olfactory neuroblastoma (ONB)

嗅觉神经母细胞瘤 (ONB)
  • 文章类型: Journal Article
    低分化的鼻窦癌(PDC)是尽管经典治疗方法取得了进展,但预后较差的肿瘤。预测和预后标志物以及新的个性化治疗可以改善患者的肿瘤预后。在这项研究中,我们分析了SOX2和βIII-微管蛋白作为可能对这些肿瘤具有预后和治疗影响的生物标志物.该队列包括57例PDC:36例鼻腔鼻窦未分化癌(SNUC),13例嗅神经母细胞瘤(ONB),鼻窦神经内分泌癌(SNEC)8例。其中26例的临床随访数据可用。在6例(75%)SNEC病例中,采用免疫组化法检测Sox2表达,19(53%)SNUC病例,6例(46%)ONB病例。没有Sox2染色与较高的复发率相关(p=0.015),尤其是远处复发。大多数病例显示βIII-微管蛋白表达,85%的人有很强的积极性,75%,SNEC的64%,ONB,和SNUC案例,分别。具有更强βIII-微管蛋白表达的肿瘤显示出比无表达或低表达的肿瘤更长的无病生存期(p=0.049)。Sox2和βIII-微管蛋白表达在低分化的鼻窦肿瘤中很常见,具有预后和治疗作用。
    Poorly differentiated sinonasal carcinomas (PDCs) are tumors that have a poor prognosis despite advances in classical treatment. Predictive and prognostic markers and new personalized treatments could improve the oncological outcomes of patients. In this study, we analyzed SOX2 and βIII-tubulin as biomarkers that could have prognostic and therapeutic impacts on these tumors. The cohort included 57 cases of PDCs: 36 sinonasal undifferentiated carcinoma (SNUC) cases, 13 olfactory neuroblastoma (ONB) cases, and 8 sinonasal neuroendocrine carcinoma (SNEC) cases. Clinical follow-up data were available for 26 of these cases. Sox2 expression was detected using immunohistochemistry in 6 (75%) SNEC cases, 19 (53%) SNUC cases, and 6 (46%) ONB cases. The absence of Sox2 staining correlated with a higher rate of recurrence (p = 0.015), especially distant recurrence. The majority of cases showed βIII-tubulin expression, with strong positivity in 85%, 75%, and 64% of SNEC, ONB, and SNUC cases, respectively. Tumors with stronger βIII-tubulin expression demonstrated longer disease-free survival than those with no expression or low expression (p = 0.049). Sox2 and βIII-tubulin expression is common in poorly differentiated sinonasal tumors and has prognostic and therapeutic utility.
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  • 文章类型: Comparative Study
    这项研究的目的是比较混合调强放射治疗(IMRT)和体积调强放射治疗(混合IMRT/VMAT),非共面(nc)IMRT和nc-VMAT治疗不可切除的嗅神经母细胞瘤(ONB)。混合IMRT/VMAT,nc-IMRT和nc-VMAT计划优化为12例改良KadishC期ONB患者。剂量处方为65Gy,分为26个部分。剂量-体积直方图参数,构象数(CN),同质性指数(HI),评估每个部分递送的积分剂量和监测单位(MU)。还评估了基于EUD模型(NTCPLogit)和Lyman-Kutcher-Burman模型(NTCPLKB)的等效均匀剂量(EUD)和正常组织并发症概率(NTCP)。我们发现,与nc-VMAT计划相比,混合IMRT/VMAT计划显着提高了临床目标体积(CTV)和计划治疗体积(PTV)的CN。总的来说,保存有风险的器官(OAR)与这三种技术相似,尽管与nc-IMRT计划相比,混合IMRT/VMAT计划导致对侧(C/L)视神经的Dmax显着降低。与nc-IMRT计划和nc-VMAT计划相比,混合IMRT/VMAT计划显着降低了同侧(I/L)和C/L视神经的EUD。但是三种技术之间的NTCP差异<1%。我们得出结论,混合IMRT/VMAT技术可以在视神经的目标一致性和EUD方面提供改进,与nc-IMRT和nc-VMAT相比,在实现相等或更好的OAR保留的同时,并且可以是用于治疗不可切除的ONB的可行的辐射技术。然而,剂量测定数据中这些微小差异的临床益处,视神经的EUD和NTCP可能最小。
    The purpose of this study was to compare hybrid intensity-modulated radiotherapy (IMRT) and volumetric-modulated arc therapy (Hybrid IMRT/VMAT), with non-coplanar (nc) IMRT and nc-VMAT treatment plans for unresectable olfactory neuroblastoma (ONB). Hybrid IMRT/VMAT, nc-IMRT and nc-VMAT plans were optimized for 12 patients with modified Kadish C stage ONB. Dose prescription was 65 Gy in 26 fractions. Dose-volume histogram parameters, conformation number (CN), homogeneity index (HI), integral dose and monitor units (MUs) delivered per fraction were assessed. Equivalent uniform dose (EUD) and normal tissue complication probability (NTCP) based on the EUD model (NTCPLogit) and the Lyman-Kutcher-Burman model (NTCPLKB) were also evaluated. We found that the Hybrid IMRT/VMAT plan significantly improved the CN for clinical target volume (CTV) and planning treatment volume (PTV) compared with the nc-VMAT plan. In general, sparing of organs at risk (OARs) is similar with the three techniques, although the Hybrid IMRT/VMAT plan resulted in a significantly reduced Dmax to contralateral (C/L) optic nerve compared with the nc-IMRT plan. The Hybrid IMRT/VMAT plan significantly reduce EUD to the ipsilateral (I/L) and C/L optic nerve in comparison with the nc-IMRT plan and nc-VMAT plan, but the difference in NTCP between the three technique was <1%. We concluded that the Hybrid IMRT/VMAT technique can offer improvement in terms of target conformity and EUD for optic nerves, while achieving equal or better OAR sparing compared with nc-IMRT and nc-VMAT, and can be a viable radiation technique for treating unresectable ONB. However, the clinical benefit of these small differences in dosimetric data, EUD and NTCP of optic nerves may be minimal.
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  • 文章类型: Journal Article
    UNASSIGNED: To report the clinical experience and short-term efficacy in the management of olfactory neuroblastoma (ONB).
    UNASSIGNED: We performed a retrospective analysis of 12 ONB patients treated with particle beam radiation therapy (PBRT) between 12/2015 and 5/2019 at the Shanghai Proton and Heavy Ion Center. Four (33.3%) patients presented with Kadish B ONB, and 8 (66.7%) presented with Kadish C or D disease. Eleven patients received proton radiotherapy (PRT) followed by a carbon ion radiotherapy (CIRT) boost, one patient received CIRT only. The 2-year survival rates were calculated using the Kaplan-Meier method. Acute and late adverse events were summarized and scored according to the CTCAE (version 4.03).
    UNASSIGNED: With a median follow-up of 17.5 (range, 2.53-49.9) months, all patients but 1 were alive. Eight patients were alive without evidence of disease, and 2 additional patients achieved partial response and remained alive with residual disease. One patient died of toxicity associated with salvage chemotherapy for distant metastasis and local failure. Another patient developed distant metastasis only and was alive at the time of the last follow-up. The 2-year OS, PFS, LRPFS, and DMFS rates were 83.3%, 75.8%, 87.5%, and 79.5%, respectively. No acute or late toxicities of ≥ grade 3 was observed.
    UNASSIGNED: Intensity modulated PBRT of ONB is well tolerated. While longer follow-up is needed, early outcomes suggested that PBRT is safe and effective for the treatment of ONB with minimal adverse events.
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