Esthesioneuroblastoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Natural History
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.[2]
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.[8] 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
ENB tumors display varying clinical behaviors ranging from indolent growth to highly aggressive invasion [6]. An appropriate treatment plan for ENB has not been outlined due to its rarity and lack of control trials; however, surgical resection has been considered as the primary form of treatment alone or in conjunction with radiation therapy and/or chemotherapy [7–9].
Unfortunately, despite aggressive therapy, ENB has been noted to have a high local recurrence rate of 50–60% with 10–62% presenting as metastatic cases and 20–30% of those cases involving the CNS [7, 10]. Once ENB cells invade the cribriform plate, they can spread to the anterior skull base, extend to the brain parenchyma or leptomeninges and can lead to drop metastasis anywhere in central neural axis [7, 11, 12]. CNS metastasis is usually noted 0–10 years after the initial diagnosis and reported as having a survival expectancy of 2 years or less [13]. Spinal metastasis of ENB is rare with only 28 documented cases involving the spine, spinal cord, or leptomeninges. We report a case of ENB with multiple drop metastasis and significant progression of the tumor despite multiple aggressive surgical resections in conjunction with chemotherapy and radiation therapy.
Clinical presentation is usually secondary to nasal stuffiness and rhinorrhoea or epistaxis. Presentation is often delayed and symptoms may have been present for many months. Patients often present late with larger tumours which can extend into the intracranial compartment (25-30% at diagnosis) and usually result in anosmia.
Complications
Prognosis
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Treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision. Prognosis is significantly affected by presence of distant metastases.
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