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150


A Next-Generation Sequencing Study of Seven Primary Central Chondrosarcomas in the Pediatric Population Showed Recurrent IDH Mutations and a Novel EWSR1-SMAD3 Fusion [Meeting Abstract]

Zhang, Lingxin; Zhu, Gord Guo; Nafa, Khedoudja; Mohanty, Abhinita; Rana, Satshil; Healey, John; Fabbri, Nicola; Hameed, Meera
ISI:000518328900109
ISSN: 0893-3952
CID: 5294052

Genomic Profiling Identifies Association of IDH1/IDH2 Mutation with Longer Relapse-Free and Metastasis-Free Survival in High-Grade Chondrosarcoma

Zhu, Guo Gord; Nafa, Khedoudja; Agaram, Narasimhan; Zehir, Ahmet; Benayed, Ryma; Sadowska, Justyna; Borsu, Laetitia; Kelly, Ciara; Tap, William D; Fabbri, Nicola; Athanasian, Edward; Boland, Patrick J; Healey, John H; Berger, Michael F; Ladanyi, Marc; Hameed, Meera
PURPOSE:mutation status and clinical outcomes in chondrosarcomas. EXPERIMENTAL DESIGN:mutation status with the patient's clinical outcome. RESULTS:mutation, suggesting a possible association and synergy among these genes in chondrosarcoma progression. We found 21% of patients with chondrosarcoma also had histories of second malignancies unrelated to cartilaginous tumors, suggesting possible unknown genetic susceptibility to chondrosarcoma. CONCLUSIONS:
PMCID:6980683
PMID: 31615936
ISSN: 1557-3265
CID: 5293642

Osteofibrous Dysplasia (Kempson-Campanacci's disease): Long Term Follow-up Study on Natural History, Results of Treatment and Relationship with Adamantinoma [Meeting Abstract]

Righi, Alberto; Gambarotti, Marco; Campanacci, Laura; Sbaraglia, Marta; Fabbri, Nicola; Dei Tos, Angelo
ISI:000518328800092
ISSN: 0023-6837
CID: 5294022

Impact of Magnetic Resonance Imaging (MRI) Findings on Management of Symptomatic Patients Following Radiofrequency Ablation (RFA) of Osteoid Osteoma (OO)

Maybody, Majid; Soliman, Mohamed M; Hwang, Sinchun; Gonzalez-Aguirre, Adrian; Martin, Ernesto G Santos; Kaye, Elena; Hsu, Meier; Moskowitz, Chaya; Healey, John H; Fabbri, Nicola
OBJECT/OBJECTIVE:To assess the impact of MRI findings on management of symptomatic patients following RFA of OO. MATERIALS & METHODS/METHODS:Retrospective review of 43 patients with RFA for OO between June 2010 and June 2017 was performed. Patient, nidus and ablation data were reviewed. Pre- and 6-8 weeks post-procedural MRI (n=32) were compared for coverage of nidus by ablation zone, bone marrow edema, nidus hyperintensity and other findings. Baseline pain levels and analgesic use were compared with post-procedural follow-up visit at 6-8 weeks. Three groups of clinical and MRI outcomes of complete (CR), partial (PR) and no response (NR) were defined. A weighted-kappa statistic was used to assess for agreement. RESULTS:Clinical responses were CR in 34/43 (79.1%, 95%CI: 64.0-90.0%), PR in 8/43 (18.6%) and NR in 1/43 (2.3%) patients. All 19/32 patients with MRI CR experienced clinical CR. One patient with MRI NR had clinical NR. All 7/32 patients with clinical PR had MRI PR. All 4/43 complications were in MRI PR or NR groups. Substantial agreement was observed between MRI and clinical outcomes (kappa:0.69, 95%CI:0.45-0.95). MRI helped determine etiologies in all symptomatic patients and their management (n=8). CONCLUSIONS:MRI is recommended for symptomatic patients after ablation.
PMCID:7681937
PMID: 33235978
ISSN: 2523-8973
CID: 5293692

Osteofibrous Dysplasia (Kempson-Campanacci's disease): Long Term Follow-up Study on Natural History, Results of Treatment and Relationship with Adamantinoma [Meeting Abstract]

Righi, Alberto; Gambarotti, Marco; Campanacci, Laura; Sbaraglia, Marta; Fabbri, Nicola; Tos, Angelo Dei
ISI:000518328900092
ISSN: 0893-3952
CID: 5294032

Treatment of Colorectal Cancer: a Multidisciplinary Approach

Anania, G; Resta, G; Marino, S; Fabbri, N; Scagliarini, L; Marchitelli, I; Fiorica, F; Cavallesco, G
BACKGROUND:Colorectal cancer is the third most prevalent cancer in the world, preceded by prostate and lung cancers in men (10%) and breast and lung cancers in women (9.4%). Colorectal cancer is the fourth leading cause of death in men (7.6%) and the third in women (8.6%). A multidisciplinary approach has radically changed the way we deal with this disease among all specialist fields. PURPOSE/OBJECTIVE:In this study, we propose comparing the multidisciplinary experience group (started in 2012) of S. Anna Hospital (University of Ferrara) with the previous approach to rectal cancer before the advent of the multidisciplinary program. RESULTS:We find that more study depth of neoplastic disease as well as of each individual patient leads to more accurate staging and to a weighted therapy based on the needs of the individual. All the studies were performed in accordance with the guidelines established by the European and Italian associations.
PMID: 29656351
ISSN: 1941-6636
CID: 5354162

Femoral Fracture in Primary Soft-Tissue Sarcoma of the Thigh and Groin Treated with Intensity-Modulated Radiation Therapy: Observed versus Expected Risk

Folkert, Michael R; Casey, Dana L; Berry, Sean L; Crago, Aimee; Fabbri, Nicola; Singer, Samuel; Alektiar, Kaled M
PURPOSE/OBJECTIVE:This study was designed to compare the observed risk of femoral fracture in primary soft-tissue sarcoma (STS) of the thigh/groin treated with intensity-modulated radiation therapy (IMRT) to expected risk calculated using the Princess Margaret Hospital (PMH) nomogram. METHODS:Expected femoral fracture risk was calculated by using the PMH nomogram. Cumulative risk of fracture was estimated by using Kaplan-Meier statistics. Prognostic factors were assessed with univariate and multivariate analysis using Cox's stepwise regression. RESULTS:Between February 2002 and December 2010, 92 consecutive eligible patients were assessed. Median follow-up was 73 months (106 months in surviving patients). IMRT was delivered preoperatively (50 Gy) in 13 (14%) patients and postoperatively in 79 (86%) patients (median dose, 63 Gy; range, 59.4-66.6 Gy). The observed crude risk of fractures was 6.5% compared with 25.6% expected risk from the nomogram; the cumulative risk of fracture using IMRT at 5 years was 6.7% (95% CI 2.8-16.0%). The median time to fracture was 23 months (range, 6.9-88.6). Significant predictors of fracture on univariate analysis were age ≥ 60 years (p = 0.03), tumor location in the anterior thigh (p = 0.008), and periosteal stripping to > 20 cm (p < 0.0001). On multivariate analysis, age ≥ 60 years and periosteal stripping > 20 cm retained significance (p = 0.04 and p = 0.009, respectively). CONCLUSIONS:In this study, the cumulative risk of femur fracture in patients treated with IMRT (6.7%) is less than the expected risk using the PMH nomogram (25.6%). Established predictors of femur fracture, such as gender, tumor size, and dose of RT, seem to have less impact on fracture risk when using IMRT.
PMCID:6458075
PMID: 30706225
ISSN: 1534-4681
CID: 5293612

How can we increase Pulmonary Rehabilitation (PR) uptake and outcomes in COPD patients? [Meeting Abstract]

Apps, Michael; Keeling, Kelly Ann; Goodrich, Carole; Olympio-Anang, Honoria; Young, Irene; Kopaxz, Anna; Fabbri, Nicola; Gisby, Tracy; Minter, Jan; Sanger, Kiran; Champion, Louise
ISI:000507372407266
ISSN: 0903-1936
CID: 5294062

Should the Use of Biologic Agents in Patients With Renal and Lung Cancer Affect Our Surgical Management of Femoral Metastases?

Gutowski, Christina J; Zmistowski, Benjamin; Fabbri, Nicola; Boland, Patrick J; Healey, John H
BACKGROUND:Biologic agents may prolong survival of patients with certain kidney and lung adenocarcinomas that have metastasized to bone, and patient response to these agents should be considered when choosing between an endoprosthesis and internal fixation for surgical treatment of femoral metastases. QUESTIONS/PURPOSES:Among patients undergoing surgery for femoral metastases of lung or renal cell carcinoma, (1) Does survival differ between patients who receive only cytotoxic chemotherapy and those who either respond or do not respond to biologic therapy? (2) Does postsurgical incidence of local disease progression differ between groups stratified by systemic treatment and response? (3) Does implant survival differ among groups stratified by systemic treatment and response? METHODS:From our institutional longitudinally maintained orthopaedic database, patients were identified by a query initially identifying all patients who carried a diagnosis of renal cell carcinoma or lung carcinoma. Patients who underwent internal fixation or prosthetic reconstruction between 2000 and 2016 for pathologic fracture of the femur and who survived ≥ 1 year after surgery were studied. Patients who received either traditional cytotoxic chemotherapy or a biologic agent were included. Patients were classified as responders or nonresponders to biologic agents based on whether they had clinical and imaging evidence of a response recorded on two consecutive office visits over ≥ 6 months. Endpoints were overall survival from the time of diagnosis, survival after the femoral operation, evidence of disease progression in the femoral operative site, and symptomatic local disease progression for which revision surgery was necessary. Our analysis included 148 patients with renal (n = 26) and lung (n = 122) adenocarcinoma. Fifty-one patients received traditional chemotherapy only. Of 97 patients who received a biologic agent, 41 achieved a response (stabilization/regression of visceral metastases), whereas 56 developed disease progression. We analyzed overall patient survival with the Kaplan-Meier method and used the log-rank test to identify significant differences (p < 0.05) between groups. RESULTS:One-year survival after surgery among patients responsive to biologic therapy was 61% compared with 20% among patients nonresponsive to biologics (p < 0.001) and 10% among those who received chemotherapy only (p < 0.009). With the number of patients we had to study, we could not detect any difference in local progression of femoral disease associated with systemic treatment and response. Radiologic evidence of periimplant local disease progression developed in three (7%) of 41 patients who responded to biologic treatment, two (3%) of 56 patients nonresponsive to biologics, and one (2%) of 51 patients treated with traditional chemotherapy. With the numbers of patients we had, we could not detect a difference in patients who underwent revision. All three patients responsive to biologics who developed local recurrence underwent revision, whereas the two without a response to biologics did not. CONCLUSIONS:Biologic therapy improves the overall longevity of some patients with lung and renal metastases to the femur in whom a visceral disease response occurred. In our limited cohort, we could not demonstrate an implant survival difference between such patients and those with shorter survival who may have had more aggressive disease. However, an increased life expectancy beyond 1 year among patients responsive to biologics may increase risk of mechanical failure of fixation constructs. LEVEL OF EVIDENCE:Level III, therapeutic study.
PMID: 30811363
ISSN: 1528-1132
CID: 5293622

STAGED REVISION FOR INFECTED RECONSTRUCTION AFTER LIMB SALVAGE SURGERY FOR BONE TUMORS [Meeting Abstract]

Fabbri, Nicola; Donati, D; Giacomini, S; Manfrini, M; Mercuri, M
ORIGINAL:0016199
ISSN: 1358-992x
CID: 5354772