Esthesioneuroblastoma Clinical Presentation

Updated: Jan 02, 2018
  • Author: Michael Somenek, MD; Chief Editor: Herbert H Engelhard, III, MD, PhD, FACS, FAANS  more...
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Presentation

History

The symptoms of esthesioneuroblastoma (ENB) can be classified into nasal, neurologic, oral, facial, cervical, and ophthalmologic and are as follows:

  • Nasal - Obstruction (70%), epistaxis (46%), discharge, unilateral polyp, anosmia

  • Neurologic - Headache, nausea

  • Oral (rare) - Mobile tooth, nonhealing tooth-extraction site, ill-fitting dental prostheses, ulceration

  • Facial (rare) - Swelling, pain, anesthesia, trismus

  • Cervical - Mass

  • Ophthalmologic - proptosis, extraocular movement paralysis, and blindness,

The average delay between the appearance of the initial symptom and the diagnosis is 6 months, but diagnosis is delayed for years in some cases. Delay is understandable because initial symptoms tend to be subtle and are frequently banal, occurring also in common nasal diseases, including long-term rhinosinusitis or allergic polypoid sinus disease. [8]

Many patients undergo sinus surgery, only to have the diagnosis established as an unexpected pathologic finding. Therefore, sending all the tissue removed during sinus surgery for pathologic examination is important for diagnosis of esthesioneuroblastoma, as is the vigilance of the pathologist in examining the tissue.

For the most part, malignancy is not considered until secondary symptoms such as facial pain and deformity or cranial nerve impairment are observed. However, early referral for an intranasal biopsy is essential to early diagnosis. [9] A patient with a unilateral nasal obstruction and/or recurrent epistaxis lasting longer than 1-2 months should undergo a thorough nasal evaluation by an otolaryngologist, although the cost-effectiveness of this approach has not been evaluated.

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Physical

Nasal examination, particularly if aided by endoscopy, reveals a reddish-gray tumor arising in the upper nasal fossa, which bleeds easily with instrumentation. Although this aspect is strictly different from the white, glistening appearance of benign nasal polyps, little differentiates esthesioneuroblastoma (ENB) from other nasal malignancies. Late findings may include signs related to extensive disease such as orbital, cranial, and cervical involvement.

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Causes

No clear etiologic agent or exposure has been documented in humans; however, a single case of occupational exposure has been reported in a woodworker. [10] Esthesioneuroblastoma (ENB) can be consistently induced by nitrosamine compounds in rodents. In cats with spontaneous ENB or in transgenic mice developing ENB, type C retroviral particles have been demonstrated and classified as feline and murine leukemia virus, respectively. The role of retrovirus sequences in human ENB remains to be evaluated.

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