Complete response of CNS-involved olfactory neuroendocrine tumor using multimodal therapy

Locally advanced tumors in the olfactory area commonly have central nervous system (CNS) involvement and are often incurable.

We report the treatment of a 25-year-old male patient who presented with a large, neuroendocrine tumor arising from the ethmoid complex. This locally invasive lesion extended beyond the orbits and into the anterior cranial fossa with associated intracranial involvement. An endoscopic nasal biopsy demonstrated a poorly differentiated neuroendocrine tumor. The patient was treated with 4 cycles of combination induction chemotherapy with irinotecan and cisplatin followed by radiation therapy with concurrent weekly cisplatin. He had a complete response to this therapy confirmed with biopsies that demonstrated no residual disease at 8 weeks after chemoradiotherapy. He remained disease free one and a half years following surgery.

To our knowledge, this combination treatment approach has not been reported in the literature.