Introduction: Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of the sinonasal cavity. Surgery has been and remains a mainstay of treatment for patients with this tumor. Open craniofacial resections have been the treatment of choice for many decades. Recently, experience has been growing with endoscopic approaches in the management ONB. The object of this study is to report our experience over the past 11 years with ONB patients treated using purely endonasal endoscopic techniques.
Methods: We performed a retrospective chart review of 20 consecutive patients with ONB who underwent a completely endonasal endoscopic approach for an oncologic tumor resection at our institution between January 2006 and January 2017. Patient demographics, tumor stage, pathological grade, frozen section analysis, permanent margin assessment, perioperative complications, postoperative therapy, length of follow-up, and outcomes at last follow-up were collected and analyzed.
Results: Eighteen patients presented with newly diagnosed disease with modified Kadish stage of A in 2, B in 3, C in 11, and D in 2. Two patients presented with recurrent tumors. An average of 25.3 specimens per patient were examined by frozen section analysis. Although analyses of intraoperative frozen section margins were negative in all but 1 case, microscopic foci of tumor were found in 7 cases (35%) on permanent histopathological analysis. Perioperative complications occurred in 7 patients (35%) including 1 patient who developed a cerebrospinal fluid leak; there were no episodes of meningitis. All but 1 patient received postoperative radiotherapy, and 5 patients received postoperative chemotherapy. With mean follow-up of over 5 years, 19 patients were alive and 1 patient died from an unrelated cause. There were 2 cases of tumor recurrence. The 5-year overall, disease-specific, and recurrence-free survival rates were 92.9%, 100%, and 92.9%, respectively.
Conclusions: The results provide additional evidence for continued use of endoscopic procedures in management of patients with ONB.
Patient Care: By providing information on current approaches in the management of a rare malignancy i.e. olfactory neuroblastoma.
Learning Objectives: To describe the outcomes of patients undergoing purely endonasal endoscopic resection of olfactory neuroblastomas (ONB)
References: Broich G, Pagliari A, Ottaviani F: Esthesioneuroblastoma: a general review of the cases published since the discovery of the tumour in 1924. Anticancer Res 17:2683-2706, 1997
Dulguerov P, Allal AS, Calcaterra TC: Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2:683-690, 2001
Gallia GL, Reh DD, Lane AP, Higgins TS, Koch W, Ishii M: Endoscopic resection of esthesioneuroblastoma. J Clin Neurosci 19:1478-1482, 2012 410 13.
Gallia GL, Reh DD, Salmasi V, Blitz AM, Koch W, Ishii M: Endonasal endoscopic resection of esthesioneuroblastoma: the Johns Hopkins Hospital experience and review of the literature. Neurosurg Rev 34:465-475, 2011
Roxbury CR, Ishii M, Richmon JD, Blitz AM, Reh DD, Gallia GL: Endonasal Endoscopic Surgery in the Management of Sinonasal and Anterior Skull Base Malignancies. Head Neck Pathol 10:13- 22, 2016
Shah K, Perez-Ordonez B: Neuroendocrine Neoplasms of the Sinonasal Tract: Neuroendocrine Carcinomas and Olfactory Neuroblastoma. Head Neck Pathol 10:85-94, 2016