title: Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine.
abstract: The esthesioneuroblastoma is a rare neuroendocrine tumor that derives from the olfactory cells. In the last 20 years, around 1,000 cases have been described, with an overall survival rate of 60-70% at 5 years. The most common symptoms are nasal bleeding, nasal clogging and, in locally advanced cases, signs/symptoms of intracranic hypertension such as papilla edema, cefalea, and vomiting. The standard treatments are surgery and radiotherapy. Chemotherapy can be used in an adjuvant/neoadjuvant setting and in the metastatic phase, even if its role is still not established with certainty. Here, the case is reported of a young man (38 years old) with a locally advanced esthesioneuroblastoma. Two months before coming to our clinic, he had been treated elsewhere with debulking surgery through bilateral frontal craniotomy. After surgery, MRI showed residual disease in the nasal cavities and in the medial wall of the orbits responsible for blindness and bilateral exophthalmos within a month: a very short time. Octreoscan and whole body CT scan confirmed a locally advanced disease, in the absence of metastases. Chemotherapy was begun with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine with granulocyte colony-stimulating factor (G-CSF) support after every cycle. Soon after the first cycle, an important reduction of pain and decrease of the exophthalmos and vertigos was observed. No improvement in blindness was seen. The patient is still stable after 24 months of follow up.
authors.affiliation: Mariano Santo Hospital, UOC Oncologia Medica Azienda Ospedaliera Cosenza, Cosenza, Italy. galactus76@yahoo.it
authors.forename: Salvatore
authors.initials: S
authors.lastname: Turano
authors.forename: Candida
authors.initials: C
authors.lastname: Mastroianni
authors.forename: Caterina
authors.initials: C
authors.lastname: Manfredi
authors.forename: Rosalbino
authors.initials: R
authors.lastname: Biamonte
authors.forename: Silvia
authors.initials: S
authors.lastname: Ceniti
authors.forename: Virginia
authors.initials: V
authors.lastname: Liguori
authors.forename: Rosanna
authors.initials: R
authors.lastname: De Simone
authors.forename: Serafino
authors.initials: S
authors.lastname: Conforti
authors.forename: Aldo
authors.initials: A
authors.lastname: Filice
authors.forename: Antonio
authors.initials: A
authors.lastname: Rovito
authors.forename: Caterina
authors.initials: C
authors.lastname: Viscomi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Patitucci
authors.forename: Salvatore
authors.initials: S
authors.lastname: Palazzo
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002945:Cisplatin; D003131:Combined Modality Therapy / Q000379:methods*; D004317:Doxorubicin; D004701:Endocrine Gland Neoplasms / Q000188:drug therapy*; D005047:Etoposide; D006801:Humans; D007069:Ifosfamide; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009447:Neuroblastoma / Q000188:drug therapy*; D014057:Tomography, X-Ray Computed; D014750:Vincristine
chemical_list: D000970:Antineoplastic Agents; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D002945:Cisplatin; D007069:Ifosfamide