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鼻腔鼻窦黑色素瘤的预后:临床经验和文献复习。

Outcome of sinonasal melanoma: clinical experience and review of the literature.

机构信息

Department of Otorhinolaryngology, University Hospital of Zurich, Switzerland.

出版信息

Head Neck. 2010 Oct;32(10):1385-92. doi: 10.1002/hed.21340.

Abstract

BACKGROUND

Primary sinonasal malignant melanoma (SNMM) is a rare clinical entity. There is neither a classification nor a staging system nor an evidence-based treatment concept established. Our objective was to find potential risk factors predicting the outcome.

METHODS

Twenty-five patients with histologically confirmed SNMM were consecutively included and retrospectively analyzed. Staging methods were nasal endoscopy, CT, MRI, and positron emission tomography (PET) scan. Patients were selected for a curative or palliative concept. All patients had postoperative follow-up with control-MRI at 3 and 6 months. Restaging was performed when local recurrence occurred.

RESULTS

Nineteen patients underwent primary surgery with curative intention; in 16 cases with tumor free margins. Thirteen patients (68%) had transnasal endoscopic surgery, 4 lateral rhinotomy, and 2 transfacial approach with orbital exenteration. Six patients (32%) had palliative therapy and 7 patients (37%) had adjuvant radiotherapy. Despite radical operations, 6 patients (37%) showed local recurrence and 8 patients (50%) developed distant metastasis. In 2 patients with incomplete surgery, regional metastasis was noted. The median disease-free interval was 18 months, and the median overall survival rate was 23 months.

CONCLUSION

SNMMs of the ethmoid and maxillary sinuses have a worse prognosis than other localizations in the nasal cavity; infiltration into the skull base, orbit, or facial soft tissue correlates with a very poor outcome corresponding to the palliative situations. Furthermore, local recurrence insinuates aggressive disease with short survival rate. A main difference from its cutaneous counterpart seems to be a primary tendency to hematogenic spread. Further research is needed to confirm these findings.

摘要

背景

原发性鼻腔鼻窦恶性黑色素瘤(SNMM)是一种罕见的临床实体。目前既没有分类系统,也没有分期系统,更没有基于循证的治疗概念。我们的目的是寻找潜在的风险因素来预测其预后。

方法

连续纳入 25 例经组织学证实的 SNMM 患者进行回顾性分析。分期方法包括鼻内镜、CT、MRI 和正电子发射断层扫描(PET)扫描。根据治疗目的选择患者进行根治性或姑息性治疗。所有患者术后均接受随访,术后 3 个月和 6 个月行 MRI 检查。局部复发时进行再次分期。

结果

19 例患者接受了根治性手术,其中 16 例切缘无肿瘤。13 例患者(68%)采用经鼻内镜手术,4 例患者采用外侧鼻切开术,2 例患者采用经面中部入路联合眶内容物切除术。6 例患者(32%)采用姑息性治疗,7 例患者(37%)接受辅助放疗。尽管进行了根治性手术,仍有 6 例患者(37%)出现局部复发,8 例患者(50%)出现远处转移。2 例患者手术不完全,出现区域性转移。无病间隔中位数为 18 个月,总生存率中位数为 23 个月。

结论

筛窦和上颌窦的 SNMM 比鼻腔其他部位的预后差;侵犯颅底、眼眶或面部软组织与极差的预后相关,对应姑息治疗情况。此外,局部复发暗示疾病具有侵袭性,生存时间短。与皮肤黑色素瘤相比,其主要区别似乎是原发性倾向于血行播散。需要进一步的研究来证实这些发现。

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