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Chordomas are very rare bone malignant tumours that have had a shortage of effective treatments for a long time. New treatments are now available for both the local and the metastatic phase of the disease, but the degree of uncertainty in selecting the most appropriate treatment remains high and their adoption remains inconsistent across the world, resulting in suboptimum outcomes for many patients. In December, 2013, the European Society for Medical Oncology (ESMO) convened a consensus meeting to update its clinical practice guidelines on sarcomas. ESMO also hosted a parallel consensus meeting on chordoma that included more than 40 chordoma experts from several disciplines and from both sides of the Atlantic, with the contribution and sponsorship of the Chordoma Foundation, a global patient advocacy group. The consensus reached at that meeting is shown in this position paper.

An allogeneic irradiated RCC cell line, engineered to produce IL-2 (ACHN-IL-2), admixed with autologous metastatic formalin-treated tumour cells, was used to vaccinate ten MRCC patients in progression of disease in spite of IL-2 immunotherapy. The cells were administered subcutaneously and/or intra-tumourally. Sixty-four MRCC patients in progressive disease, not treated by vaccination but receiving similar IL-2 immunotherapy, were considered as the control group. Patients received 4-16 injections (mean 9 +/- 4), containing an average of 10.6 x 10(7) +/- 7.7 x 10(7) ACHN-IL-2-transfected cells (a minimum of 4 x 10(7), and a maximum of 31 x 10(7)). Four patients also received intra-tumour injections. Vaccination was administered during 30-418 days, and the follow-up continued for 649 +/- 353 days (190-1342). Throughout this period, the patients continued receiving the previously set immunotherapy treatment. No adverse side effects related to the treatment were observed. One complete and one partial tumour response were observed, as well as two stable and one no-relapse disease. All but one patient died. Responding patients resumed progression in 4-11 months and died 18 and 36 months after beginning the vaccine therapy. In spite of the small number of treated patients, Wilcoxon's test showed a significant (P0.05) improvement of the survival in the vaccinated group compared to that of the control. The described vaccination protocol seems safe, devoid of adverse side effects and promising. It warrants further investigation.

Study Design. A prospective, international, multicenter study of 400 patients who received the BWM fixator system. Objectives. To assess the effectiveness and safety of the system in the management of various conditions requiring spinal fixation and bone grafting. Summary of Background Data. The BWM system was developed for the management of spinal instability of all etiologies occurring in the thoracic, thoracolumbar, and lumbosacral spine. Methods. Patients with fracture, tumor, spondylolisthesis, spondylitis, failed back, or other degenerative conditions of the spine received the BWM instrumentation as described in the study literature and were regularly reviewed for 2 years. Results. The results from the first 200 patients to complete the study showed an overall graft fusion rate of 94% (95% confidence interval: 91.3%-97.6%). There were marked improvements in measures of functional ability (P

Fractures of the lower part of the humerus in adults

Antibiotic prophylaxis in orthopedic surgery: considerations on a short experience

Spinal metastasis: experience in diagnosis and treatment at the Rizzoli Institute

Myoepithelioma is a very rare tumour. This tumor type has been reported in the soft tissue, ear, sinonasal cavity, breast and lung. Although rare, myoepithelioma can occur in bone. We present the first case of myoepithelioma in the spine, documenting the clinical, radiographic and pathological features.

Minimally invasive spine surgery has gained a great consent in the treatment of vertebral osteoporotic fractures. We perform a retrospective clinical and radiographic review on 32 consecutive patients (22 female and 10 male) surgically treated for a thoracolumbar osteoporotic fracture (type A) by a minimally invasive system. By this study, we propose to determine the safety and efficacy of an expandable, percutaneous, minimally invasive technique to reduce the disability caused by vertebral osteoporotic fractures.We retrospectively reviewed 32 patients who were operated on between 2003 and 2004 by means of an innovative technique which employs an expandable system inserted by a minimally invasive approach into the vertebral body. Average age at surgery was 64.8 years (range, 27-82). All patients were mobilized in first post-operative day with no external immobilization and discharged from the Hospital in the second post-operative day.This innovative technique which employs an expandable system inserted by a minimally-invasive approach into the vertebral body permits to obtain a double mechanical support for the vertebral plate, to partially reduce the fracture, to mobilize the patient immediately, reducing disability and costs related to the vertebral osteoporotic fractures.

Pedicle screw and rod instrumentation has become the preferred technique for performing stabilization and fusion in the surgical treatment of lumbar spine degenerative disease. Rigid fixation leads to high fusion rates but may also contribute to stress shielding and adjacent segment degeneration. Thus, the use of semirigid rods made of polyetheretherketone (PEEK) has been proposed. Although the PEEK rods biomechanical properties, such as anterior load sharing properties, have been shown, there are few clinical studies evaluating their application in the lumbar spine surgical treatment. This study examined a retrospective cohort of patients who underwent posterior lumbar fusion for degenerative disease using PEEK rods, in order to evaluate the clinical and radiological outcomes and the incidence of complications.

This study is a retrospective consecutive case series analysis of 198 patients who underwent spine surgery between 2009 and 2010. The aim of this paper was to assess the efficacy and safeness of bed rest and lumbar drainage in treating postoperative CSF fistula. Postoperative cerebrospinal fluid (CSF) fistula is a well-known complication in spine surgery which lead to a significant change in length of hospitalization and possible postoperative complications. Management of CSF leaks has changed little over the past 20 years with no golden standard advocated from literature.Postoperative CSF fistulas were described in 16 of 198 patients (8%) who underwent spine surgery between 2009 and 2010. The choice of the therapeutic strategy was based on the clinical condition of the patients, taking into account the possibility to maintain the prone position continuously and the risk of morbidity due to prolonged bed rest. Six patients were treated conservatively (position prone for three weeks), ten patients were treated by positioning an external CSF lumbar drainage for ten days. The mean follow-up period was ten months.All patients healed their wound properly and no adverse events were recorded. Patients treated conservatively were cured in a mean period of 30 days, while patients treated with CSF drainage were cured in a mean period of 10 days.Lumbar drainage seems to be effective and safe both in preventing CSF fistula in cases of large dural tears and debilitated/irradiated patients and in treating CSF leaks.

Iliac crest bone graft (ICBG) is considered the gold standard for spine surgical procedures to achieve a successful fusion, because of its known osteoinductive and osteoconductive properties. Considering its autogenous origin, the use of ICBG has not been associated to an increase of intraoperative or postoperative complications directly related to the surgery. However, complications related to the harvesting procedure and to the donor site morbidity have been largely reported in the literature, favoring the development of a wide range of alternative products to be used as bone graft extenders or substitutes for spine fusion. The family of ceramic-based bone grafts has been widely used and studied during the last years for spine surgical procedures in order to reduce the need for iliac crest bone grafting and the consequent morbidity associated to the harvesting procedures.We report here the results of a post-market surveillance analysis performed on four independent cohorts of patients (115 patients) to evaluate the safety of three different formulations of hydroxyapatite-derived products used as bone graft extenders/substitutes for lumbar arthrodesis.No intraoperative or post-operative complications related to the use of hydroxyapatite-derived products were detected, during medium and long follow up period (minimum 12 months-maximum 5 years).This post-market surveillance analysis evidenced the safety of ceramic products as bone graft extenders or substitutes for spine fusion. Moreover, the evidence of the safety of hydroxyapatite-derived products allows to perform clinical studies aimed at evaluating the fusion rates and the clinical outcomes of these materials as bone graft extenders/substitutes, in order to support their use as an alternative to ICBG for spine fusion.

To evaluate the role of enbloc resection on the oncological outcome of patients with Ewing's sarcoma of the mobile spine treated with systemic multiagent chemotherapy combined with radiation therapy. While the role of chemotherapy and radiation therapy is well known and accepted in the treatment of Ewing sarcoma, there is no consensus on the role of enbloc resection in those tumors occurring in the mobile spine and, therefore, it can be difficult to conclude the decision making process, particularly if to achieve a tumor-free margin resection functionally relevant structures should be sacrificed. The study design of this work was the retrospective analysis of a series of 27 cases of Ewing sarcoma of the mobile spine homogeneously treated.Twenty-seven patients with primary ES of the mobile spine were treated from 1979 to 2008 by the same multidisciplinary team. All the patients presented with pain. Motor deficits were present in 6 patients. All the patients were submitted to multiagent protocols of chemotherapy (always) and radiotherapy (alternative to surgery or associated to). Surgery was performed in 17 cases both for functional purpose (7 cases: intralesional piecemeal excision) both for curative purpose (10 cases: enbloc resection, resulting characterized by marginal/wide or intralesional margins). Patients were observed for a minimum of 2 years or until death. The mean follow-up time was 65 months (median 28 months; ranging 2 to 218 months). Neurological function, local recurrence, distant relapse, and treatment-related complications were evaluated.Three periods were considered according to the evolution of therapeutic strategies. Four patients were treated in the period 1979 to 1982. All were submitted to chemotherapy (REA-2) and radiation therapy (RT). Two of them were submitted to intralesional excision. All these patients died 2 to 29 months later without significant difference in the two groups. In the period 1983 to 1990 all patients were treated with chemotherapy (REN-1/2) and RT. Two were submitted to intralesional excision and had a worse evolution as died of the disease at 2 and 11 months follow up, while the patients who did not received surgery evolved more favourably: 1 died of the disease 57 months after the end of the treatment, 3 are continuously disease free at 130, 190, 290 months. The sixteen patients treated in the period 1991 to 2008 received chemotherapy (REN-3 and ISG-SSG) and RT, combined with intralesional excision in 3, with enbloc with intralesional margins in 4, enbloc with tumor-free margins (wide or marginal) in 6. Only one patient submitted to tumor-free margin enbloc resection had local recurrence and died 29 months after the treatment (biopsy was performed by open approach), the other surviving continuously disease free at 17 to 193 months follow-up (av. 76 months). All the patients submitted to intralesional excision and to enbloc resection resulting in margin violation had the same prognosis, as died of the disease 10 to 63 months after the treatment. Only one of the 3 patients who had no surgery died of the disease 8 months after the treatment, the other surviving 9 and 49 months follow-up. Among the seven patients who had neurological symptoms at the occurrence, just those with no metastasis and who underwent enbloc resection are alive.The current study seems to demonstrate that tumor-free margin enbloc resection provides better local control and longer survival, while the results after intralesional margin resection or piecemeal excision combined with chemotherapy and RT is less effective than chemotherapy and RT alone.

In biology, it is easy to understand how a damaged functional system may generate wrong signals, but why this should happen when the system is disconnected is less clear. For this reason, among other pain syndromes, neuropathic pain (NP) following spinal cord injury (SCI) leaves most questions unanswered.Our purpose is to review current knowledge on NP after SCI, focusing on the mechanisms, assessment and management of the syndrome.The mechanisms responsible for NP following SCI are poorly understood: NP is classically considered a "central pain syndrome" but recent evidence from experimental models reveals a possible "peripheral sensitization". Assessment of NP following SCI is well-established: in addition to clinical evaluation and self-reported scales, many neurophysiological, radiological and microscopic investigations may be performed. The management of NP following SCI is very difficult: evidence of effective drugs is lacking and alternative new treatment approaches yield different outcomes.Recently clinical and instrumental tools have increased our knowledge on NP, suggesting that the discovery of new treatment agents will depend on an explanation of what changes after SCI: future research must point in this direction.

The results of 131 cases of classic non-metastatic osteosarcoma of the extremities treated with preoperative chemotherapy, surgery and postoperative chemotherapy are reported. Preoperatively, the patients were given Methotrexate (MTX) intravenously (high and average doses) and Cisplatinum (CDP) intraarterially. For postoperative chemotherapy, the drugs and treatment protocols were based on the degree of necrosis observed (good, fair, poor). Conservative surgery was performed in 73% of the cases and amputation in 27%. At an average 30 month follow-up (9-48 months) 59.5% of the patients had remained disease-free, 37.5% had developed metastases, and 3% had developed local recurrence. The percentage of disease-free patients was higher for those treated with high doses of MTX (68% vs. 51%) and for those in whom preoperative chemotherapy had induced good necrosis (75% vs. 44%). Neither the age or sex of the patients, nor the site of the tumor or type of surgery performed influenced prognosis.

The combination of surgery plus radiotherapy is a valid treatment option when en bloc resection with wide margins is not feasible. The aim of this paper is to show a novel technique for fixation of cervico-thoracic junction with carbon fiber reinforced peek pre-shaped rods with sublaminar bands in order to guarantee cervical spine stability and particle therapy efficacy. A 42 years-old man with a Sclerosing Epithelioid Fibrosarcoma (SEF) arising from C7, involving the right latero-cervical region from C5 to the apex of the right lung, underwent an en bloc resection with a double approach (anterior plus posterior). Fixation of cervical spine was achieved with composite PEEK/carbon fiber rods connected to C4 and T2 laminae with sublaminar bands. After surgery, the patient received 9 cycles of chemotherapy (cisplatin, etoposide and adriamycin) and proton therapy. At 2 years follow-up, no local recurrence was detected with implant stability demonstrating no mechanical failure. The main issues related to this case were: planning en bloc resection able to achieve an Enneking appropriate margin; incorporate in the decision making process the functional loss related to the sacrifice of neurological structures infiltrated by the tumor; establish a therapeutic strategy that included, in addition to surgery, adjuvant chemotherapy and radiotherapy for improving local and systemic control; stabilize with implants that do not interfere with accelerated particle radiotherapy.

Aims: To present our experience on spinal sclerosing epithelioid fibrosarcoma (SEF) and review the existing literature pertaining to SEF of the spine. Methods and results: Six cases of spinal SEF were reviewed, and a literature search of all primary SEFs of the spine was performed. All tumours occurred in adults (median age, 41years) and were located all along the spine, the lumbar vertebrae being the most commonly involved. All patients presented with pain that they had experienced for months. The mean tumour size at diagnosis was 52mm. Five tumours showed a spectrum of microscopic features consistent with pure SEF, and one showed a hybrid morphology with areas of low-grade fibromyxoid sarcoma. All were diffusely and strongly positive for mucin 4. Two cases were initially misdiagnosed as epithelioid haemangioendothelioma and aggressive chondroblastoma. Fluorescence in-situ hybridisation showed rearrangements of either FUS or EWSR1 in four cases. Reverse transcription polymerase chain reaction showed the presence of FUS–CREB3L1 and EWSR1–CREB3L1 fusion transcripts in two cases and one case, respectively. Of five patients with follow-up data available, two developed one or more local recurrences and three patients had metastatic disease. Distant metastases were mainly to other osseous locations, followed by lungs and lymph nodes. At last follow-up, three patients had died of disease and one was alive with multiple metastases. Conclusions: SEF is an aggressive sarcoma that can involve the spine. It is important to recognise the spine as the primary location of SEF, in order to avoid misdiagnosis as more common primary spinal neoplasms, which can impact on therapeutic approaches.

Objective: Even though carbon ions treatment (CIRT) of sacral chordoma (SC) substantially reduces tumor mass, tumor remnants are observed in most patients. Differentiating tumor remnants from necrosis is challenging, expensive in terms of imaging and time-consuming. So far, there has not been a systematic histological and metabolic analysis of post-CIRT lesions. We designed a prospective study aiming to histologically a metabolically differentiate between viable tumor and foci of necrosis and of fibrosclerosis after CIRT and correlate these findings to clinical outcome in patients with SC. Patients and methods: Between January 2013 and December 2016 18 patients, 12 males and 6 females, with histological confirmation of sacral chordoma, underwent CIRT. The total dose was 70.4 GyE, with a daily fraction of 4.4 GyE, for 4 weeks. MRI was performed every three months after treatment. FDG PET-CT scan and CT-guided needle biopsy were performed 6-12 months after CIRT. The incidence of complications (intraoperative and postoperative), local control (LC), overall survival (OS) and progression-free survival (PFS), changes in neurological status, clinical outcomes and toxicity were considered. Results: All histological analysis but 2 reported signs of necrosis and of fibrosclerosis after CIRT. One of these 2 patients turned into a dedifferentiated chordoma. Radiological partial response (PR) was observed in 10 patients (56.3%) and stable disease (SD) in 5 patients (28.3). Two patients (11%) had a local relapse. The overall survival rate was 100% at 24 months. FDG PET CT after CIRT showed uptake decreasing compared with the baseline exam in all but one patient. Conclusions: The histological presence of necrosis and of fibrosclerosis after CIRT at the histological analysis supports the previous clinical evidence on the efficacy of CIRT. Volumetric stability of the residual mass should be considered as a success of treatment. In cases of a volumetric increase of the mass, a CT needle biopsy should always be performed. In our series, during the follow-up, the FDG-PET was able to promptly detect an increased uptake in the case which later was histologically defined as dedifferentiated chordoma.

OBJECTIVE: This systematic review focuses on 5 key elements that may improve the decision-making process in spondylodiscitis: the infective agent, segmental instability, abscess development, neurological compromise and focus of infection. MATERIALS AND METHODS: We included 64 studies published between May 2012 and May 2017, that reported both a description of the discitis and comparative data regarding the disease and its complications. RESULTS: The majority of cases were caused by Staphylococcus spp (40.3%) and involved the lumbosacral region (52.3%). 27.8% of cases were associated to neurological compromise, 30.4% developed an abscess, 6.6% were associated to instability, and 54.7% underwent surgery. The abscesses mostly involved the lumbosacral region (60.4%) with paravertebral localization; 32.6% of cases involved the thoracic region, showing mostly epidural localization; a small number of cases (7%) involved the cervical region, mostly with epidural localization. 95% of paravertebral abscesses were treated percutaneously, while 85.7% of epidural cases underwent “open” surgery. Spinal cord compression mainly occurred in the cervical region (55.9%), neurological deficit was observed in over half of cases (65%), and surgery was required in most of the cases (83.9%). The majority of cases of instability involved the lumbosacral region (53.3%) and underwent surgery (87%). The focus of infection was mostly lumbosacral (61%) and almost all cases (95%) were treated surgically. CONCLUSIONS: Spondylodiscitis is a complex and multifactorial disease, whose diagnosis and management are still challenging. Due to its potential morbidity, it is extremely important to investigate the 5 key elements discussed in this paper in order to provide an early diagnosis and initiate the most effective treatment.

Chordomas are rare, malignant bone tumors of the skull-base and axial skeleton. Until recently, there was no consensus among experts regarding appropriate clinical management of chordoma, resulting in inconsistent care and suboptimal outcomes for many patients. To address this shortcoming, the European Society of Medical Oncology (ESMO) and the Chordoma Foundation, the global chordoma patient advocacy group, convened a multi-disciplinary group of chordoma specialists to define by consensus evidence-based best practices for the optimal approach to chordoma. In January 2015, the first recommendations of this group were published, covering the management of primary and metastatic chordomas. Additional evidence and further discussion were needed to develop recommendations about the management of local-regional failures. Thus, ESMO and CF convened a second consensus group meeting in November 2015 to address the treatment of locally relapsed chordoma. This meeting involved over 60 specialists from Europe, the United States and Japan with expertise in treatment of patients with chordoma. The consensus achieved during that meeting is the subject of the present publication and complements the recommendations of the first position paper.

In the clinical practice, it is quite common to diagnose a lesion in the bone or soft tissues that is amenable to biopsy. This is particularly true in patients who come to the hospital with a history of cancer, with radiological signs consistent with malignancy, or with the suspicion of focal septic inflammation.

We report a case of a 19-year-old woman with hereditary multiple exostoses, confirmed via EXT2 gene mutation, who presented to our clinic with a 4-month history of low back pain.

Spine infections require a multidisciplinary approach to be treated and solved. A guide line to drive physicians in the deep complexity of such a disease is extremely helpful. SIMP suggests a flow-chart built up on clear concepts such as right and well managed antibiotic therapy, sound stability of the spine, correct and smart use of the standard and functional imaging techniques, such as f18 FDG PET/CT. In 16 months a total of 41 patients have been treated for spondylodiscitis, discitis and vertebral osteomyelitis by our team of physicians and 25 patients have been enrolled in a prospective study whose target is the assessment of the SIMP flow-chart and of every single aspect that characterize it.

The objective of this study is to determine the best local treatment combined with neoadjuvant chemotherapy for ESFT of the spine and sacrum, for the best local treatment for Ewing sarcoma family tumors (ESFT) according to the primary site is still unclear. Nowadays surgery is used in local treatment of ESFT, but literature is scarce on the best local treatment in sites where surgery is problematic, such as the spine. This study evaluates the outcome and the rate of local recurrence of ESFT in the spine and sacrum when treated with neoadjuvant chemotherapy, and locally by radiotherapy alone or surgery, followed by reduced doses of radiotherapy. Forty-three patients with nonmetastatic ESFT located in the spine and sacrum were treated at our institution between 1983 and 2000 with neoadjuvant chemotherapy, and locally by radiotherapy alone in 26 cases, and surgery followed by radiotherapy at reduced doses in 17. The 5- and 10-year event-free survival (EFS) was 37 and 30%, and the 5- and 10-year overall survival was (OS) 42 and 32%. The prognosis was unrelated to gender and age, tumor volume, chemotherapy protocol, and local treatment. The outcome seemed worse for patients with primary tumors located in the sacrum than for patients with tumors located in the rest of the spine (5-year EFS = 23 vs. 46%). For these patients the results were significantly worse than for those we achieved with neoadjuvant treatment for ESFT located in other sites. However, no differences were observed between patients locally treated with radiotherapy alone and those treated by radiotherapy followed by surgery. We concluded that regardless of the type of local treatment even when associated with neoadjuvant therapy, ESFT in the spine and sacrum has a poor outcome and prognosis is significantly worse than that of primary ESFT in other sites.

The authors describe the clinical case of a patient aged 18 years affected with giant cell tumor (GCT) at C3 who came to the surgical unit of Orthopaedics and Traumatology at the Ospedale Maggiore in Bologna after being treated by surgery elsewhere. Particular attention is paid to surgical access by means of median transmandibuloglossotomy used in order to obtain a sufficiently wide surgical field that can adequately expose the vertebral segment affected by neoplastic disease. In particular, possible complications that may be observed postsurgery can be compared to other surgical approaches to the upper cervical spine and above all that there are no permanent clinical sequelae.