Treatment of olfactory neuroblastoma: a new approach.

Abstract

A 24-year-old male presented with a 6-month history of progressive left nasal obstruction, hypoanosmia, and discrete enophthalmos. As well, hypertension is present for years and peeling hands of palmar side 2–3 times a year during the winter months. Computed tomography and magnetic resonance imaging of the head showed an expansive mass in the left maxillary sinus of maximum oblique diameter 47 mm, extending into the nasal cavity (Fig. 1a). There was a partial dehiscence of the medial wall of the left maxillary sinus. Magnetic resonance angiography indicated a slightly higher caliber of maxillary artery on the left side and cervical artery angiography identified visible signs of pathological vascularization of the tumor mass, which comes from branches of the left maxillary artery and ophthalmic artery (Fig. 2). According to MRI examination, structure of tumor mass was nonhomogeneous, with numerous flow-voids that indicate the good vascularization. Intrusion of the mass through expanded sinus antrum was present in middle meatus. After application of contrast, intensive and easily nonhomogeneous staining was displayed. Endoscopic examination revealed a polypoid mass in the left nasal cavity and left maxillary sinus. The biopsy of lesion was indicated and performed under the general anesthesia and then the last tamponade was made because of massive hemorrhage which occurred during the procedure. Immunohistochemical analysis showed deposits of lobular tumors in the mucosa (Fig. 3). Tumor cells were relatively uniform, small scant cytoplasm, with some places formed pseudorosettes. Nucleus of tumor cells were round, there was no visible mitosis or nucleoluses. Blood vessels were dilated. There were no areas of necrosis. Cells were well-differentiated. Numerous rounded calcifications were found on the margins. Tumor cells were chromogranin and synaptophysin positive and analysis was detected olfactory neuroblastomagrade I (Hyams’ grading system, 1988). Described localization of tumor is atypical, since the most of the mass is in the maxillary sinus. This localization and expansion corresponds to Kadish Stage B. Embolization of maxillary and ophthalmic artery branches was performed to prevent a massive bleeding during operation. The 80 % of arteries lumen were embolized because the total lumen embolization could result with vision loss. A day after embolization, the patient was treated with endoscopic resection assisted by Caldwell-Luc with navigation under general anesthesia. The lesion in anterior and posterior ethmoidal regions was excised. Ethmoidal bone was skeletonized. There was no cerebrospinal fluid leak. Operation was performed with endoscopic transnasaltransmaxillar (Caldwell-Luc incision) approach. Tumor was excised subperiosteal and piecemeal. We did not perform a N. Vucinic (&) M. Eric Department of Anatomy, Faculty of Medicine, University of Novi Sad, Hajduk Veljkova 3, 21000 Novi Sad, Serbia e-mail: nikolavucinic87@gmail.com