Olfactory neuroblastoma is a rare
tumor originating in the upper nasal cavity. It rarely extends intracranially. We report a clinical case of
olfactory neuroblastoma with intracranial extension and distant
metastasis. A 35-year-old man complained of nasal stuffiness and
bleeding,
headache and
vomiting. Neurological examination showed
anosmia and
papilledema. MRI showed a huge mass that occupied the right nasal and paranasal cavities, and extended into the right frontal base. The
tumor was removed totally and was histologically diagnosed as
olfactory neuroblastoma. About two months after surgery, however, MRI demonstrated a rapid recurrence of the
tumor in the nasal and paranasal cavities and the frontal lobe. Metastatic lesions were also seen in the right cervical lymph nodes.
Chemotherapy was administered using
cisplatin and
etoposide. The
tumor in the frontal lobe shrunk markedly but the other lesion persisted. Whole brain irradiation and local irradiation of the cervical lymph nodes were performed. The
tumors became smaller but did not disappear. MRI demonstrated spinal dissemination. Irradiation of the whole spinal cord was performed. The metastatic lesions disappeared. The patient was discharged without neurological deficits, but died of
pneumonia 15 months after surgery.
Olfactory neuroblastoma is a slow-growing
tumor and is highly radiosensitive, but it rarely extends or develops multiple distant
metastases and seldom shows a short survival time, as in our case. A review of the literature documented responses in patients treated with a
cisplatin-based
drug combination. We recommend systemic control using
cisplatin-based
chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic
olfactory neuroblastoma.