of intra-articular, in close contact with cartilage and extra-articular osteoid osteomas: comparative analysis and new classification system.

PURPOSE: We investigated procedural safety, technical and clinical outcomes of the percutaneous image-guided radiofrequency ablation (PRFA) of intra-articular (IA), intra-articular close to cartilage (IACC), and extra-articular (EA) osteoid osteomas (OO). We proposed a new radiologic classification for osteoid osteoma depending on the degree and location of sclerosis which may correlate with technical failure and/or difficulties. MATERIAL AND METHODS: According to the inclusion criteria, we enrolled consecutive patients who were referred to the investigation center from June 2018 to January 2022. After clinical and CT imaging features were suggestive for the diagnosis of OO, all the patients were treated by percutaneous CT-guided RFA with a standardized technique. Biopsy of the lesion was not performed in all patients. A retrospective analysis was conducted to assess the procedure's technical, primary clinical, and secondary clinical successes, recurrence rate, and complications. We classified all the OOs according to a new proposed classification of the site and the amount of sclerosis. RESULTS: A total number of 55 patients were enrolled in our study according to the inclusion criteria. The mean age of the enrolled patients was 24.07 ± 14.71 years (ranges from 7 to 57 years). The M/F ratio was roughly 2:1. The mean follow-up was 20.18 ± 12.60 months (ranges from 2 to 44 months). EA group included 36 patients, IA included 5 and IACC included 14 patients. Technical success was achieved in all cases of IA and IACC groups. Technical success in the EA group was 97.22% (1 technical failure). Primary clinical success was 100%, 92.85%, and 91.66% for IA, IACC, and EA groups, respectively. Accordingly, the recurrence rate was 5.88% in EA, and 7.14% in IACC, while no recurrence occurred in the IA group. No complications occurred. The secondary success rate of the 3 cases of recurrence was 100%. CONCLUSIONS: PRFA proved to be a safe procedure with a high rate of success for OO treatment even in intra-articular lesions in close contact with cartilage. This study showed that the results in terms of technical and clinical success are comparable for IA OO, IACC OO, and EA OO, even if the recurrence rate was higher in EA OO. Our proposed new classification of the degree and location of sclerosis may correlate to technical failure, but further studies with a larger number of patients are needed for validation.

Antibacterial hydrogel coating in joint mega-prosthesis: results of a comparative series.

PURPOSE: Joint mega-prosthesis after bone tumors, severe trauma or infection is associated with high rates of post-surgical septic complications. A fast-resorbable antibacterial hydrogel coating (DAC®, Defensive Antibacterial Coating) has previously been shown to be able to significantly reduce surgical site infection in various clinical settings. Aim of the present study was to evaluate the safety and efficacy of the DAC hydrogel coating to prevent early periprosthetic joint infection after joint mega-prosthesis. METHODS: In this three-centers, case-control study, 43 patients, treated with an antibacterial hydrogel coated mega-prosthesis for oncological (N = 39) or non-oncological conditions (N = 4), were retrospectively compared with 43 matched controls, treated with mega-implants without the coating. Clinical, laboratory and radiographic examinations were performed to evaluate the occurrence of post-surgical infection, complications and adverse events. RESULTS: At a mean follow-up of 2 years, no evidence of infection or adverse events were observed in the DAC-treated group, compared to six cases of post-surgical infection in the control group. CONCLUSION: This matched case-control study shows that a fast-resorbable, antibiotic-loaded coating can be safely used to protect joint mega-prosthesis, providing a reduction of early surgical site infections with no side effects. Larger prospective trials with longer follow-ups are warranted to confirm this report. TRIAL REGISTRATION: RS1229/19 (Regina Elena National Cancer Institute Experimental Registry Number).

Expression profiling of microRNAs and isomiRs in conventional central chondrosarcoma.

Conventional central chondrosarcoma (CCC) is a malignant bone tumor that is characterized by the production of chondroid tissue. Since radiation therapy and chemotherapy have limited effects on CCC, treatment of most patients depends on surgical resection. This study aimed to identify the expression profiles of microRNAs (miRNAs) and isomiRs in CCC tissues to highlight their possible participation to the regulation of pathways critical for the formation and growth of this type of tumor. Our study analyzed miRNAs and isomiRs from Grade I (GI), Grade II (GII), and Grade III (GIII) histologically validated CCC tissue samples. While the different histological grades shared a similar expression profile for the top abundant miRNAs, we found several microRNAs and isomiRs showing a strong different modulation in GII + GIII vs GI grade samples and their involvement in tumor biology could be consistently hypothesized. We then in silico validated these differently expressed miRNAs in a larger chondrosarcoma public dataset and confirmed the expression trend for 17 out of 34 miRNAs. Our results clearly suggests that the contribution of miRNA deregulation, and their targeted pathways, to the progression of CCC could be relevant and strongly indicates that when studying miRNA deregulation in tumors, not only the canonical miRNAs, but the whole set of corresponding isomiRs should be taken in account. Improving understanding of the precise roles of miRNAs and isomiRs over the course of central chondrosarcoma progression could help identifying possible targets for precision medicine therapeutic intervention.

Fibulo-scapho-lunate fusion after resection of the distal radius: Case series, review of the literature and critical analysis of bone fixation.

Fibulo-scapho-lunate fusion is a technique that allows residual movement in the wrist in case of wide bone resection replacing the distal radius by a vascularised fibular transfer. Some authors have used this technique with favourable results but the distal synthesis seems to not be standardised at all, many different osteosynthesis methods have been proposed. This paper reports a complete review of the present day literature about this subject and, evaluating the different proposed osteosynthesis techniques referred in literature, suggests a standardization of the synthesis methods with dorsal plating. We report some technical considerations and results of three cases operated with a stable dorsal osteosynthesis (twice with a double plate and once with a long plate). We evaluate the time of healing and the clinical result.

Extensor hallucis longus tendon and soft-tissue reconstruction with palmaris longus tendon included in a radial forearm free flap: A case report.

Reconstruction of foot structures is challenging because of the need to restore both anatomy and function. Extensor hallucis longus (EHL) excision without reconstruction could result in a flexion deformity of the toe at the interphalangeal joint. In this report, we present a case of the use of a palmaris longus tendon included in a fasciocutaneous radial forearm free flap to reconstruct EHL tendon and soft tissues of the distal foot dorsum. A 41-year-old woman presented with a recurrence of myxoinflammatory fibroblastic sarcoma on the medial part of the left foot dorsum. The EHL tendon was sacrificed, leaving a soft-tissue defect of 14 × 5 cm and 14-cm tendon gap. A 14 × 5 cm radial forearm flap with a 16-cm section of palmaris longus tendon was harvested from the left forearm. Radial artery and its comitant vein were anastomosed with dorsalis pedis artery and vein. EHL tendon repair was performed from the composite mass to the proximal and distal stumps. No complication in the postoperative period occurred. At latest follow-up, 12 months postoperatively, the patient showed a normal joint function and was satisfied with the treatment. Palmaris longus tendon included in a radial forearm free flap could be recommended for combined soft tissue and tendon defects, particularly on the foot dorsum.

Multicentric, multifocal, and recurrent osteoid osteoma of the hip: first case report.

BACKGROUND: Osteoid osteoma is a benign bone-forming tumour, which very unfrequently has multifocal or multicentric presentation. We report the first known case of a multicentric, multifocal and recurrent osteoid osteoma treated using radiofrequency ablation. CASE PRESENTATION: A 39-year-old man with two-year history of left hip pain was admitted at our Institution. The pain was more intense during the night and partially relieved by salicylates. Pelvis CT demonstrated two lytic lesions (8 and 7 mm, respectively) with surrounding sclerotic reactive bone, both with a central focal area of high attenuation, located in the femoral neck and along the anterior portion of the acetabulum, respectively. Both lesions had clinical and imaging findings consistent with multicentric osteoid osteoma. Thus, the two lesions were biopsied - with pathologic confirmation of osteoid osteoma - and treated using radiofrequency ablation. Hip pain decreased but did not disappear, actually increasing a few months after treatment. CT and MRI were performed showing a smaller lesion (5 mm) with the same imaging features, surrounded by marrow oedema, along the posterior column of the acetabulum. The lesion was considered suspicious for osteoid osteoma, overlooked on previous examinations. Therefore, a diagnosis of multicentric and multifocal osteoid osteoma was established. The new lesion was again treated with radiofrequency ablation with symptom disappearance. However, hip pain relapsed after 18 months, and CT and MRI showed an osteoid osteoma recurrence on the posterior column of the acetabulum, which was biopsied and successfully treated using radiofrequency ablation. CONCLUSIONS: To our knowledge, this is the first reported case of multicentric, multifocal, recurrent osteoid osteoma. Our case report highlights the importance of considering a diagnosis of multifocal osteoid osteoma when dealing with multifocal lytic lesions of the bone and with pain persistence after treatment. It also emphasises the combined role of CT and MRI in this setting.

Calcaneal reconstruction after total calcanectomy with iliac crest free flap.

INTRODUCTION: Calcaneal tumors being a rare occurrence, surgical options and outcomes are not well-known. Extensive defects following wide resection, especially in weight-bearing areas, still remain a challenge and different reconstructive techniques have been proposed. The aim of this report was to analyze the clinical and functional long-term outcomes of heel reconstruction using an iliac crest free flap. PATIENTS AND METHODS: Four patients who underwent calcaneal reconstruction between 1999 and 2012 were included. Two were females and mean age was 27 years, ranging 18-42 years. Each patient underwent total calcanectomy, for two osteoblastomas, one osteosarcoma, and one Ewing's sarcoma. An iliac crest flap was harvested and shaped to fit the residual space. After the articular cartilage at recipient site was debrided, the flap was fixed to the talus and the cuboid. RESULTS: The average size of the flaps was 2 x 7 x 5 cm. Postoperatively wound dehiscence, screw breaking, and graft fracture healed conservatively. All the arthrodesis healed successfully and no donor site complication occurred. At an average follow-up of 13 years (range 6-19 years) any patient claimed pain, evident limp or limitation of daily activities. Computerized pedobarographic examination and gait analysis revealed a satisfactory result and an acceptable weight-bearing area in the reconstructed limb in each patient. CONCLUSIONS: Calcaneal reconstruction with iliac crest free flap is likely to provide good chances of a long-lasting result, especially in young patients. Particularly, it provides the possibility to adequately shape the graft to fit the bone loss while using the crest as the weight-bearing surface.

Atypical diaphyseal femoral fractures: Considerations on surgical technique.

In the last two decades bisphosphonates (BP) has become the mainstream therapy for osteoporosis and the benefit in reducing the incidence of fractures has been well demonstrated in several studies, together with the drug long term (5-10 years) efficacy and safety. A complication of the long-term use of bisphosphonates is a low-energy stress fractures located in the sub-trochanteric region and the femoral shaft called atypical femur fracture (AFF). These stress fractures can be seen on plain radiographs as simple transverse patterns, with unicortical beaking and hypertrophy of the diaphyseal lateral cortex. Usually a localized thigh pain in the prodromal phase may precede the fracture by months. The current surgical treatment of choice for AFFs is intramedullary nailing (IMN). However, the treatment of AFFs is associated with a higher rate of intra-operative and post-surgical complications. This is related to anatomical e biomechanical reasons. Iatrogenic fractures, deformities, medial gap opening, eccentric position of the distal nail tip with anterior cortex perforation, delayed or non-union are frequent complications of this procedure and healing rate of AFFs. The average healing time of almost 8 months for AFFs appeared to be longer than that for typical femoral fractures, which heal at an average of 3-6 months. The purpose of this study is to analyze the different surgical devices and techniques and to advance some considerations that can be useful to diminish the rate of failure and/or complications in the treatment of AFFs in both oncologic and osteoporotic patients.

Primary metastatic osteosarcoma: results of a prospective study in children given chemotherapy and interleukin-2.

To improve the poor prognosis for children with metastatic osteosarcoma (OS), interleukin-2 (IL-2) was added to the standard treatment due to its capacity to activate lymphocytes and differentiate lymphocyte subsets into lymphokine-activated killer (LAK) cells that are capable of recognizing and killing various tumor cells. This study concerns a cohort of unselected patients aged < 18 years with metastatic OS, who were treated with IL-2, high-dose methotrexate, doxorubicin, cisplatin, ifosfamide, LAK reinfusion, and surgery, between 1995 and 2010. Thirty-five patients aged 4-17 years were involved. Thirty-two of the 35 patients underwent surgery on their primary tumor, and 25 had surgery on lung metastases too. Twenty-seven patients received IL-2 plus LAK reinfusion. The median follow-up was 130 months (77-228), and the 3-year event-free and overall survival rates were 34.3 and 45.0%, respectively. Eleven patients remained alive, all of whom achieved a complete surgical removal of the primary tumor and lung metastases (1 patient did not receive lung resections due to complete lung metastases remission). Patients who had a complete surgical remission of the primary and metastatic sites and who responded well to chemotherapy had a better event-free survival. These results confirm the importance of complete surgical remission and point to a noteworthy (though still be ameliorate) survival rate in our series of patients, underling a potential role for immunotherapy with IL-2 and LAK/NK cell activation.

Ewing's Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue.

The molecular mechanism responsible for Ewing's Sarcoma (ES) remains largely unknown. MicroRNAs (miRNAs), a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs) by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis.

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.

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.

BACKGROUND: We recently developed a clinical decision support tool, capable of estimating the likelihood of survival at 3 and 12 months following surgery for patients with operable skeletal metastases. After making it publicly available on www.PATHFx.org , we attempted to externally validate it using independent, international data. METHODS: We collected data from patients treated at 13 Italian orthopaedic oncology referral centers between 2010 and 2013, then applied to PATHFx, which generated a probability of survival at three and 12-months for each patient. We assessed accuracy using the area under the receiver-operating characteristic curve (AUC), clinical utility using Decision Curve Analysis (DCA), and compared the Italian patient data to the training set (United States) and first external validation set (Scandinavia). RESULTS: The Italian dataset contained 287 records with at least 12 months follow-up information. The AUCs for the three-month and 12-month estimates was 0.80 and 0.77, respectively. There were missing data, including the surgeon's estimate of survival that was missing in the majority of records. Physiologically, Italian patients were similar to patients in the training and first validation sets. However notable differences were observed in the proportion of those surviving three and 12-months, suggesting differences in referral patterns and perhaps indications for surgery. CONCLUSIONS: PATHFx was successfully validated in an Italian dataset containing missing data. This study demonstrates its broad applicability to European patients, even in centers with differing treatment philosophies from those previously studied.

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. Rehabilitative 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.