[A case of olfactory neuroblastoma with intracranial, intraorbital extension and multiple metastases]

A 51-year-old man presented with headache, vomiting and exophthalmus. Neurological examination revealed anosmia, papilledema, decrease in visual acuity, and disability in ocular movement. MRI showed a huge mass which occupied the whole nasal cavity and compressed the frontal lobe upwards and the eyes laterally. CT revealed an extensive bony destruction of the frontal base and bilateral orbits. The mass was biopsied transnasally, and was histologically diagnosed as olfactory neuroblastoma. It was highly radiosensitive and disappeared with a local irradiation of 40 Gy. Three months later the patient complained of a pain radiating from the neck to the right arm. MRI demonstrated a metastasis at the vertebral body of C5. Local irradiation of 30 Gy was performed. The metastatic lesion was removed, and a bone graft taken from the iliac bone was transplanted via an anterior cervical approach. Three weeks later, however, a hard mass appeared in the right of his neck and was surgically removed. By histological examination, it was also identified as a metastatic neuroblastoma to the cervical lymph node. A week after the removal of the cervical metastatic lesion, the metastasis extended rapidly to the left cervical and the bilateral hilar lymph nodes of the lungs. Chemotherapy was performed with a total doses of 800mg of cyclophosphamide, 1.5mg of vincristine, 40mg of pirarubicin, and 80mg of cisplatin. The lesions disappeared within 7 days. However, the patient died from disseminated intravascular coagulation 10 months after the onset. Olfactory neuroblastoma is usually an intranasal neoplasm, but it rarely extends intracranially and intraorbitally as is shown in our case. Basically, olfactory neuroblastoma is a relatively slow-growing tumor though it has a tendency to develop local recurrences over long periods even after aggressive primary treatment, and accompanied with distant metastases. However, our patient showed a very short survival time. Invasive extension and multiple metastases occurred during a short period, followed by disseminated intravascular coagulation. Combined chemotherapy at the initial treatment may be recommended in such an extensive case.