Kempf H G, Becker G, Weber B P, Ruck P
HNO-Klinik der Medizinischen Hochschule, Hannover.
HNO. 1994 Jul;42(7):422-8.
Esthesioneuroblastomas (EN) exhibit problems in early diagnosis and therapy due to their localization at the frontal skull base. Analysis of six cases with EN (four male, two female; average age, 34.8 years) showed atypical initial symptoms, beginning as nasal bleeding, hyposmia and frontal headache. CT scans demonstrated hypo- to isodense tumors at the anterior skull base with extension to the sinuses and orbits. Five patients were operated on by an extranasal approach; one patient required orbital exenteration with later reconstructive surgery of the orbit by a microvascularly adapted forearm flap. One patient underwent a neurosurgical procedure first that was followed by chemotherapy and stereotactically guided radiation. One patient died 1 year after onset of therapy due to intracranial tumor. One patient developed lung metastasis 5 years after treatment. Four patients remain in clinical remission and receive regular follow-ups. Our analysis shows the guarded prognosis of EN despite multimodality therapy. This includes the problems of advanced disease with complications of surgery and radiation. All therapeutic procedures should be planned in collaboration with otolaryngologists, neurosurgeons and radiotherapists. New computer-aided and stereotactically guided radiation procedures can be helpful, especially in patients with extensive disease.
嗅神经母细胞瘤（EN）因其位于额颅底的位置而在早期诊断和治疗方面存在问题。对6例EN患者（4例男性，2例女性；平均年龄34.8岁）的分析显示，其初始症状不典型，起初表现为鼻出血、嗅觉减退和额部头痛。CT扫描显示前颅底有低密度至等密度肿瘤，并延伸至鼻窦和眼眶。5例患者采用鼻外入路手术；1例患者需要行眼眶内容剜除术，随后采用微血管吻合的前臂皮瓣对眼眶进行重建手术。1例患者首先接受了神经外科手术，随后进行化疗和立体定向放射治疗。1例患者在治疗开始后1年因颅内肿瘤死亡。1例患者在治疗后5年发生肺转移。4例患者仍处于临床缓解期并接受定期随访。我们的分析表明，尽管采用了多模式治疗，EN的预后仍不容乐观。这包括晚期疾病的问题以及手术和放疗的并发症。所有治疗程序都应在与耳鼻喉科医生、神经外科医生和放疗科医生合作的情况下进行规划。新的计算机辅助和立体定向放射治疗程序可能会有所帮助，尤其是对于患有广泛疾病的患者。
