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Olfactory neuroblastoma (esthesioneuroblastoma)

Case contributed by: Dr Frank Gaillard

CT and MRI through the paranasal sinuses demonstrate a large aggressive mass the epicentre of which appears to be centred in the superior aspect of the nasal cavity.

The mass extends superiorly into the anterior cranial fossa, laterally into the orbit and posteriorly into the sphenoid sinus. The outflow of the sphenoid sinus and left frontal sinus are presumably obstructed as the sinuses are opacified. 

The mass is of soft tissue density, intermediate signal on T1 and T2 weighted images and demonstrates extensive heterogeneous enhancement following administration of contrast.

CT and MRI through the paranasal sinuses demonstrate a large aggressive mass the epicentre of which appears to be centred in the superior aspect of the nasal cavity.

The mass extends superiorly into the anterior cranial fossa, laterally into the orbit and posteriorly into the sphenoid sinus. The outflow of the sphenoid sinus and left frontal sinus are presumably obstructed as the sinuses are opacified. 

The mass is of soft tissue density, intermediate signal on T1 and T2 weighted images and demonstrates extensive heterogeneous enhancement following administration of contrast.

The patient went on to have excision of the mass. 

Histology

Sections show a densely cellular high-grade neoplasm displaying small and large areas of necrosis. The tumour is composed of sheets, lobules and broad trabeculae of tumour cells with intervening fibrovascular septa and reactive desmoplastic stroma. Focal formation of Homer Wright rosettes is also noted. Tumour cells have scant cytoplasm and round nuclei with fine chromatin pattern and show nuclear molding. There is focal nuclear pleomorphism. Mitoses and apoptotic bodies are frequent. Occasional foci  display larger neoplastic cells with abundant cytoplasm and larger nuclei. 

The tumor shows wide dissemination within the respiratory mucosa and also infiltrates the adjacent bone. In the orbital region, the tumour invades the connective tissue, skeletal muscles and fat. Foci suggestive of vascular invasion are noted. Tumour cells are strongly immunoreactive for synaptophysin and NSE and weakly positive for chromogranin and S100 protein. Neurofilament immunostaining is negative.

Case Discussion:

Location and appearances are typical for an olfactory neuroblastoma which was proven histologically.

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