Background. Esthesioneuroblastoma is a rare tumor arising from olfactory epithelium. This retrospective review analyzed the patterns of failure and long term outcome of patients with esthesioneuroblastoma evaluated at a single institution.
Methods. Forty patients with esthesioneuroblastoma were evaluated at the University of Virginia, with a median follow‐up of 130 months. In most cases, treatment consisted of combined‐modality therapy, including radiotherapy and surgery for Stages A and B disease and the addition of chemotherapy for Stage C disease. Fifteen patients received chemotherapy that included cyclophos‐phamide plus vincristine. Thirty‐eight patients received radiotherapy, with a median dose of 50 Gy. Initial surgery for 23 patients included craniofacial resection, whereas the remainder had less extensive surgery (3 had no initial surgery). Five patients were salvaged with high dose chemotherapy and autologous bone marrow transplantation (CTX/BMT).
Results. Actuarial survivals at 5, 10, and 15 years are 78%, 71%, and 65% respectively. Fifty‐five percent of patients failed therapy, and 68% of the failures were locore‐gional. Thirty‐nine percent of recurrences occurred later than 5 years from diagnosis. Three of the five patients were successfully salvaged with CTX/BMT compared with four of seventeen patients who underwent conventional salvage therapy.
Conclusions. Esthesioneuroblastoma is associated with long term survival and late recurrences. Multimodality therapy should be used initially. Durable remissions of failures can be achieved with CTX/BMT.
There is a statistically significant difference between the 5-year disease-free survival for the esthesioneuroblastoma patients and the non-esthesioneuroblastoma patients (90% vs 59.1%; p = 0.028), and aggressive salvage therapy appears to be a more successful option in the esthesioneuroblastoma group of patients.
Sixteen cases of schneiderian-type mucosal papillomas arising in the nasopharynx and oropharynx are reported. The patients include 11 men and 5 women ranging in age from 45 to 79 years (median 62 years). In 12 patients, the papilloma was discovered as an incidental finding, and 2 patients complained of nasal airway obstruction. In the remaining 2 cases, information regarding the cause that led to discovery was unavailable. None of the patients had a prior or concurrent history of sinonasal (schneiderian) papillomas. Histologically, all of the tumors were identical to sinonasal inverted papillomas. Transnasal or transoral surgical excision was the treatment of choice. In 4 of the patients, recurrent tumor occurred within 6 months of initial resection, necessitating additional surgery. Extended follow-up information was available in 9 cases. Eight of the 9 patients are alive and have been free of disease over periods ranging from 15 to 201 months (median 114 months) from diagnosis. One patient was found to have a separate nasopharyngeal squamous cell carcinoma 14 months after the diagnosis of the schneiderian-type papilloma. This patient died secondary to the squamous cell carcinoma 30 months after his initial evaluation.
The near-total laryngectomy can be performed outside the Mayo Clinic with creditable results, with 82% of the patients attaining successful speech, an average of 5.3 months postoperatively. Eighty-nine percent of the speakers have had a mean disease-free survival of 25.5 months. Analysis of larger series from multiple institutions in conjunction with voice analyses of these patients compared to those with tracheoesophageal punctures is needed to confirm our initial enthusiasm for this procedure.
Within the first 72 hours after surgery, no complications were observed in our study groups. Patients with BMIs greater than 35 were at increased risk for postoperative desaturations. The uncomplicated OSAS patient, one without significant comorbid factors, can be treated in a safe and prudent fashion outside of an intensive care unit.
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