Symptoms and their implications on quality of life and psychological distress in sarcoma patients.

The aim of this study was to investigate symptoms, their variation over time and their relationship with quality of life (QoL)/psychological distress in sarcoma patients, as few data regarding QoL and psychological distress in this set of patients are currently available. A total of 188 sarcoma patients from an Italian referral center were involved. Symptoms and financial difficulties were evaluated with the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire from the first treatment and over the follow-up period, up to 6 years. The authors found that patients with sarcoma experience several symptoms, especially fatigue and pain, which may dramatically worsen QoL and psychological distress. In conclusion, patients with sarcoma often experience fatigue, pain and financial difficulties, which negatively impacts QoL and psychological distress. To ameliorate overall QoL, proper control of symptoms is necessary.

Arthroscopic guided biopsy and radiofrequency thermoablation of a benign neoplasm of the tibial spines area: a treatment option.

BACKGROUND: Lesions located in the area of the tibial spines are rare. In most cases, treatment follows histological diagnosis, but when imaging and clinical data are considered to be "very" characteristic for benign lesions, such as chondroblastoma or osteoid osteoma, treatment may be performed without biopsy. Traditional curettage requires opening the joint, which presents a high risk of contamination of the joint itself and surrounding structures, such as the popliteal area, with possible contamination of the neurovascular bundle when performing curettage with the posterior approach. In this case, the re-excision of a local recurrence would be extremely difficult. RESULTS: We describe a technique using arthroscopic guidance for radiofrequency thermoablation of a benign lesion in the tibial spines area. We report on an illustrative case. The patient so treated, reported immediate relief from the pain, and after two weeks, was free of pain. The biopsy performed before the treatment confirmed the radiological diagnosis of chondroblastoma. At one year of follow-up, the patient is without pain, with a 0-130° range of motion, has no activity limitations and is apparently free of disease. CONCLUSION: This technique allows a radiofrequency thermoablation of a lesion in the tibial spines area and in the posterior tibial surface to be performed without opening the joint, monitoring the tibial plateau surface, probably decreasing the risk of cartilage damage. Unfortunately, in the case presented, the high pressure from the arthroscopy's pump broke the tibial plateau surface creating a communication to the tibial tunnel used for thermoablation.

Interventional Radiology in the Management of Metastases and Bone Tumors.

Interventional Radiology (IR) has experienced an exponential growth in recent years. Technological advances of the last decades have made it possible to use new treatments on a larger scale, with good results in terms of safety and effectiveness. In musculoskeletal field, painful bone metastases are the most common target of IR palliative treatments; however, in selected cases of bone metastases, IR may play a curative role, also in combination with other techniques (surgery, radiation and oncology therapies, etc.). Primary malignant bone tumors are extremely rare compared with secondary bone lesions: osteosarcoma, Ewing sarcoma, and chondrosarcoma are the most common; however, the role of interventional radiology in this fiels is marginal. In this review, the main techniques used in interventional radiology were examined, and advantages and limitations illustrated. Techniques of ablation (Radiofrequency, Microwaves, Cryoablation as also magnetic resonance imaging-guided high-intensity focused ultrasound), embolization, and Cementoplasty will be described. The techniques of ablation work by destruction of pathological tissue by thermal energy (by an increase of temperature up to 90 °C with the exception of the Cryoablation that works by freezing the tissue up to -40 °C). Embolization creates an ischemic necrosis by the occlusion of the arterial vessels that feed the tumor. Finally, cementoplasty has the aim of strengthening bone segment weakened by the growth of pathological tissue through the injection of cement. The results of the treatments performed so far were also assessed and presented focused the attention on the management of bone metastasis.

Ectopic primary type A thymoma located in two thoracic vertebras: a case report.

BACKGROUND: The thymus arises in the ventral portion of the third and fourth pharyngeal pouch. It descends into the anterior mediastinum at 6th week of gestation. Any errors occurring during this process can cause dissemination of aberrant nodules that are responsible for most atypical thymomas. CASE PRESENTATION: The authors report a unusual case of type-A thymoma located in D10 and D11 vertebral bodies.The histology showed a uniform growth of short, spindle shaped, mitotically inactive cells. A few small, normal lymphocytes were seen scattered or in small groups. The immunohistochemical investigation for neuroectodermal, neuroendocrine, vascular and muscular markers were negative. It also confirmed the presence of CD3+, CD5+ T lymphocytes and the absence of immature T-lymphocyte markers. CONCLUSIONS: The case described shows a thymic hystogenesis for spindle cell tumours. To our knowledge no other cases of vertebral thymomas have been described in international literature.

Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients.

BACKGROUND: Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. METHODS: We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. RESULTS: Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six patients (24%) had primary disease, 14(56%) a recurrent disease, and 5(20%) a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23%) and intralesional in 17(77%) patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons) were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94%) patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR) beta were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54%) symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively. CONCLUSIONS: Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multi-disciplinary therapeutic team is, however, essential to succeed in improving patients' outcome.

The advantages of carbon fiber based orthopedic devices in patients who have to undergo radiotherapy.

BACKGROUND AND OBJECTIVES: The modern approach to primary and secondary muscular skeletal tumors is multidisciplinary. The right combination of chemotherapy, surgery and radiotherapy (RT) makes obtaining local and distant disease control more likely. When surgery is indicated, radiotherapy often has a fundamental role as an adjuvant treatment; however, the titanium alloy instrumentations interfere with Radiotherapy setting, decreasing its effectiveness. It is common opinion that carbon fiber-reinforced devices are convenient in case of adjuvant RT in muscular skeletal oncology. The aim of the study is to support this intuition with experimental data, verifying the more accurate estimation of the delivered dose during RT, comparing Carbon Fiber-Reinforced PEEK (CFRP) plates with titanium-alloy orthopedic devices in order to evaluate their effects on target volume identification and dose distribution for radiation treatment. METHODS: Phantoms were then irradiated with a linear accelerator Varian 2100 C/D with photon beams of 6 and 15 MV energies. Absorbed dose in the point of interest was verified by EBT3 gafchromic films above and below the two materials. Images from CT simulations were also analyzed in terms of Hounsfield numbers in patients with titanium and carbon fiber orthopedic implants in the spine or in the femur. RESULTS: For a 6 MV photon beam, the doses measured just under the titanium-alloy plate were less than approximately 20% of the value calculated by the TPS. For a 15 MV beam energy, these differences were slightly lower. Using CFRP plate, the difference between measured and calculated doses was within ±3% for both energies, which was comparable with the statistical uncertainties. In the cases of simulated treatment of humerus titanium implants, the difference varies in range ± 10% with hot spot of + 10% and cold spot of -15%. CONCLUSIONS: The use of CFRP for orthopedic devices and implants provides a valuable advantage in identifying the target due to the reduction of artifacts. Clear imaging of the soft tissues surrounding the bone is useful and reduces the discrepancies between calculated/delivered and measured doses, generating a more homogeneous dose distribution. Furthermore, there is a significant benefit in detecting the state of disease in CT imaging during the follow-up of treated patients. In-vivo studies are encouraged to verify whether a more effective radiotherapy leads to a decrease in local recurrence and local progression.

An osteoporotic hip fracture in a 14-year-old girl undergoing chemotherapy and operated for knee osteosarcoma.

Non-weight-bearing, pre- and postsurgical immobilization, neoadjuvant and adjuvant chemotherapy are known to act on bone turnover, causing osteoporosis over short and long time periods. Treatment of fracture insurgence is very difficult because it really depends on being able to choose the right time (i.e., when immunodeficiency is less important). We report a case of spontaneous neck femur fracture during adjuvant chemotherapy in a young girl treated with resection and prosthesis reconstruction for distal femur osteosarcoma. Possible prevention and the correct approach and surgical timing are emphasized considering immunodeficiency following chemotherapy.

Anterior robotic approach in en-bloc sacrectomy: a preliminary experience.

En-bloc sacrectomy is a highly demanding surgical procedure necessary to obtain wide margin in sacral tumor. The double approach, anterior and posterior approach, is usually preferred for tumors extending proximally to S3 level where iliac internal vessels are at a higher risk for damage during posterior surgery. It can be justified also in selected cases to decrease the risk of posterior approach as in local recurrence or in patients who already underwent laparotomy. Our intent was to apply robotic-assisted techniques for performing anterior preparatory approach for sacrectomy surgery. Between December 2010 and December 2014, three cases of sacrectomies were performed in a previous robotic-assisted preparatory approach to separate the rectum from the tumor. Dissections were successfully performed in all cases close to the pelvic floor. The surgeon was able to position a Gore-Tex spacer between the anterior tumor surface and the rectum in all cases. The anterior dissections were performed with a perfect control of bleeding. No complications related to the anterior approach were reported. Robot-assisted surgery can be considered a valid and minimally invasive technique which allows a safe anterior dissection of the pelvic structures dividing tumors from surrounding tissues. It allows to place a spacer to protect organs during posterior sacral resection performed on the same day or at a later time. Further experiences are advocated to evaluate its efficiency in sacral tumors of greater size.

The Carbofix™ "Piccolo Proximal femur nail": A new perspective for treating proximal femur lesion. A technique report.

Metastases to proximal femur are common and surgery is often suggested to prevent fractures; otherwise it is necessary in cases where this has already occurred. Adjuvant radiotherapy is necessary to reduce the risk of local progression. Nevertheless, the success or failure of radiation therapy treatments depends upon the accuracy in which target identification is correct and dose prescription is fulfilled. Unfortunately, the use of titanium nails consistently limits radiation dose; indeed, the presence of ferromagnetic artifacts interferes with target identification. We present the technique for implant a new carbon fiber nail useful to reduce the ferromagnetic artifacts which allows a better adjuvant radiotherapy.

Radiation-induced osteosarcoma arising 20 years after the treatment of Ewing's sarcoma.

We report the case of a 17 years old female with a Ewing's sarcoma of the left femur treated with limb sparing surgery followed by local radiotherapy of 45 Gy and adjuvant chemotherapy with vincristine, doxorubicine, cyclophosphamide, actinomycin D. The patient received neoadjuvant chemotherapy for osteosarcoma and a left femur resection with endoprosthesis replacement. The patient is alive and free of disease 4 years after the treatment of this second malignant neoplasm (SMN). This case shows that radioinduced SMN can occurr with relatively low doses of RT (<50 Gy) and that it may occur very late.