20 research outputs found
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Diagnosis and prognosis for the Ewing family of tumors
No full textBackground: Ewing's sarcoma was first described by James Ewing in 1921. It is the second most common bone sarcoma seen in children, adolescents and young adults after osteosarcoma and belongs to the group of 'small round blue cell tumors', showing an aggressive natural history. Once almost invariably fatal and treated only with palliative radiation, thanks to a multidisciplinary approach, the probability of survival at 5years is now - 65 - 75% for patients with localized disease. This percentage is no more than 20 - 25% for patients with metastatic disease at presentation. Objective: To review epidemiology, diagnosis and prognosis of Ewing's sarcoma family tumors (EFT) of bone. This entity includes a wide spectrum of tumors, from the less differentiated or classic Ewing's sarcoma to the more differentiated peripheral neuroectodermal tumor. A translocation involving the EWS gene on the chromosome 22 band q12 is characteristic of EFTs. Conclusion: A complete clinical and radiological assessment is essential for initial patient evaluation. Owing to the rarity of this entity, whenever an EFT is suspected patients should be referred to a bone tumor treatment center before a diagnostic biopsy is performed. The molecular characterization of chromosomal translocations has an important role in the diagnosis of EFT. © 2009 Informa UK Ltd. All rights reserved
Is the association of electrochemotherapy and bone fixation rational in patients with bone metastasis?
Get PDFBackgroundBone metastases are frequent in patients with cancer. Electrochemotherapy (ECT) is a minimally invasive treatment based on a high-voltage electric pulse combined with an anticancer drug. Preclinical and clinical studies supported the use of ECT in patients with metastatic bone disease, demonstrating that it does not damage the mineral structure of the bone and its regenerative capacity, and that is feasible and efficient for the treatment of bone metastases. Year 2014 saw the start of a registry of patients with bone metastases treated with ECT, whose data are recorded in a shared database. Questions/Purposes(1) Among patients who underwent ECT and internal fixation for bone metastasis, how many experienced a reduction of pain? (2) How many cases showed a radiological response? (3) How many patients presented local or systemic complication after ECT and fixation? Patients and MethodsPatients were treated in Bologna at Rizzoli Orthopaedic Institute between March 2014 and February 2022 and recorded in the REINBONE registry (a shared database protected by security passwords): clinical and radiological information, ECT session, adverse events, response, quality of life indicators, and duration of follow-up were registered. We consider only cases treated with ECT and intramedullary nail during the same surgical session. Patients included in the analysis were 32: 15 males and 17 females, mean age 65 +/- 13 years (median 66, range 38-88 years), mean time since diagnosis of primary tumor 6.2 +/- 7.0 years (median 2.9, range 0-22 years). Nail was indicated in 13 cases for a pathological fracture in, 19 for an impending fracture. Follow-up was available for 29 patients, as 2 patients were lost to follow-up and 1 was unable to return to controls. Mean follow-up time was 7.7 +/- 6.5 months (median 5, range 1-24), and 16 patients (50%) had a follow-up longer than 6 months. ResultsA significant decrease in pain intensity was observed at the mean Visual Numeric Scale after treatment. Bone recovery was observed in 13 patients. The other 16 patients remained without changes, and one presented disease progression. One patient presented a fracture occurrence during the ECT procedure. Among all patients, bone recovery was observed in 13 patients: complete recovery in 1 patient (3%) and partial recovery in 12 patients (41%). The other 16 patients remained without changes, and one presented disease progression. One patient presented a fracture occurrence during the ECT procedure. However, healing was possible with normal fracture callus quality and healing time. No other local or systemic complications were observed. ConclusionWe found that pain levels decreased after treatment in 23 of the 29 cases for a pain relief rate of 79% at final follow-up. Pain is one of the most important indicators of quality of life in patients that undergo palliative treatments. Even if conventional external body radiotherapy is considered a noninvasive treatment, it presents a dose-dependent toxicity. ECT provides a chemical necrosis preserving osteogenic activity and structural integrity of bone trabeculae; this is a crucial difference with other local treatments and allows bone healing in case of pathological fracture. The risk of local progression in our patient population was small, and 44% experienced bone recovery while 53% of the cases remained unchanged. We observe intraoperative fracture in one case.This technique, in selected patients, improves outcome in bone metastatic patients combing both the efficacy of the ECT in the local control of the disease and the mechanical stability with the bone fixation to synergize their benefits. Moreover, the risk of complication is very low. Although encouraging data, comparative studies are required to quantify the real efficacy of the technique. Level of Evidence Level I, therapeutic study
Non-surgical treatment of chondrosarcoma: Current concepts and future perspectives
No full textChondrosarcoma represents a heterogeneous group of malignant cartilage tumors. Surgery is the most important component of treatment. Although convincing evidence of its benefit is lacking, chemotherapy might be useful in the treatment of mesenchymal and dedifferentiated chondrosarcoma. Radiotherapy plays an important role in the treatment of chondrosarcomas located in the spheno-occipital region and cervical spine. New modern radiation techniques could further improve the outcome for patients with lesions in sites that are surgically difficult to access. Finally, better understanding of molecular diagnostics and pathogenesis of chondrosarcoma, together with the development of new therapeutic agents, has resulted in numerous studies investigating potentially active treatment approaches to this disease. However, at present, the majority of these new compounds are being tested in preclinical settings, and their future clinical application still needs to be defined. © 2010 Elsevier Inc. All rights reserved
Prognostic significance of immunohistochemical expression of ezrin in non-metastatic high-grade osteosarcoma
No full textBackground. Ezrin is a membrane-cytoskeleton linker protein involved in regulating growth and metastatic behaviour of cancer cells. The study evaluated ezrin expression and its prognostic value in patients with non-metastatic osteosarcoma. Procedures. Ezrin expression and pattern of staining (cytoplasmic or membraneous and cytoplasmic) were assessed using immunohistochemistry on slides from tumour biopsy. We studied 95 patients (median 16 years, range: 4-39 years) with primary non-metastatic osteosarcoma of the extremity treated by neoadjuvant chemotherapy based on methotrexate, cisplatin, doxorubicin and ifosfamide. Results. Seventy-six patients (80%) showed ezrin immunoreactivity: in cytoplasm (37, 47%) and in cytoplasm and membrane (42, 53%) of tumour cells. Immunohistochemical staining score was: 1+ (16, 24%), 2+(10, 13%), 3+ (17, 21.5%) and 4+ (36, 45.5%). Ezrin and score expression were not related to gender, site, alkaline phosphatase (AP), LDH serum levels, chemotherapy-induced tumour necrosis or patient outcome. A significant association was seen between expression pattern and prognosis. The 3-year probability of disease-free survival was 80% for patients with only cytoplasmic immunostaining and 54% for patients with cytoplasmic and membranous immunostaining (P 80% at 5 years can be expected. These results suggest further investigations to define the relation between expression pattern, ezrin functional status and outcome in patients with non-metastatic osteosarcoma. © 2007 Wiley-Liss, Inc
Is core decompression and bone marrow concentrate with demineralized bone matrix and platelet-rich fibrin suitable for treating femoral head osteonecrosis?
Get PDFThe aim of this article is to determine the safety and efficacy of core decompression (CD) combined with injection of autologous bone marrow concentrate (BMC), demineralized bone matrix (DBM), and platelet-rich fibrin (PRF) for treating femoral head osteonecrosis. Seventy-seven patients (53 males and 24 females) for a total of 87 hips were treated for hip osteonecrosis with CD combined with injection of autologous BMC, DBM, and PRF at Rizzoli Orthopedic Institute from September 2008 to December 2019. Patients were assessed at baseline, at 45 days, and at 3, 6, 12, 24, and 36 months postoperatively. The primary outcome was the survival rate of hips not converted to total hip arthroplasty (THA). The secondary outcomes were (I) radiographic positive evolution assessed by X-ray films and magnetic resonance imaging and (II) the clinical symptoms evaluated with the Harris Hip Score (HHS). Eighty-seven hips from 77 patients with femoral head osteonecrosis (FHON), 60 males and 27 females, with a median age of 34 years (range 15-55) were included. The cause of necrosis was steroid treatment in 30 patients (17 of these for hematological malignancies, 2 for lupus, 1 for Churg-Strauss syndrome, and the remaining for other causes), 1 was alcohol-related, 4 followed hip injury, while 15 patients had idiopathic causes. THA was carried out in 20 hips (40%). These patients had lesions classified as IIa on the Ficat stage in four cases, six were IIb, nine were III, and one was 4. No CD-related complications were found during THA surgery or at the last follow-up in these cohorts of patients. Radiographic progression of the FHON was found in 14 hips (28%), with a higher percentage on Ficat's stage IIb. There were procedure-related complications in two hips, including one femoral neck fracture and one deep infection. Nineteen hips with successful treatment had good to excellent functional results at a 3-year follow-up or more (HHS ≥ 80). The long-term outcomes of treatment with CD and injection with BMC combined with DBM and PRF are promising to prevent femoral head collapse in patients with FHON. Moreover, CD does not influence the outcome in cases of THA
Osteosarcoma of the pelvis: a monoinstitutional experience in patients younger than 41 years
Get PDFSupported by: Istituto Ortopedico Riz-zoli research funds. All authors have contributed signifi-cantly to the manuscript and declare that the work is original and has not been submitted or published else-where. None of the authors have any financial disclosure or conflict of inter-est
The use of expandable megaprostheses for reconstruction of the distal femur in pediatric patients after bone tumor resection
Get PDFExpandable prostheses are becoming increasingly popular in the reconstruction of children with bone sarcomas of the lower limb. Since the introduction of effective chemotherapy in the treatment of these pathologies, in the 70s, there has been need for new limb salvage techniques. In children, limb salvage of the lower limbs is particularly challenging, not in the last place, because of the loss of growth potential. Therefore, expandable prostheses have been developed. However, the first experiences with these implants were not very successful. High complication rates and unpredictable outcomes raised major concerns on this innovative type of reconstruction. The rarity of the indication is one of the main reasons why there has been a relatively slow learning curve and implant development regarding this type of prosthesis. This PhD thesis, gives an overview of the introduction, the development, the current standards, and the future perspectives of expandable prostheses for the reconstruction of the distal femur in children
Sex- and age-related chemotherapy toxicity in patients with non-metastatic osteosarcoma
No full textThe influence of age and sex on chemotherapy-related toxicity was evaluated in children and adults with non metastatic osteosarcoma. Treatment consisted of methotrexate (MTX, 12 g/m2), cisplatin (CDP 120 mg/m2) and doxorubicin (ADM 75-90 mg/m2) and high-dose ifosfamide (HDIFO). Toxicity data from 1,051 courses (295 with MTX, 756 based on doxorubicin, cisplatin and high-dose ifosfamide) were analyzed. Children (4-14 yrs) and females showed a higher incidence of grade 4 neu-tropenia and thrombocytopenia and were more frequently hospitalized for neutropenic fever compared to adolescents and young adults (AYA, 15-19 yrs) and adults (>20-40 yrs). Delayed MTX excretion was higher in adults than AYA and children. Adults (up to 40 years) can be treated with pediatric protocols for osteosarcoma and they experience lower hematologic toxicity compared to pediatric population. Further investigations on sex-related susceptibility to chemotherapy in osteosarcoma patients are recommended
Periosteal osteosarcoma: a single-institution experience.
No full textBackground: Periosteal osteosarcoma is a rare variant of osteosarcoma. Wide surgical removal is the mainstay of treatment, but controversy remains about the role of chemotherapy. The objective of this study was to review and analyze the clinical and treatment-related factors that influence the survival of patients with periosteal osteosarcoma who received treatment in a single institution. Methods: Thirty-three patients with periosteal osteosarcoma (19 males and 14 females) with a median age of 16 years (range, ages 6-32 years) underwent surgery (32 patients) and received radiotherapy (1 patient). Chemotherapy was received according to different regimens for high-grade osteosarcoma by 14 patients who had grade 3 tumors. Results: The 10-year overall survival rate was 84%. The only patient who did not undergo surgery died of disease after 9 months; for the remaining 32 patients the 10-year disease-free survival rate was 65%. Survival was not influenced by the receipt of chemotherapy. The patients who received chemotherapy had a 10-year overall survival rate of 86%, and those who received only local treatment had an overall survival rate of 83% (P =.73). Conclusions: The authors' experience indicated that the treatment of periosteal osteosarcoma requires only wide surgical removal of the tumor and that adjuvant chemotherapy does not improve survival. © 2010 American Cancer Society
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Pexidartinib for the treatment of adult patients with symptomatic tenosynovial giant cell tumor: safety and efficacy
No full textINTRODUCTION: Tenosynovial giant cell tumor (TGCT) is a benign clonal neoplastic proliferation arising from the synovium often causing pain, swelling, joint stiffness, and reduced quality of life. The optimal treatment strategy in patients with diffuse-type TGCT (dt-TGCT) is evolving. Surgery is the main treatment, with a high recurrence rate and surgery-related morbidity. Radiotherapy is associated with important side effects. TGCT cells overexpress colony-stimulating factor 1 (CSF1). Pexidartinib (Turalio™) is a selective CSF1 R inhibitor, which was recently approved by the FDA for the treatment of TGCT. AREAS COVERED: This article reviews the pharmacological properties, clinical efficacy, and safety of pexidartinib. EXPERT OPINION: Pexidartinib was effective with an acceptable safety profile for advanced TGCT in phase I-III studies. The phase III trial (ENLIVEN) in unresectable TGCT met its primary endpoints of overall response rate. These results led to FDA approval for this TGCT population. Mixed or cholestatic hepatotoxicity was observed in rare cases. For this reason, pexidartinib is currently available only through a Risk Evaluation and Mitigation Strategy (REMS) Program in the USA. TGCT significantly impairs patients' quality of life. The approval of pexidartinib has changed the therapeutic armamentarium for this condition. However, strict monitoring of liver function is warranted
