CASE 8985 Published on 28.01.2011

Esthesioneuroblastoma of the right nasal cavity with intracranial extension

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Koutsokosta E, Pilavaki M, Chondros N, Tsanaktsidis I, Palladas P.
CT and MRI Department, General Hospital ‘G.Papanikolaou’, Thessaloniki, Greece.

Patient

16 years, male

Categories
Area of Interest Head and neck, Oncology ; Imaging Technique MR
Clinical History
A 16 year old male without previous health problems was referred to our hospital with difficulty in breathing by a mass with bloody discharge in the right nasal cavity.
Imaging Findings
In order to evaluate the cause of right nasal obstruction, a pre and post-contrast brain and splachno-cranium MRI examination were performed.
MRI examination revealed a lesion in the right nasal cavity, which extended to the cribriform plate and then into the right frontal lobe of the brain. The mass appeared hypointense on T1-weighted MR image and slightly hyperintense on T2–weighted MR image. DWI demonstrated low signal intensity with increased ADC values. No restriction of diffusion was observed. After intravenous administration of gadopentetate dimeglumine the mass enhanced heterogeneously. The intracranial part of the mass was surrounded by edema and displacement of the intermediate fissure.
Findings were compatible with esthesio-neuroblastoma of the right nasal cavity with intracranial extension. The diagnosis was confirmed by biopsy.Surgical resection was performed.Because of the big size of the tumor, a small part of the mass remained intracranially. After 18 months of the surgical treatment the patient is in good condition.
Discussion
Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, first described by Berger et al. in 1924 as a rare, malignant slowly growing neurogenic neoplasm arising from the olfactory mucous membrane. It usually occurs in all age groups with bimodal peaks in the 2nd and 5th decades. There is no genetic predilection. ENB arises high in the nasal fossa and it can extend to the paranasal sinuses, the nasopharynx, palate, orbit, base of the skull and brain.
It should be suspected in patients with persistent unilateral nasal obstruction and severe epistaxis and rhinorrhoea. Anosmia can be caused by penetration into the cribriform plate. When the tumour obstructs the Eustachian tube it can cause ear pain and lead to otitis media. Frontal headache suggests involvement of the frontal sinus. Because of the non-specificity of the symptoms patients tend to present with advanced stage disease. The mass is growing very slowly and causes some expansion and remodelling of bony structures. “Hyperostosis” may be an inflammatory reaction to the obstruction of neighbouring air cells. This is a very aggressive neoplasm, which causes lymphatic and haematogenous metastases. The most common metastases are in the cervical and retropharyngeal lymph nodes, parotid glands, skin, lungs, bone, liver, orbit, spinal cord and spinal canal.
CT and MRI are the best examinations used for diagnosis, staging and follow up. On CT, the tumour presents as iso- to slightly hyperdense compared to muscle mass in the nasal cavity with homogenous enhancement. Bone erosion is a common finding which is often accompanied by bone remodelling. Calcifications within the mass are a characteristic diagnostic feature. On T1W MR Images esthesioneuroblastoma presents as a hypointense mass with mild homogenous enhancement and iso- to hyperintense on T2W MR Images.
Despite the bibliographic data , in our case the mass showed heterogeneous enhancement after intravenous administration of gadopentetate dimeglumine
In our case the most likely diagnosis was esthesioneuroblastoma because:
extracranial nasal meningioma usually causes hyperostosis to the adjacent skull base; sinonasal undifferentiated carcinoma is most common in older patients; squamous cell carcinoma is more common in the maxillary antrum than in the nasal cavity and it doesn’t enhance as much as ENB; a non-Hodgkin-lymphoma rarely breaches the skull base and it does not enhance to the same degree as ENB.
ENB are most frequently staged using a system proposed by Kadish et al in 1976 as following:
A: tumour is limited to the nasal cavity; B: tumour is limited to the nasal cavity and one or more paranasal sinuses; C: tumour extends beyond the nasal cavity and paranasal sinuses including skull base, intracranial compartment, orbit and distant metastatic disease.
An additional group D has been proposed by Chao et al in 2001 - D: cervical node metastases. In our case, the mass was staged as Kadish stage C tumor.
Treatment involves surgery, chemotherapy and radiotherapy. Negative prognostic factors include female sex, age over 50 years at presentation, tumor recurrence, metastases (especially distant ones), high tumour grade and Kadish stage C at presentation.
Differential Diagnosis List
Esthesioneuroblastoma of the right nasal cavity with intracranial extension.
undifferentiated carcinoma
Hodgkins and non-Hodgkins lymphoma
plasmatocytoma
extracranial meningioma
Final Diagnosis
Esthesioneuroblastoma of the right nasal cavity with intracranial extension.
Case information
URL: https://www.eurorad.org/case/8985
DOI: 10.1594/EURORAD/CASE.8985
ISSN: 1563-4086

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