Esthesioneuroblastoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Sinusitis and nasal congestion. Evaluate for sinus disease.

Patient Data

Age: 35 years
Gender: Male
ct

There is complete opacification of the left maxillary, sphenoid, ethmoid, and frontal sinuses. There is obstruction of the osteomeatal unit on the left. Erosive changes of the osseous portion of the turbinates and resorption of some of the ethmoid septa are noted on the left. The left nasal cavity is slightly expanded compared to the contralateral side and there is a large polypoid filling defect extending through the nasal choana into the nasopharynx.

There are two adjacent subcentimeter retention cysts or polyps along the inferolateral wall of the right maxillary sinus. There is mild mucosal thickening in the region of the infundibulum causing partial obstruction of the right ostiomeatal unit. There is mild to moderate right ethmoid sinus mucosal thickening.

Case Discussion

This is a case of pathologically proven olfactory neuroblastoma (esthesioneuroblastoma). Gross pathologic examination of specimens from the ethmoid sinus and left maxillary mass revealed aggregates of tan-pink fibromembranous tissues. Histopathologic examination of the specimens revealed esthesioneuroblastoma tumor tissue intermixed with normal sinonasal tissue. Synaptophysin immunostain revealed rare nests of tumor throughout the specimens. The tumor cells were also positive for chromogranin, CD56, S-100, and SOX-10. These cells were negative for MNG116, Cam5.2, AE1:AE3, LCA, CD138, myogenin, desmin, CD99, MITF, HMB45, and MART-1.

Initial surgical interventions included a left sphenopalatine artery ligation, left total ethmoidectomy, left sphenoidotomy, and removal of the large nasal mass. The patient underwent a second round of surgeries, including an extended endonasal (sinonasal and cranial) resection of the esthesioneuroblastoma. The patient then underwent a repair with a right vascularized pedicle nasal septal flap. The patient initially did well post-operatively and received post-operative adjuvant therapy. The patient continues to follow with otolaryngology with no evidence of recurrence. He endorses ongoing olfactory dysfunction, but is overall doing well from a sinonasal standpoint.

Co-author:
Mason Soeder

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