Esthesioneuroblastoma - A Unique Nasal Tumor

Introduction:

This tumor arises from the olfactory nerve. These are extraordinary malignant tumors arising from the smelling epithelium situated at the roof of the nasal cavity, cribriform plate, upper portion of supeior top-shaped and corresponding upper portion of nasal septum. These tumors typically cover the upper portion of nasal void and the cribriform plate area. These tumors classically arise from the basal cells of smelling neuroepithelium. No clear cut genetic / other causes own been attributed for this tumor. This tumefaction represents about 3% of all virulent tumors involving the nasal cavity.

This swelling was first reported by Berger etal in 1924. Since soon afterward only less than 1000 cases desire been reported in literature.

Incidence:

Affects both male and female patients with equal frequent occurrence.

Age incidence shows a bimodal top (peaking at the second and the sixth decades of life).

Etiopathogenesis (theories):

Belongs to peripheral neuroectodermal race. (Not supported by immunohistochemistry)

Presence of Trisomy 8 has been documented in these tumefaction cells

None of these theories convincingly solve etiopathogenesis of this tumor.

Clinical features:

This swelling classically involves the nasal cavity with extension into the ethmoid sinus, preceding skull base and orbit. Since these tumors are real aggressive ones they tend to metastasize extensively. Spread to cervical nodes are inferior. Retropharyngeal nodes are the first echelon process. Rarely these tumors may be diligent in an endocrine sense. These tumors receive known to cause ectopic secretion of ACTH causing Cushing's syndrome.

Common presenting symptoms were usually associated with the nasal cavity:

Nasal block

Epistaxis

Anosmia

Staging hypothesis:

Staging a tumor helps in deciding the optimal direction modality and also in determining the prophasis of the disease.

Kadish staging body:

This system stages Esthesioneuroblastoma into three stages:

Stage A: Tumor limited to nasal cavity

Stage B: Tumor extending to paranasal sinuses

Stage C: Tumor extending over paranasal sinuses

Major inadequacy of this scaffold system is too broad a platform at the level of Stage C.

Modified TNM scaffold system:

Tumor

T1 - tumor involving nasal cavity and / or paranasal sinuses (excluding wedge-shaped) sparing most of the superior ethmoidal cells
T2- tumor involving nasal cavity and / or paranasal sinuses including sphenoid with extension to the cribriform lamina
T3 - tumor extending to orbit with involvement of extradural anterior cranial fossa
T4 - tumor involving brain

Node

N0 - No nodal metastasis
N1 - some form of nodal metastasis

Metastasis

M0 - No metastasis
M1 - Metastasis propitious

Hyam came out with a histopathological grading hypothesis which turned out to be additional accurate than the preceding two staging systems.

Hyam's Histopathological grading regularity

Grade
LA preservation
Mitotic index
Nuclear polymorphism
Fibrillary matrix
Rosettes
Necrosis

I
+
Zero
None
Prominent
HW
None

II
+
Low
Low
Present
HW
None

III
+/-
Moderate
Moderate
Low
FW
Rare

IV
+/-
High
High
Absent
None

Frequent

Fw - Flexner-wintersteiner
HW - Homer - Wright

Management:

This tumor is managed with wide surgical resection with adequate tumor margins. Radiotherapy is indited in the same manner with primary treatment modality in unresectable tumors and viewed like a follow up to surgical debulking.