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Primary bone sarcoma with BCOR internal tandem duplication.


  • 影响因子:2.52
  • DOI:10.1007/s00428-019-02729-z
  • 作者列表:"Malik F","Zreik RT","Hedges DJ","Nakitandwe J","Lee S","Ward RA","McCarville MB","Pappo A","Bahrami A
  • 发表时间:2020-06-01

:BCOR internal tandem duplications (ITDs) and rearrangements are implicated in the oncogenesis of a subset of undifferentiated sarcomas. To date, BCOR ITD sarcomas have been exclusively found in non-appendicular infantile soft tissues, whereas BCOR-rearranged sarcomas occur in both bones and soft tissues affecting a wider patient age range. Little is known about patient outcome in BCOR ITD sarcomas. We present a BCOR-expressing, primary bone, undifferentiated sarcoma case involving an adolescent male's left tibia that, unexpectedly, harbored a BCOR ITD instead of a BCOR rearrangement. Furthermore, the patient achieved a partial histologic response after receiving a Ewing sarcoma chemotherapy regimen. Our case expands the clinical spectrum of BCOR ITD sarcomas and suggests that childhood and adult BCOR-expressing sarcomas with an undifferentiated histology should be considered for both BCOR rearrangement and ITD screening. Accurate BCOR mutation identification in undifferentiated sarcomas is essential to define their clinical spectrum and to develop effective management strategies.


: BCOR内部串联重复 (itd) 和重排与未分化肉瘤子集的肿瘤发生有关。迄今为止,BCOR ITD肉瘤仅见于非阑尾婴儿软组织,而BCOR重排肉瘤发生于骨骼和软组织,影响更大的患者年龄范围。对BCOR ITD肉瘤的患者结局知之甚少。我们介绍了一例BCOR表达的原发性骨未分化肉瘤病例,涉及一名青少年男性的左胫骨,意外地携带BCOR ITD而不是BCOR重排。此外,患者在接受尤文肉瘤化疗方案后获得了部分组织学缓解。我们的病例扩大了BCOR ITD肉瘤的临床谱,并提示在BCOR重排和ITD筛查中应考虑儿童期和成人BCOR表达的未分化组织学肉瘤。未分化肉瘤中准确的BCOR突变鉴定对于确定其临床谱并制定有效的管理策略至关重要。



作者列表:["Tran S","Puric E","Walser M","Poel R","Datta NR","Heuberger J","Pica A","Marder D","Lomax N","Bolsi A","Morach P","Bachtiary B","Seddon BM","Schneider R","Bodis S","Weber DC"]

METHODS:OBJECTIVE:Large inoperable sacral chordomas show unsatisfactory local control rates even when treated with high dose proton therapy (PT). The aim of this study is assessing feasibility and reporting early results of patients treated with PT and concomitant hyperthermia (HT). METHODS: :Patients had histologically proven unresectable sacral chordomas and received 70 Gy (relative biological effectiveness) in 2.5 Gy fractions with concomitant weekly HT. Toxicity was assessed according to CTCAE_v4. A volumetric tumor response analysis was performed. RESULTS: :Five patients were treated with the combined approach. Median baseline tumor volume was 735 cc (range, 369-1142). All patients completed PT and received a median of 5 HT sessions (range, 2-6). Median follow-up was 18 months (range, 9-26). The volumetric analysis showed an objective response of all tumors (median shrinkage 46%; range, 9-72). All patients experienced acute Grade 2-3 local pain. One patient presented with a late Grade 3 iliac fracture. CONCLUSION:Combining PT and HT in large inoperable sacral chordomas is feasible and causes acceptable toxicity. Volumetric analysis shows promising early results, warranting confirmation in the framework of a prospective trial. ADVANCES IN KNOWLEDGE: :This is an encouraging first report of the feasibility and early results of concomitant HT and PT in treating inoperable sacral chordoma.

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作者列表:["Gyori DJ","Bullington SM","Crawford BS","Vernon VP"]

METHODS:BACKGROUND:National guidelines recommend screening and treatment for cancer-related bone disease and continued monitoring of bone-modifying agents. It is unclear whether a standardized screening tool is utilized to identify eligible patients and ensure appropriate supportive care is implemented. The purpose of this study was to evaluate current prescribing practices and optimize management of bone-modifying agents. METHODS:A retrospective chart review was performed to identify patients who received hormone deprivation therapy or had bone metastases through Hematology/Oncology or Urology clinics from 1 November 2016 to 31 October 2017. The primary endpoints of this study were the incidence of completed baseline dual-energy X-ray absorptiometry (DEXA) scan for patients on hormone deprivation therapy and percent of patients started on a bone-modifying agent for the prevention of skeletal-related events secondary to bone metastasis. Secondary endpoints included percent of patients with dental examinations prior to initiation, adequate calcium and vitamin D supplementation, incidence of osteonecrosis of the jaw or flu-like symptoms and education, and percent of bisphosphonate doses appropriately adjusted based on renal function. RESULTS:A total of 375 patients were assessed for baseline DEXA scans and bone-modifying therapy. Of the 226 patients on hormone deprivation therapy, 111 (49%) patients were appropriately screened with a DEXA scan prior to initiation of hormone deprivation therapy. Among the 149 patients with bone metastases, only 94 (63.1%) patients were started on a bone-modifying agent. CONCLUSIONS:Opportunities have been identified to optimize management of patients with cancer-related bone disease. Implementation of standardized tools may increase the rate of appropriate screening and initiation of bone-modifying therapy when warranted.

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作者列表:["Dohzono S","Sasaoka R","Takamatsu K","Hoshino M","Nakamura H"]

METHODS:PURPOSE:Low skeletal muscle mass has been associated with poor prognosis in patients with advanced lung cancer. However, little is known about the relationship between skeletal muscle mass and overall survival in patients with bone metastases from lung cancer. The objective of the present study was to evaluate the prognostic value of low trunk muscle mass in predicting overall survival in these patients. METHODS:The data from 198 patients who were diagnosed with bone metastases from lung cancer from April 2009 to May 2017 were retrospectively reviewed. The areas of the psoas and paravertebral muscles were measured at the level of the third lumbar vertebra on computed tomography scans taken at the time nearest to the diagnosis of bone metastasis. Muscle area was evaluated for male and female cohorts separately using different cutoff points. Cox proportional hazards analysis was performed to evaluate the factors independently associated with overall survival. RESULTS:The overall survival of patients in the lowest quartile for psoas muscle area or paravertebral muscle area was significantly shorter than that of patients above the 25th percentile for muscle area (p < 0.001). Multivariate analyses showed that paravertebral muscle mass (hazard ratio, 1.73; 95% confidence interval, 1.17-2.56; p = 0.006), epidermal growth factor receptor-targeted therapy, and performance status were independent prognostic factors. CONCLUSIONS:Low paravertebral muscle mass was associated with shorter survival, independently of known prognostic factors.

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