Fifteen patients were identified with histologically confirmed esthesioneuroblastoma who were treated between 1994 and 2007. The patients ranged in age from 19 to 71 years (Fig. ) with 4 women and 11 men. Thirteen of the patients were Caucasian with one patient of Asian ancestry and one of Hispanic ancestry. Most patients presented with nasal obstruction (eight patients, 53%) or epistaxis (six patients, 40%). Other symptoms included asymptomatic nasal mass noted on exam in four (27%) patients, and one patient (7%) with each of the following presenting complaints: cushingoid appearance, hyponatremia, sinusitis, proptosis, ecchymosis, epiphora, hyposmia, and behavioral changes due to frontal lobe involvement (Fig. ). At the time of presentation, one patient had disease in the neck. No patient presented with a tumor isolated to the nasal cavity (Kadish stage A). Thirteen patients presented with disease involving at least one paranasal sinus (Kadish stage B) and two patients with disease that extended beyond the paranasal sinuses (Kadish stage C; Fig. ).
The age distribution of patients with esthesioneuroblastoma.
Kadish stage of patients with esthesioneuroblastoma.
The initial treatment was surgery alone for nine patients, surgery followed by postoperative radiation treatment in five patients, and surgery followed by postoperative chemotherapy and radiation in one patient (Fig. ). All patients underwent complete gross surgical and radiographic excision of tumor. Two patients had positive microscopic surgical margins. The mean and median follow-up times were 75 and 64 months, respectively, with a range of 2 to 240 months. All patients are still living. Eleven patients are currently alive without disease, one patient is alive with disease, and three patients are alive with an unknown disease status.
The initial treatment used in this series of patients.
Eight patients had recurrence of disease (Fig. ). Seven of these eight patients recurred locally and three had regional recurrence in the neck. One of the two patients with positive surgical margins recurred. Of the three patients with regional recurrence, two also had local recurrence. Interestingly, the patient with isolated regional recurrence was the only one of the eight patients who recurred that received immediate postoperative radiation therapy (the neck was not a target of the radiation treatment). Of the eight patients who recurred, all but one received surgery alone as the initial treatment. All recurrences were treated with surgical resection followed by radiation therapy with the exception of one patient with isolated regional disease who was treated with neck dissection alone.
The mean time to recurrence was 82.1 months (95% confidence interval, 48.8 to 115.4 mos) and the overall recurrence-free survival was 49% at 5 years and 24% at 15 years (Fig. ). There was no difference in disease recurrence between genders. Patients who presented with Kadish stage B had 5- and 15-year disease-free survival of 57%, whereas Kadish stage C patients had 5- and 15-year disease-free survival of 30% and 0%, respectively (Fig. ).
Overall recurrence-free survival in months.
Recurrence-free survival by stage (stage B, solid line; stage C, broken line) in months.
Patients treated with surgery and postoperative radiation therapy fared better than those treated with surgery alone (Fig. ). Patients who were treated with surgery alone had a mean recurrence time of 57.5 months (95% confidence interval, 17.0 to 98.1 mos), a 26.7% 5-year recurrence-free survival, and a 0% 15-year recurrence-free survival. Patients whose initial treatment was surgical resection followed by radiation therapy had a mean recurrence time of 122 months (95% confidence interval, 79.1 to 164.9 mos) and 5- and 15-year recurrence-free survival of 83.3%. Two patients received chemotherapy and each received this treatment at outside hospitals. One of the patients received three cycles of cisplatin and etoposide postoperatively in combination with radiation therapy. The second patient had esthesioneuroblastoma in combination with neuroendocrine carcinoma and received chemotherapy and radiation therapy postoperatively.
Recurrence-free survival by initial treatment (solid line, surgery only; broken line, surgery with postoperative radiation) in months.
Functionally, patients who underwent subcranial resection maintained their preoperative functional status at 1, 6, and 12 months postoperatively (Table ). One of the patients who recurred received whole-brain radiation and had a subsequent decline in Karnofsky Performance Score.
Karnofsky Performance Scores for Patients Preop and at 1, 6, and 12 Months Postoperatively
A total of nine complications were encountered. Two patients had cerebrospinal fluid leaks that resolved with conservative treatment, two patients had transient epiphora, two patients had postoperative diplopia, and one patient each had hydrocephalus, non-tension pneumocephalus, and a wound infection. The patient with hydrocephalus had a complicated medical history with a prolonged postoperative course and eventually required ventriculoperitoneal shunt placement for treatment of the hydrocephalus. The non-tension pneumocephalus was induced by nose-blowing and developed after the patient was discharged from the hospital. This patient was successfully treated with aspiration of the air and application of a compressive dressing. There was one patient who developed postoperative tension pneumocephalus and was treated successfully with tracheostomy and aspiration of the air.