Malignant lesions of nose

Carcinoma nasal cavity:

Primary carcinoma involving the nasal cavity is rather rare. If present it could be an extension of tumors from maxillary / ethmoidal sinuses. Nearly 80% of these tumors happens to be squamous carcinoma while the rest happens to be adenocarcinomas and adenocystic carcinomas.

Squamous cell carcinoma:

Can arise from the vestibule of the nose, anterior portion of nasal septum or the lateral nasal wall. Majority of these lesions are seen in men of more than 50 years of age.

Vestibular squamous cell carcinoma:

This tumor arises from the skin lined vestibule of the nose. These tumors account for less than 1% of all tumors of head and neck. Risk factors of these tumors include sun exposure and smoking.

T classification of squamous cell carcinoma of nasal vestibule:

T1 - The lesion is limited to nasal vestibule. Lesions are relatively superficial involving one or more sites within.

T2 - The lesion extends from the vestibule to adjacent structures such as the upper portion of nasal septum, upper lip, philtrum, skin of the nose, and nasolabial fold. The lesion is not fixed to underling bone.

T3 - The lesion is massive with extension to hard palate, buccogingival sulcus, large portion of upper lip, upper nasal septum, tubinates and adjacent paranasal sinuses. There is also associated fixation to deep muscles and bone.

UICC classification:

T1 - Limited to one subsite

T2 - Involves two subsites or adjacent nasoethmoidal site

T3 - Invasion of medial wall / floor of the orbit, maxillary sinus, palate, cribriform plate

T4a - Involvement of anterior orbit, skin of nose / cheek. anterior cranial fossa, pterygoid plates, sphenoid and frontal sinuses

T4b - Involvement of orbital apex, dura, brain, middle crainal fossa, cranial nerves other than second division of 5th cranial nerve, nasopharynx and clivus.

Management:

Early small lesions T1 and T2 can be managed by total resection or irradiation. Extensive lesions can be managed by radical surgery in combination with irradiation.

Adenocarcinoma / Adenocystic carcinoma nose:

These tumors arise from the glands of mucous membrane or minor salivary glands. These lesions are commonly seen in the upper part of the lateral wall of the nasal cavity.

Etiology:

Exposure to industrial fumes, wood dust, nickel refining and leather tanning have been implicated. Exposure to chromium / chromium oil have also been implicated.

These lesions can be managed by a combination of radical surgery and irradiation.

Malignant melanoma nose:

This is a very rare tumor and is found to be more aggressive than its cutaneous counterpart. It is usually seen in persons of more than 50 years of age. It is found to be equally occurring in both sexes. It is commonly seen in the anterior portion of nasal septum, followed by middle and inferior turbinates. Usually it occurs as a grey or bluish black polypoidal mass. Amelenotic variants of this tumor lacks the blue / black color since they are not pigmented. These tumors are more commonly seen in whites than in black races.

This tumor usually spreads via lymphatics and blood stream. Hence nodal metastais in the neck could be present during the diagnosis.

Wide surgical excision is the treatment of choice. It should be stressed that normal immunological mechanism play a vital role in control of this disease and hence radio and chemotherapy should be avoided at all costs as they could depress the immune mechanism.

Symptoms:

Nasal block

Nasal discharge (blood tinged)

Presence of blue / black nasal polypoidal mass

On histological examination nasal melanoma demonstrate more atypia of cells when compared to that of cutaneous variety.

Esthesioneuroblastoma:

Introduction:

This tumor arises from the olfactory nerve. These are rare malignant tumors arising from the olfactory epithelium situated at the roof of the nasal cavity, cribriform plate, upper portion of supeior turbinate and corresponding upper portion of nasal septum. These tumors typically involve the upper portion of nasal cavity and the cribriform plate area. These tumors classically arise from the basal cells of olfactory neuroepithelium. No clearl cut genetic / other causes have been attributed for this tumor. This tumor represents about 3% of all malignant tumors involving the nasal cavity.

This tumor was first reported by Berger etal in 1924. Since then only less than 1000 cases have been reported in literature.

Incidence:

Affects both male and female patients with equal frequency.

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

Etiopathogenesis (theories):

Belongs to peripheral neuroectodermal family. (Not supported by immunohistochemistry) Presence of Trisomy 8 has been documented in these tumor cells None of these theories convincingly explain etiopathogenesis of this tumor.

Clinical features:

This tumor classically involves the nasal cavity with extension into the ethmoid sinus, anterior skull base and orbit. Since these tumors are very aggressive ones they tend to metastasize extensively. Spread to cervical nodes are common. Retropharyngeal nodes are the first echelon node. Rarely these tumors may be active in an endocrine sense. These tumors have 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 system:

Staging a tumor helps in deciding the optimal management modality and also in determining the prognosis of the disease.

Kadish staging system:

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 beyond paranasal sinuses

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

Modified TNM staging system:

Tumor

T1 – tumor involving nasal cavity and / or paranasal sinuses (excluding sphenoid) sparing most of the superior ethmoidal cells

T2- tumor involving nasal cavity and / or paranasal sinuses including sphenoid with extension to the cribriform plate

T3 – tumor extending to orbit with involvement of extradural anterior cranial fossa

T4 – tumor involving brain

Node

N0 – No nodal metastasis

N1 – any form of nodal metastasis

Metastasis

M0 – No metastasis

M1 – Metastasis present

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

Hyam's Histopathological grading system:

Fw – Flexner-wintersteiner

HW – Homer – Wright

Management:

This tumor is managed with wide surgical resection with adequate tumor margins. Radiotherapy is indicated as primary treatment modality in unresectable tumors and as a follow up to surgical debulking.

Hemangiopericytoma:

These are very uncommon vascular tumors arising from extracapillary cells known as pericytes. It has a high rate of recurrence even after successful excision. Reginal metastasis also develop rather early during the disease.

Lymphoma:

Very rarely non-Hodgkin lymphoma could present as a nasal septal mass.

Plasmacytoma:

Solitary plasmacytoma without generalized osseous disease can be seen involving the nasal cavity. It commonly occurs in males after the 4th decade of life. Ideal treatment modality is radiotherapy followed by surgery three months later.

Sarcomas:

Osteogenic sarcoma, chondrosarcoma, rhabdomyosarcoma, and angiosarcoma have been described involving the nasal cavity.