Esthesioneuroblastoma



Esthesioneuroblastoma


Michelle A. Michel, MD





Key Facts


Terminology



  • Malignant neuroectodermal tumor arising from olfactory mucosa in superior nasal cavity


Imaging



  • Enhanced MR with bone-only CT best maps ENB for en bloc craniofacial surgery


  • Dumbbell-shaped mass with “waist” at level of cribriform plate


  • Bone CT: Bone remodeling mixed with bone destruction, especially of cribriform plate


  • CECT/T1 C+ MR: Homogeneously enhancing mass



    • Cysts at intracranial tumor-brain margin


Top Differential Diagnoses



  • Sinonasal squamous cell carcinoma


  • Sinonasal adenocarcinoma


  • Sinonasal non-Hodgkin lymphoma


  • Sinonasal undifferentiated carcinoma


Pathology



  • No etiologic basis or risk factors elucidated


  • Kadish staging system



    • Good predictor of outcome


  • Staging criteria: Kadish classification; good predictor of outcome


  • Histologic grading: Hyams system


Clinical Issues



  • Adolescent or middle-aged patient with unilateral nasal obstruction & mild epistaxis



    • Bimodal distribution in 2nd & 6th decades


  • Combined surgical resection & radiotherapy is treatment of choice


  • Excellent prognosis vs. other sinonasal malignancies



    • 5-year survival rates: 75-77% overall


    • Recurrences in ˜ 30%


    • Metastases in 10-30% of patients






(Left) Coronal graphic shows the classic features of esthesioneuroblastoma (ENB) centered below the cribriform plate and extending into the anterior cranial fossa and right orbit image. Cyst formation image is noted at the tumor-brain interface. (Right) Coronal bone CT shows an ENB filling the upper nasal cavity and ethmoid sinuses. The lesion extends through the anterior skull base image. The lamina papyracea on the right is thinned and laterally displaced image.






(Left) Axial STIR MR demonstrates a large, heterogeneous ENB image centered in the midline below the skull base and occupying the nasal cavity and ethmoid sinuses. The mass is predominantly hypointense in this case and causes hypertelorism. (Right) Coronal T1WI C+ FS MR shows an avidly enhancing ENB with extension into anterior cranial fossa image and both orbits image. Avid enhancement is characteristic of this highly vascular neoplasm. Note the trapped maxillary secretions image.



TERMINOLOGY


Abbreviations



  • Esthesioneuroblastoma (ENB)


Synonyms



  • Olfactory neuroblastoma, pleomorphic olfactory neuroblastoma


Definitions



  • Rare malignant neuroectodermal tumor that arises in nasal cavity


IMAGING


General Features



  • Best diagnostic clue



    • Dumbbell-shaped mass with upper portion in anterior cranial fossa, lower portion in upper nasal cavity, & “waist” at level of cribriform plate


    • Peripheral tumor cysts at intracranial tumor-brain margin is highly suggestive of diagnosis of ENB


  • Location



    • Superior nasal cavity at cribriform plate



      • Smaller ENB: Unilateral nasal mass centered on superior nasal wall; local spread in nose & sinuses


      • Large ENB: Tumor in anterior cranial fossa with parenchymal & dural infiltration, extension into orbits


  • Size



    • Range from < 1 cm nodule to mass filling entire nasal cavity & lower anterior cranial fossa


  • Morphology



    • Polypoid mass when small; dumbbell-shaped when large


CT Findings



  • NECT



    • Bone CT



      • Bone remodeling causing enlargement of nasal cavity mixed with bone destruction, especially of cribriform plate area


      • Speckled pattern of calcification within tumor matrix unusual


  • CECT



    • Homogeneously enhancing mass


    • When large, may see nonenhancing areas of necrosis

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Sep 20, 2016 | Posted by in HEAD & NECK IMAGING | Comments Off on Esthesioneuroblastoma

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