Esthesioneuroblastoma presenting as diplopia and proptosis in a young female patient. Case report and review of the literature

Dan Andrei Mitrea¹*, Ana-Maria Vladila¹, Sanda Maria Nica^1,2, Ioan Buraga^1,2, Gabriela Mihailescu^1,2 and Emilia Diaconu³

¹Department of Neurology - Colentina Clinical Hospital - Bucharest, Romania

²Carol Davila University of Medicine - Bucharest, Romania

³Department of Diagnostic Imaging - Regina Maria Clinic - Bucharest, Romania

*Corresponding Author E-mail: dann.mitrea@gmail.com; Telephone: +40 723 226 233; Fax: +40 21 222 13 68

Accepted 09 December, 2014

Abstract

Esthesioneuroblastoma is a rare, primary sinonasal malignant tumor with an incidence between 3%-6% of all the sinonasal malignancies and estimated occurrence rate of 4 in 10 million individuals. We report the case of a 36 years old female patient admitted for progressive lower gaze diplopia and proptosis, diagnosed using Computer Tomography (CT), Magnetic Resonance Imaging (MRI) and histopathology examination with highly differentiated grade C esthesioneuroblastoma. Treatment included total surgical removal via endonasal approach and adjuvant radiotherapy with good prognostic. Diplopia and proptosis are rare presentations of esthesioneuroblastomas; a biopsy should always be performed in case of a nasosinusal infiltrating tumor. The current staging system, Kadish and Hyam’s scales proved to be useful instruments in choosing the appropriate treatment and also in predicting the survival rate of these patients. Long-term follow-up is mandatory due to the extended interval for disease recurrence, sometimes up to 10 years since the diagnosis.

Keywords: esthesioneuroblastoma, sinonasal malignant tumor, diplopia, proptosis,  chronic sinusitis, endoscopic nasal surgery, Kadish scale, Hyam’s scale