• esthesioneuroblastoma
• olfactory neuroblastoma

Esthesioneuroblastoma (ENB) is an uncommon malignant tumor of the upper nasal cavity and anterior skull base and accounts for approximately 2-6% of nasal cavity and paranasal sinus cancer. The mean time from onset of symptoms to diagnosis of ENB ranges between 6–12 months. For this reason, many patients have advanced stage at the time of diagnosis. Physical exam and flexible fiber-optic endoscopic evaluation, complemented with both contrast-enhanced CT scan and MR imaging, represent the key in the diagnostic workup. Particular attention should be dedicated to the neck examination considering a significant percentage of patients (5-20%) have neck metastases at presentation. Several staging systems have been proposed, but there is still no consensus on the best staging system for ENB. The three options used for the treatment of esthesioneuroblastoma are surgery, external beam radiation, and chemotherapy. Often, treatment utilizes a combination of these modalities; treatment depends on the local tumor stage and whether or not there is regional or distant metastatic disease. ENB has excellent survival outcomes with the reported 5-year overall survival is between 57–93%. Despite the high rate of survival, ENB has a high recurrence rate, thus patients require long-term follow-up, up to 10-15 years. Locoregional recurrence represents the most common failure, taking place within the first 5-10 years of clinical follow-up. The risk of loco-regional recurrence was higher among those patients presenting poor prognostic factors such as high Hyams grade, advanced Kadish staging, intracranial extension, and positive resection margins.
The purpose of this review was to give an update of the most recent literatures’ results about ENB management, including a reliable discussion about indications to neck disease management and salvage procedures indications, in order to provide a comprehensive overview of the most recent advances in the treatment of this rare pathological condition.
In the meta-analysis were included 32 articles that reported outcome of 3180 patients. Of these, 44,24% was treated with pure endoscopic technique and 30,44% with open surgery. Study periods range from 84-420 months (median 204 months) and median age was 49 years (range 14-61). The majority, 55,5 % was Kadish stage C. 5,62% of study population presented at diagnosis with neck metastasis. Median overall survival (OS) was 80% at 5-years; disease free survival (DFS) for endoscopic and open surgery was 82,4% vs 70,7% at 5 years, respectively. At 10 years of follow-up, DFS was 75,3% for endoscopic surgery vs 57,1% in open technique. 10 years OS was 71,3%. Local recurrence, independently from the type of surgery treatment occurred in 161 (5,06%) patients and neck recurrence in 135 cases (4,24%).