Nasal cavity, paranasal sinuses, nasopharynx
Other tumors
Olfactory neuroblastoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 24 May 2018, last major update November 2004

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Olfactory neuroblastoma[TI] nasal[TIAB]

Cite this page: Pernick, N. Olfactory neuroblastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalolfactoryneuroblastoma.html. Accessed June 22nd, 2018.
Definition / general
  • Also called esthesioneuroblastoma
  • Rare, malignant neuroectodermal tumor thought to arise from olfactory membrane or olfactory placode ("placode": plate-like thickening of embryonic ectoderm from which a nerve ganglion or sensory organ will develop), which extends from roof of nasal cavity in fetus to midnasal septum and superior turbinate
  • Not related to neuroblastomas elsewhere in body
  • Median age 50 years, range 3 - 79 years; no gender preference
  • Usually upper nasal vault; rarely in nasopharynx, maxillary or ethmoid sinus
  • Locally invasive into paranasal sinuses, nasopharynx, palate, orbit, base of skull, brain
  • 20% develop distant metastases, usually to cervical lymph nodes and lung; late recurrence (after 10 years) is common
Staging (Kadish)
  1. Confined to nasal cavity
  2. Confined to nasal cavity and paranasal sinuses
  3. Other

  • 40 - 50% are stage B
  • 5 year survival by stage: A: 75%, B: 68%, C: 41%
Treatment
  • Complete surgical excision (may require craniofacial resection), with radiation therapy or chemotherapy
Gross description
  • Red-gray, highly vascular, polypoid mass of soft tissue
Microscopic (histologic) description
  • Nests or sheets of uniform small cells with scant cytoplasm, round nuclei with indistinct nuclear membrane and punctuate chromatin, no / indistinct nucleoli
  • Mild to moderate nuclear pleomorphism
  • Prominent fibrillary or reticular background in 85% of cases
  • May have abundant fibrovascular stroma that obscures tumor
  • Often crush artifact
  • Variable mitotic figures
  • Variable Homer Wright rosettes (cells surrounding central zones of fibrils), ganglion cells and tumor cell melanin
  • Necrosis is poor prognostic factor
Positive stains
Negative stains
Electron microscopy description
  • Dense core neurosecretory granules, microtubules, neuritic processes, neurofilaments
Molecular / cytogenetics description
  • No t(11;22)(q24;q12) of Ewing / PNET
Additional references