嗅觉神经母细胞瘤:在澳大利亚三级中心14年的经验和长期监测的作用。

C. Schmidt, N. Potter, S. Porceddu, B. Panizza
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引用次数: 9

摘要

背景:鼻腔神经母细胞瘤是一种罕见的鼻窦恶性肿瘤,治疗方案不明确。回顾性评估三级中心的管理,以告知未来的治疗和随访。方法选取2000-2014年收治的有治愈意向的病例。收集数据,计算总生存率和无病生存率。结果共发现6例,中位随访87个月。1例为卡迪什A期,1例为B期,8例为C期,1例为d期。后1例患者单独接受放化疗。其余患者进行内镜辅助下广泛局部切除术(n = 2),前颅面切除术(n = 4)或内镜颅面切除术(n = 4)。没有患者患有原发性淋巴结疾病或选择性颈部治疗。1例患者行新辅助放化疗。术后放疗6例;其中3人接受了辅助化疗。2例患者发生迟发性宫颈结衰竭,行颈部清扫术及术后放疗。2例局部晚期复发。十年总体生存率和无病生存率分别为68.2%和46.7%。结论考虑到局部和晚期复发的发生率,支持长期随访。局部晚期宫颈淋巴结的预防性治疗是一个有待进一步研究的领域。
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Olfactory neuroblastoma: 14-year experience at an Australian tertiary centre and the role for longer-term surveillance.
BACKGROUND Olfactory neuroblastoma is a rare sinonasal malignancy, with poorly defined treatment protocols. Management at a tertiary centre was retrospectively evaluated to inform future treatment and follow up. METHODS Cases treated with curative intent (2000-2014) were included. Data were collected, and overall and disease-free survival rates were calculated. RESULTS Eleven cases were identified, with a median follow up of 87 months. One patient was Kadish stage A, one was stage B, eight were stage C and one was stage D. The latter patient underwent chemoradiotherapy alone. The remaining patients proceeded to: endoscopic-assisted wide local excision (n = 2), anterior craniofacial resection (n = 4) or endoscopic craniofacial resection (n = 4). No patients had primary nodal disease or elective neck treatment. One patient had neoadjuvant chemoradiation. Six patients had post-operative radiotherapy; three received adjuvant chemotherapy. Two patients had late cervical node failure, and proceeded to neck dissection and post-operative radiotherapy. Two patients had late local recurrence. Ten-year overall and disease-free survival rates were 68.2 and 46.7 per cent, respectively. CONCLUSION Longer-term follow up is supported given the incidence of late regional and local recurrence. Prophylactic treatment of cervical nodes in locally advanced disease is an area for further investigation.
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