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Esthesioneuroblastoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Nasal obstruction with left-sided cervical lymphadenopathy.

Patient Data

Age: 16 years
Gender: Female
mri

There is a large, heterogeneously enhancing mass within the left nasal cavity, predominantly adjacent to the turbinates, but also involves the maxillary sinus, ethmoid air cells, and sphenoid sinus. There is suspected inspissated mucus within the left maxillary sinus. The tumor abuts but does not appear to invade through the cribriform plate.

There are several enlarged and enhancing cervical lymph nodes, primarily level I-3 lymph nodes on the left.

Case Discussion

This is an olfactory neuroblastoma (esthesioneuroblastoma) with metastasis to the locoregional lymph nodes (found on neck dissection). Gross pathologic examination of specimens removed from the left nasal cavity, maxillary sinus, and cervical lymph nodes revealed soft, friable, irregular, tan-red tissue. Histopathologic examination of these tissues revealed nests and large clusters of tumor cells. The nuclei contained granular chromatin with scant cytoplasm. Mitoses and apoptosis were frequent. Staining of these cells were positive for neuron specific enolase, synaptophysin, and chromogranin.

This patient initially did well post-operatively but required total parenteral nutrition for nutritional support for several months afterward. The patient began an extensive chemoradiation regimen after surgery. Five years post-operatively, the patient developed hypothyroidism secondary to neck irradiation but is otherwise doing well without signs/symptoms of recurrence.

Co-author:
Mason Soeder
Travis Bevington, MD

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