Esthesioneuroblastoma

Case contributed by A.Prof Frank Gaillard

Presentation

Nasal stuffiness in a young man of southeast asian ancestry.

Patient Data

Age: 25 years
Gender: Male
MRI

Abnormal enhancing T2 hypointense material that spans right middle ethmoid air cells and the upper extreme of the right nasal cavity, with obstruction of the right frontal and sphenoid sinuses are at least partial obstruction of the right maxillary sinus. There is a defect in the anterior cranial fossa floor in the region of the right cribriform plate, through which there is a subtle bulge of tumor resulting in indentation of the under surface of right gyrus rectus. There is smooth overlying dural enhancement. No convincing evidence that tumor has extended beyond dura. No evidence of orbital extension, although medial, orbital wall is broadly abutted by tumor in ethmoid. No evidence of perineural spread. A prominent vessel, most likely a developmental venous anomaly, is incidentally noted in the left paramedian frontal lobe.

Conclusion: Right sinonasal mass eroding cribriform plate with superior bowing of overlying dura. This is most likely an esthesioneuroblastoma. Nasopharyngeal carcinoma is less likely in this age group, but could be considered given ethnic background. Lymphoma would typically have very low ADC values, and juvenile nasopharyngeal angiofibroma typically enhances more vividly, is centered differently and has a slightly younger demographic peak.

CT

Opacification of the right upper nasal cavity and ethmoidal air cell without overt bony destruction.  No infiltration into the orbit.

Case Discussion

The patient went on to have a resection.

Histology

Sinonasal mucosa, bone, olfactory bulb and numerous nerves. The tissue is extensively infiltrated by cellular tumor forming lobules separated by thin blood vessels. Tumor cells have small round to oval nuclei with finely granular chromatin, small nucleoli and a moderate amount of wispy cytoplasm. No rosettes are identified. There is focal calcification. Mitoses are present but not prominent, with 3 per 10 hpf. Nuclear pleomorphism is moderate overall. There is no fibrillary matrix seen. There are areas of crush artefact but no convincing tumor necrosis. There are changes focal consistent with a previous biopsy.

By immunohistochemistry, the tumor cells are positive for calretinin, chromogranin, synaptophysin, and CD56 and negative for CAM5.2, CD99, AE1/3, HMB-45, Melan-A, desmin, CD99, CD45, CD3, CD20 and GFAP. S100 shows some sustentacular cells. 

FINAL DIAGNOSIS: esthesioneuroblastoma (olfactory neuroblastoma)

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Case information

rID: 61664
Published: 10th Oct 2018
Last edited: 10th Oct 2018
System: Head & Neck
Inclusion in quiz mode: Included

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