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  • 1
    Language: English
    In: Journal of clinical oncology, 2012-06-10, Vol.30 (17), p.2112-2118
    Description: We compared two chemotherapy regimens that included methotrexate (MTX), cisplatin (CDP), and doxorubicin (ADM) with or without ifosfamide (IFO) in patients with nonmetastatic osteosarcoma of the extremity. Patients age ≤ 40 years randomly received regimens with the same cumulative doses of drugs (ADM 420 mg/m(2), MTX 120 g/m(2), CDP 600 mg/m(2), and IFO 30 g/m(2)) but with different durations (arm A, 44 weeks; arm B, 34 weeks). IFO was given postoperatively when pathologic response to MTX-CDP-ADM was poor (arm A) or given in the primary phase of chemotherapy with MTX-CDP-ADM (arm B). End points of the study included pathologic response to preoperative chemotherapy, toxicity, and survival. Given the feasibility of accrual, the statistical plan only permitted detection of a 15% difference in 5-year overall survival (OS). From April 2001 to December 2006, 246 patients were enrolled. Two hundred thirty patients (94%) underwent limb salvage surgery (arm A, 92%; arm B, 96%; P = .5). Chemotherapy-induced necrosis was good in 45% of patients (48% in arm A, 42% in arm B; P = .3). Four patients died of treatment-related toxicity (arm A, n = 1; arm B, n = 3). A significantly higher incidence of hematologic toxicity was reported in arm B. With a median follow-up of 66 months (range, 1 to 104 months), 5-year OS and event-free survival (EFS) rates were not significantly different between arm A and arm B, with OS being 73% (95% CI, 65% to 81%) in arm A and 74% (95% CI, 66% to 82%) in arm B and EFS being 64% (95% CI, 56% to 73%) in arm A and 55% (95% CI, 46% to 64%) in arm B. IFO added to MTX, CDP, and ADM from the preoperative phase does not improve the good responder rate and increases hematologic toxicity. IFO should only be considered in patients who have a poor histologic response to MTX, CDP, and ADM.
    Subject(s): Biological and medical sciences ; Medical sciences ; Diseases of the osteoarticular system ; Tumors of striated muscle and skeleton ; Tumors ; Osteosarcoma - drug therapy ; Femur - pathology ; Humans ; Child, Preschool ; Male ; Tibia - pathology ; Cisplatin - administration & dosage ; Humerus - pathology ; Disease-Free Survival ; Ifosfamide - administration & dosage ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Chemotherapy, Adjuvant - methods ; Adolescent ; Adult ; Female ; Methotrexate - administration & dosage ; Bone Neoplasms - drug therapy ; Child ; Doxorubicin - administration & dosage
    ISSN: 0732-183X
    E-ISSN: 1527-7755
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
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  • 2
    Language: English
    In: BMC cancer, 2018-10-20, Vol.18 (1), p.1003-1003
    Description: Sarcomas that arise from the scapula or periscapular soft tissues often require a total scapulectomy. This often implies a large complex tissue defect that needs adequate reconstruction of both bone and soft tissue. Although various methods have been developed, no optimal procedure has emerged. Postoperative complications are common and functional recovery is not always satisfactory. This study aims to present a new surgical technique that combines a custom-made scapular prosthesis with a functional latissimus dorsi flap. Two patients diagnosed with malignant tumour of the scapular region were surgically treated after proper multidisciplinary evaluation. The first patient underwent the procedure as a first surgery, the second as revision surgery. The new technique comprises three surgical stages: excisional surgery with soft tissue resection and scapulectomy, bone reconstruction with custom-made prosthesis, and soft tissue reconstruction using a latissimus dorsi rotational flap overturned on the prosthesis. The goal is to set up a new functional unit combining an anatomically shaped implant (manufactured using latest three-dimensional printing technology) and a muscular flap, and to maintain the neurovascular supply. The patients were followed up to evaluate functional outcome and complications. Both patients were alive with no evidence of disease. Functional results were satisfactory and the Musculoskeletal Tumor Society scores were 87% and 63%, respectively. No surgical complications such as implant breakage, joint collapse, wound dehiscence, or infection were observed. This new technique upgrades the role of the latissimus dorsi flap to a functional tool in combination with an anatomical, three-dimensionally printed, custom-made prosthesis, and provides adequate well-vascularized and healthy tissue to maximize the likelihood of successful limb salvage.
    Subject(s): Scapula - surgery ; Humans ; Middle Aged ; Reconstructive Surgical Procedures - methods ; Sarcoma, Ewing - diagnosis ; Sarcoma, Synovial - diagnosis ; Surgical Flaps - transplantation ; Sarcoma, Synovial - surgery ; Scapula - pathology ; Shoulder Prosthesis ; Superficial Back Muscles - transplantation ; Female ; Child ; Sarcoma, Ewing - surgery ; Case studies ; Orthopedic implants ; Usage ; Care and treatment ; Sarcoma ; Patient outcomes ; Research ; 3D printing ; Implants, Artificial ; Prosthesis ; Surgery ; Index Medicus ; Latissimus dorsi flap ; Scapulectomy ; Scapular custom-made prosthesis ; Oncology ; Orthopaedics
    ISSN: 1471-2407
    E-ISSN: 1471-2407
    Source: BioMedCentral Open Access
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 3
    Language: English
    In: Journal of functional morphology and kinesiology, 2018-01-14, Vol.3 (1), p.7
    ISSN: 2411-5142
    E-ISSN: 2411-5142
    Source: PubMed Central
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  • 4
    Language: English
    In: Pediatric blood & cancer, 2015-06, Vol.62 (6), p.994-999
    Description: Background Post‐relapse survival (PRS) was evaluated in patients with Ewing sarcoma (EWS) enrolled in chemotherapy protocols based on the use of high‐dose chemotherapy with busulfan and melfalan (HDT) as a first‐line consolidation treatment in high‐risk patients. Procedure EWS patients enrolled in ISG/SSG III and IV trials who relapsed after complete remission were included in the analysis. At recurrence, chemotherapy based on high‐dose ifosfamide was foreseen, and patients who responded but had not received HDT underwent consolidation therapy with HDT. Results Data from 107 EWS patients were included in the analysis. Median time to recurrence (RFI) was 18 months, and 45 (42%) patients had multiple sites of recurrence. Patients who had previously been treated with HDT had a significantly (P = 0.02) shorter RFI and were less likely to achieve a second complete remission (CR2). CR2 status was achieved by 42 (39%) patients. Fifty patients received high‐dose IFO (20 went to consolidation HDT). The 5‐year PRS was 19% (95% CI 11 to 27%). With CR2, the 5‐year PRS was 48% (95% CI 31 to 64%). Without CR2, median time to death was six months (range 1–45 months). According to the multivariate analysis, patients younger than 15 years, recurrence to the lung only, and RFI longer than 24 months significantly influenced the probability of PRS. Conclusions Age, pattern of recurrence, RFI, and response to second‐line chemotherapy influence post‐relapse survival in patients with recurrent Ewing sarcoma. No survival advantage was observed from chemotherapy consolidation with HDT. Pediatr Blood Cancer 2015;62:994–999. © 2015 Wiley Periodicals, Inc.
    Subject(s): metastases ; Ewing sarcoma ; chemotherapy ; high-dose chemotherapy ; high‐dose chemotherapy ; Bone Neoplasms - mortality ; Humans ; Neoplasm Recurrence, Local ; Male ; Sarcoma, Ewing - mortality ; Busulfan - administration & dosage ; Sarcoma, Ewing - drug therapy ; Melphalan - administration & dosage ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Adolescent ; Adult ; Female ; Bone Neoplasms - drug therapy ; Child ; Care and treatment ; Chemotherapy ; Relapse ; Sarcoma ; Patient outcomes ; Analysis ; Diseases ; Cancer ; Index Medicus ; Clinical Medicine ; Pediatrics ; Medical and Health Sciences ; Medicin och hälsovetenskap ; Pediatrik ; Klinisk medicin
    ISSN: 1545-5009
    ISSN: 1545-5017
    E-ISSN: 1545-5017
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 5
    Language: English
    In: Pediatric blood & cancer, 2016-07, Vol.63 (7), p.1290-1292
    Description: A newborn with unresectable kaposiform hemangioendothelioma associated with Kasabach Merritt phenomenon, unresponsive to vincristine and prednisone, received second‐line treatment with propranolol at a dose of 2 mg/kg/day, starting at 2 months of life and continued for 13 months. There was only slight reduction in tumor mass, but measurement of propranolol levels showed extremely low plasma concentrations. The propranolol dose was progressively increased to 3.5 mg/kg/day, leading to a substantial increase in plasma levels associated with clinically relevant tumor reduction. This case highlights the importance of relating propranolol dose to its plasma concentration before considering the treatment ineffective for this vascular tumor.
    Subject(s): beta-blockers ; kaposiform hemangioendothelioma ; propranolol ; beta‐blockers ; Infant, Newborn, Diseases - drug therapy ; Propranolol - administration & dosage ; Humans ; Kasabach-Merritt Syndrome - drug therapy ; Male ; Hemangioendothelioma - drug therapy ; Infant, Newborn ; Drug Resistance, Neoplasm - drug effects ; Sarcoma, Kaposi - drug therapy ; Propranolol hydrochloride ; Index Medicus
    ISSN: 1545-5009
    E-ISSN: 1545-5017
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 6
    Language: English
    In: Journal of clinical medicine, 2021-03-04, Vol.10 (5), p.1056
    Description: Recently, custom-made 3D-printed prostheses have been introduced for limb salvage surgery in adult patients, but their use has not been described in pediatric patients. A series of 11 pediatric patients (mean age 10.8 years; range 2-13) with skeletal tumors treated with custom-made implants for the reconstruction of bony defects is described. Patients were followed up every 3 months. Functional results were evaluated by the Musculoskeletal Tumor Society Score (MSTS) for upper and lower limbs. The mean follow-up was 25.7 months (range 14-44). Three patients died after a mean of 19.3 months postoperatively-two because of disease progression and the other from a previous malignancy. Three patients experienced complications related to soft tissues. One patient required device removal, debridement, and antibiotic pearls for postoperative infection. Partial osseointegration between grafts and host bone was observed within a mean of 4 months. At the final follow-up, mean MSTS score was 75%. 3D prostheses may yield biological advantages due to possible integration with the host bone and also through the use of vascularized flaps. Further research is warranted.
    Subject(s): custom-made prosthesis ; vascularized flap ; pediatric limb salvage ; bone tumor
    ISSN: 2077-0383
    E-ISSN: 2077-0383
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 7
    Language: English
    In: BMC cancer, 2010-09-22, Vol.10 (1), p.504-504
    Description: Treatment strategies for Retinoblastoma (RB), the most common primary intraocular tumor in children, have evolved over the past few decades and chemoreduction is currently the most popular treatment strategy. Despite success, systemic chemotherapeutic treatment has relevant toxicity, especially in the pediatric population. Antiangiogenic therapy has thus been proposed as a valuable alternative for pediatric malignancies, in particolar RB. Indeed, it has been shown that vessel density correlates with both local invasive growth and presence of metastases in RB, suggesting that angiogenesis could play a pivotal role for both local and systemic invasive growth in RB. We present here two cases of sporadic, bilateral RB that did not benefit from the conservative treatment and we provide evidence that the VEGF-A pathway is significantly up-regulated in both RB cases along with an over expression of hERG1 K+ channels. Two patients showed a sporadic, bilateral RB, classified at Stage II of the Reese-Elsworth Classification. Neither of them got benefits from conservative treatment, and the two eyes were enucleated. In samples from both RB cases we studied the VEGF-A pathway: VEGF-A showed high levels in the vitreous, the vegf-a, flt-1, kdr, and hif1-α transcripts were over-expressed. Moreover, both the transcripts and proteins of the hERG1 K+ channels turned out to be up-regulated in the two RB cases compared to the non cancerous retinal tissue. We provide evidence that the VEGF-A pathway is up-regulated in two particular aggressive cases of bilateral RB, which did not experience any benefit from conservative treatment, showing the overexpression of the vegf-a, flt-1, kdr and hif1-α transcripts and the high secretion of VEGF-A. Moreover we also show for the first time that the herg1 gene transcripts and protein are over expressed in RB, as occurs in several aggressive tumors. These results further stress the relevance of the VEGF-A pathway in RB and the correlation with hERG1, making aggressive and recurrent RB cases good candidates for antiangiogenesis therapies based on the targeting of VEGF-A.
    Subject(s): Magnetic Resonance Spectroscopy - methods ; Follow-Up Studies ; Retinoblastoma - therapy ; Neoplasm Invasiveness ; Humans ; Gene Expression Regulation, Neoplastic ; Angiogenesis Inhibitors - pharmacology ; Ether-A-Go-Go Potassium Channels - metabolism ; Drug Resistance, Neoplasm ; Infant ; Male ; Vascular Endothelial Growth Factor A - metabolism ; Retinoblastoma - metabolism ; Neoplasm Metastasis ; Female ; Retinal Neoplasms - therapy ; Retinal Neoplasms - metabolism ; Case studies ; Care and treatment ; Patient outcomes ; Physiological aspects ; Ion channels ; Retinoblastoma ; Vascular endothelial growth factor ; Cancer ; Index Medicus
    ISSN: 1471-2407
    E-ISSN: 1471-2407
    Source: BioMedCentral Open Access
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 8
    Language: English
    In: Pediatric blood & cancer, 2014-08, Vol.61 (8), p.1369-1375
    Description: Background Symptoms of epidural compression (SEC) in children with neuroblastoma (particularly infants) may be misinterpreted, leading to delay in diagnosis. Patients and Methods Clinical, imaging and follow‐up data of 34 infants with neuroblastoma and SEC diagnosed between 2000 and 2011 at Italian AIEOP centers were retrieved and reviewed. Results Median age at initial SEC was 104 days (IQR 47–234). Main symptoms included motor deficit (85.3%), pain (38.2%), bladder and bowel dysfunctions (20.6% each). In the symptom‐diagnosis interval (S‐DI) (median, 12 days; IQR 7–34), the frequency of grade 3 motor deficit increased from 11.8% to 44.1% and that of bladder dysfunction from 20.6% to 32.4%. S‐DI was significantly longer (P = 0.011) for patients developing grade 3 motor deficit. First treatment of SEC was neurosurgery in 14 patients, and chemotherapy in 20. SEC regressed in 11 patients (32.3%), improved in 9 (26.5%), and remained stable in 14 (41.2%), without treatment‐related differences. Median follow‐up was 82 months. At last visit, 11 patients (32.3%) were sequelae‐free while 23 (67.7%) had sequelae, including motor deficit (55.9%), bladder (50.0%) and bowel dysfunctions (28.4%), and spinal abnormalities (38.2%). Sequelae were rated severe in 50% of patients. Severe sequelae scores were more frequent in patients presenting with spinal canal invasion 〉66% (P = 0.039) and grade 3 motor deficit (P = 0.084). Conclusions Both neurosurgery and chemotherapy provide unsatisfactory results once paraplegia has been established. Sequelae developed in the majority of study patients and were severe in a half of them. Greater awareness by parents and physicians regarding SEC is warranted. Pediatr Blood Cancer 2014; 61:1369–1375. © 2014 Wiley Periodicals, Inc.
    Subject(s): neuroblastoma ; infant ; late effects ; epidural compression ; Paraplegia - pathology ; Urinary Bladder Diseases - etiology ; Prospective Studies ; Hereditary Sensory and Motor Neuropathy - pathology ; Humans ; Arthrogryposis - etiology ; Infant ; Male ; Neuroblastoma - therapy ; Arthrogryposis - diagnosis ; Hereditary Sensory and Motor Neuropathy - etiology ; Hereditary Sensory and Motor Neuropathy - physiopathology ; Neuroblastoma - physiopathology ; Arthrogryposis - therapy ; Paraplegia - physiopathology ; Urinary Bladder Diseases - therapy ; Female ; Bowen's Disease - etiology ; Hereditary Sensory and Motor Neuropathy - therapy ; Arthrogryposis - pathology ; Child ; Infant, Newborn ; Neuroblastoma - pathology ; Bowen's Disease - physiopathology ; Bowen's Disease - therapy ; Arthrogryposis - physiopathology ; Paraplegia - diagnosis ; Bowen's Disease - pathology ; Neuroblastoma - complications ; Paraplegia - etiology ; Neuroblastoma - diagnosis ; Bowen's Disease - diagnosis ; Hereditary Sensory and Motor Neuropathy - diagnosis ; Paraplegia - therapy ; Adolescent ; Urinary Bladder Diseases - diagnosis ; Urinary Bladder Diseases - pathology ; Urinary Bladder Diseases - physiopathology ; Chemotherapy ; Neuroblastoma ; Cancer ; Index Medicus
    ISSN: 1545-5009
    E-ISSN: 1545-5017
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 9
    Language: English
    In: Biomedicines, 2020-10-13, Vol.8 (10), p.413
    Description: Ewing sarcoma (EWS) is a paediatric aggressive malignant tumour of bones and soft tissues. Multidisciplinary chemotherapies, surgical resection, and radiation represent the only strategies counteracting the disease, however spreading and relapse of disease still remain a clinical issue. Circulating tumour cells (CTCs) are an important feature of EWS but the prognostic significance has not been, yet, clarified. CTCs have been found both in patients with localized disease and in those who recur or metastasize. The identification of markers that can detect recurrences and metastasis remains an important challenge for research. Unfortunately, even most of patients with localized cancer relapsed and the reason has not yet been fully understood. In this clinical study on EWS patients, we evaluated the expression of CD99 antigen and beta-3 adrenergic receptor (β3-AR) on CTCs and bioptic derived cells by flow cytometry. The preliminary data revealed a higher β3-AR expression on cells derived from metastatic or relapsed patients, suggesting a role for the β3-AR as a possible predictive maker of disease recurrence in both patients with metastatic and localized disease.
    Subject(s): circulating tumour cells ; ewing sarcoma ; β3-adrenergic receptors
    ISSN: 2227-9059
    E-ISSN: 2227-9059
    Source: PubMed Central
    Source: ProQuest Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 10
    Language: English
    In: Pediatric nephrology (Berlin, West), 2012-08, Vol.27 (8), p.1351-1359
    Description: We evaluated urinary endothelin (ET)-1-like Immunoreactivity (uET-1 L) excretion in Wilms tumor (WT) survivors and investigated its relationships with glomerular filtration rate (GFR) and effective renal plasma flow (ERPF). Glomerular hemodynamics were also assessed by Gomez formulae.Seventeen WT survivors underwent renal sequential scintigraphy for residual kidney function determination including ERPF and GFR. Forty-five healthy individuals were selected as the control group. uET-1 L was measured by radioimmunoassay from the 24-h urine collection.In WT survivors, uET-1 L excretion was significantly higher than in controls. Significant correlations were found between uET-1 L and ERPF and GFR. Cluster analysis, applied on uET-1 L, identified two different patient groups. Between them, GFR and ERPF were significantly different. No significant difference existed between the two clusters for age and sex, elapsed time from nephrectomy, treatment, or nephrectomy side. Applying Gomez formulae, significant difference was found for afferent and total renal resistance.According to our results, uET-1 L seems to be a marker of glomerular injury in patients with renal mass loss revealing renal overload condition. The uET-1 L role in renal damage progression and hemodynamic glomerular worsening in nephrectomized patients should be proven by prospective long-term follow-up studies, even for potential ET-1 receptor antagonist therapeutic use.
    Subject(s): Urinary endothelin-1 ; Glomerular hemodynamic ; Pediatrics ; GFR ; Wilms’ tumor ; ERPF ; Medicine & Public Health ; 99m Tc-DTPA ; 123 I-OIH ; Gomez formulae ; Biomarkers - urine ; Glomerular Filtration Rate ; Humans ; Survivors ; Child, Preschool ; Infant ; Male ; Young Adult ; Wilms Tumor - urine ; Endothelin-1 - urine ; Renal Circulation ; Adolescent ; Age of Onset ; Kidney Neoplasms - urine ; Adult ; Female ; Child ; Index Medicus
    ISSN: 0931-041X
    E-ISSN: 1432-198X
    Source: Academic Search Ultimate
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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