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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).The study concerns survivors of childhood and adolescence primary neoplasms (PN) treated with chemotherapy, with or without radiotherapy and surgery, subsequently diagnosed with SOS.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.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.

Giant-cell tumor (GCT) is locally aggressive bone neoplasm, with an unpredictable pattern of biological aggressiveness. The optimal treatment had to achieve a negligible local recurrence rate while maximizing musculoskeletal function. Numerous options for reconstruction are available, but in the literature there is a lack of salvage surgery data. We present a case of a 67-year-old woman who underwent complete wrist arthrodesis with vascularized fibular graft as salvage procedure for allograft necrosis, after excision of a distal radius GCT. The patient did not complain of any impairment in daily use, and the functional score was 22 points (73%) at latest follow-up of 14 months. Despite joint salvage remains the most favorable treatment with regard to functional outcome for aggressive tumors of the distal radius, vascularized fibular grafts is a valuable alternative especially in salvage procedures, where the use of another allograft could lead to higher complications rate. Keywords: Vascularized fibular graft, Wrist arthrodesis, Giant-Cell Tumor, Fibula free flap.

INTRODUCTION Pelvic ring reconstruction after internal hemipelvectomy is an extremely challenging surgical procedure, and mandatory reconstruction is to provide a durable and pain-free functional outcome, especially for young, active patients. One of the most widely employed techniques is reconstruction with a double-barreled fibular free flap (DBF). The aim of our work was an in-depth analysis of the outcome of pelvic ring reconstruction performed using the above-mentioned method, in particular looking for a correlation between the fixation technique and either ambulation status or complications. MATERIALS AND METHODS A systematic review was performed in November 2020 using PubMed and MedLine Ovid databases according to the PRISMA guidelines and the results were statistically analyzed. RESULTS Studies were published between 1994 and 2015. A DBF was used in each case, with a total of 30 patients. Reconstruction was performed with screws in 13 cases, Cotrel-Dubousset rod fixation in 6, screws and plate in 5, screws with external fixation in 4, and ISOLA in 2. Follow-up time ranged from 3 to 131 months. Functional outcome was excellent in 4 patients and good in 26 patients. Statistical analysis showed no statistical evidence of existing correlation between fixation technique and complications (p = .873), while statistical correlation between age and fixation technique was found (p

Paget's disease is characterized by disorganized bony tissue due to altered equilibrium of bone formation and resorption. Its malignant transformation is well known and is usually described to an osteosarcoma, often with bad prognosis. In this paper, we present a case clinic of an angiosarcoma from a Paget’s disease of a pelvic bone. The patient underwent a surgical procedure of tumor resection with wide margins (Enneking zone 2-3). The follow-up of 5 years in this case is unique in the literature.

Introduction: Osteosarcoma is the most common malignant bone tumor. It is currently treated with pre-and postoperative chemotherapy, associated with surgical resection of the tumor.Area covered: Re...

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.

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.

Il radio distale è la terza sede più frequentemente coinvolta dal tumore a cellule giganti dell’osso (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 è la resezione en-bloc della lesione. La ricostruzione del radio distale è tutt’ora una sfida per le difficoltà legate alla scarsa copertura dei tessuti molli, ai rapporti di contiguità 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.

Prompt evaluation and effective treatment of long bone metastasis are a priority in the management of cancer patients. The main goals are to achieve local tumor control, pain relief, prevention and treatment of fractures, and maintenance of patient independence and quality of life.

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

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).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).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.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.

Rita De Sanctis,1 Andrea Marrari,1 Silvia Marchetti,1 Chiara Mussi,2 Luca Balzarini,3 Fabio Romano Lutman,3 Primo Daolio,4 Stefano Bastoni,4 Alexia Francesca Bertuzzi,1,5 Vittorio Quagliuolo,2 Armando Santoro1 1Department of Medical Oncology and Haematology, 2Department of Surgical Oncology, 3Department of Radiology, Humanitas Cancer Center, IRCCS, Rozzano, 4Department of Surgical Oncology, Orthopaedic Institute “G.Pini”, Milan, Italy; 5Department of Medical Oncology, Adelaide and Meath Hospital, Incorporating the National Children’s Hospital (AMNCH), Dublin,Ireland 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/m2 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. Keywords: trabectedin, soft tissue sarcoma, liposarcoma, leiomyosarcomaCorrigendumfor this paper has been published

The knee is the most frequent location for osteosarcoma (60%). Until 30 years ago patients with osteosarcoma were treated with an amputation of the affected limb; today 85% of these cases are treated with a "Limb Salvage" procedure, through replacement of the affected bone with a mega prosthesis implant.To measure functional recovery of patients with a knee mega-prosthesis and how rehabilitation impacts on quality of life (QOL); another aim is to define rehabilitation guidelines to manage these patients in the rehabilitation pathway.A sample of outpatients with knee mega-prosthesis were selected in the follow-up orthopedic oncology surgery between march and may 2013. We measured the functional outcome using the multidimensional standardized Musculoskeletal Tumor Society scale (MSTS) that gives a score (0-5) for each of 7 items: pain, range of motion, stability, deformity, strength, functional activity, emotional acceptance.The sample included 28 outpatients. The median MSTS score was 29 (min 19, max 33) equal to 83%. Only 14 patients (50%) followed a correct rehabilitation path after surgery.21 cases got "excellent" outcomes and 3 "good" (based on MSTS classification). In few cases (n 4) the outcome was "fairly good", related to necessity of a prosthesis surgical revision based on instability of implant (because of usage). Quadricep strength is the item that most affects the final score. There is a statistically significant correlation between high scores and patients who have recently undergone surgery. We found higher scores in patients that followed a physiotherapy program after surgery: the difference was statistically significant.Mega-prosthesis implants give satisfactory functional outcomes. These outcomes keep high score in time. It is recommended to follow the guidelines described in the purposed rehabilitation program.

To identify the best surgical approach to atypical lipomatous tumors we reviewed 171 patients who underwent surgery at two sarcoma referral centers with different surgical policies. Of the 151 patients (88 %) with primary tumors, 95 were treated at Institution A and 76 were treated at Institution B. At Institution A, a wide surgical resection, including a slight cuff of soft tissue around the mass, was adopted, which was defined as marginal resection (MR) according to the Enneking classification. At Institution B, a simple tumor resection (SR), according to the Enneking classification, was employed. En bloc surgical resection was the goal in both centers. The primary outcomes of the study were local recurrence-free survival (LRFS), incidence of secondary dedifferentiation at recurrence, and presence of residual tumor after re-excision. Sixteen patients (9 %) had local recurrence. The 10-year LRFS was 82 %. No cases of secondary dedifferentiation were observed. Residual tumor after re-excision was found in 46 % of cases. In univariate analysis, sclerosing subtype, tumor rupture, and SR were unfavorable prognostic factors for LRFS. Sclerosing subtype and tumor rupture were independent prognostic factors for LRFS in multivariate analysis. SR was significantly associated with tumor rupture. Sclerosing subtype and tumor rupture are unfavorable prognostic factors for local recurrence. MR is associated with a lower risk of tumor rupture than SR. Neurovascular and major muscle resections are not necessary in principle. Re-excision after unplanned surgery is not always mandatory. A preoperative core needle biopsy could be useful in identifying the sclerosing subtype.