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Nose and Paranasal Sinuses Tumors (Malignant tumors (Olfactory…
Nose and Paranasal Sinuses Tumors
very rare
Inverting papilloma is the most common benign tumor
Squamous cell carcinoma is the most common malignant tumor.
males > females
Age: Sixth to seventh decades
maxillary sinus is the most common location (70%), the ethmoid sinuses the second most common (20%). The sphenoid sinus (3%), and the frontal sinus (1%) are the least common locations for primary tumors.
Nasal cavity
Benign - inverting papilloma, osteoma / Malignant – SCCA
Sinuses (malignant)
SCCA, Maxillary sinus most common
Epidemiology
Occupational exposure in >40%
nickel workers - SCCA
hardwood dust & leather tanning - adenoca
Viral - HPV
Cigarettes & alcohol
Signs and symptoms
Oral occur in 25-35% and include pain involving the maxillary dentition and frank erosion into the oral cavity.
Nasal findings in up to 50% of patients and include obstruction, discharge, stuffiness, congestion, epistaxis, and extension into the nasal cavity.
Ocular findings occur in 25%, unilateral tearing, diplopia, fullness of lids, pain, and exophthalmos.
Facial signs include infraorbital nerve hypesthesia, cheek swelling, pain, and facial asymmetry.
Auditory include hearing loss secondary to serous otitis media due to nasopharyngeal extension.
Benign tumors
Osteomas
slow-growing
15 to 40 years
Localisation: frontal (95%)> ethmoid > maxillary
90% without any sings, found accidentally
Treatment: local excision
Fibrous Dysplasia
Normal bone replaced by collagen, fibroblasts, and osteoid
< 20 years
Treatment: surgery; no irradiation
Neurogenic tumors
Schwannomas
surface of nerve fibers
no malignant degeneration
along trigeminal nerve
Neurofibromas
within nerve fibers
von Recklinghausen’s disease
malignant degeneration in 15%
Complete excision
Inverting papilloma (Schneiderian)
Inverting (47%) - lateral nasal wall
0.3-4% of all nasal tumors
Etiology -
HPV
Recurs, locally destructive, malignant potential
M>W, 6th-7th decades,
unilateral
SCCA in inverting papilloma - 2-13%
Recurrence - 0-80%
It is a benign tumor that needs to be treated as a malignant one becouse of its high rate of recurence and potential of malignat transformation
Papillomas:
Fungiform (50%) - septum
Cylindrical (3%) - lateral nasal wall
Inverting (47%) - lateral nasal wall
Advanced disease
Classic Triad
facial asymmetry
tumor bulge in oral cavity
nasal mass
All three - 40-60% cases
One - 90% cases
Diagnosis
Physical exam, Nasal rhinoscopy, Endoscopy, Biopsy, Radiography (essential, CT, MRI)
CT vs. MRI
CT
Bone erosion
85% accuracy
Differentiation: tumor vs. inflammation process
MRI
Superior to CT
multiplanar, no ionizing radiation
94% accuracy
98% accuracy with gadolinium
Malignant tumors
Risk factors
Wood dust, Smoke , Chemicals , Viruses EBV i HPV, Diet ( salted, smoked products, alkohol), Radiation,
Chronic sinusitis
Staging
T1: without bony erosion
T2: erosion or destruction of the infrastructure
T3: Tumor invades: skin of cheek, posterior wall of sinus, inferior or medial wall of orbit, anterior ethmoid sinus
T4: tumor invades orbital contents or base of skull
Squamous cell carcinoma (SCCA)
Most common tumor (80%)
Location:
Maxillary sinus (70%)
Nasal cavity (20%)
90% have local invasion by presentation
Lymphatic drainage:
First echelon:
retropharyngeal nodes
Second echelon:
subdigastric nodes
Adenocarcinoma
2nd most common malignant tumor in the maxillary and ethmoid sinuses
Present in the superior portions
associated with occupational exposures
High grade: solid growth with poorly defined margins.
30% present with metastasis
Low grade: uniform and glandular with less incidence invasion/metastasis/
Adenoid Cystic Carcinoma /cylindroma/
Palate > major salivary glands > sinuses
Multiple recurrences, distant metastases
Perineural spread
Mucoepidermoid Carcinoma
Extremely rare
local invasion makes resection difficult, radiation is often indicated
Olfactory Neuroblastoma Esthesioneuroblastoma
Origin: Neural crest
at 20 and 50 years
locally aggressive
rosettes are hallmark
Kadish staging
local recurrence 50-75%
metastasis 20-30%
UCLA Staging system
T1: involving nasal cavity and/or paranasal sinus, excluding the sphenoid and superior most ethmoids
T2: Tumor involving the nasal cavity and/or paranasal sinus including sphenoid/cribriform plate
T3: Tumor extending into the orbit or anterior cranial fossa
T4: Tumor involving the brain
Rhabdomyosarcoma
Most common malignancy in children
Localisation:
parameningeal
Triple therapy is often necessary
Aggressive chemotherapy
Adults: Surgical resection with postoperative XRT for positive margins.
Hemangiopericytoma
Uncommon
pericytes of Zimmerman
80% of sinonasal tumors in ethmoids
resembles nasal polyps
average in 55 years
excision, XRT for (+) margins
Melanoma malignum
1% originate in sinonasal cavity
distribution: 5th-8th decades
anterior septum
maxillary antrum
polypoid mass
Rare malignant tumors
Osteogenic Sarcoma
most common primary bone tumor
only 5% in H & N, mandible most involved
Fibrosarcoma
rarely seen in sinuses
Chondrosarcoma
distribution: 3rd-5th decades
histologic diagnosis: difficult
slow erosion of skull base
Rhabdomyosarcoma
(embryonal, alveolar, pleomorphic)
most common in children
35-45% in H&N, 8% in sinuses
Lymphoma
bimodal presentation
treatment: irradiation +/- chemo
Extramedullary plasmacytoma
40% in paranasal sinuses/nose
treatment: excision or irradiation
Surgical resection of malignant tumors
endoscopic excision
medial maxillectomy
total maxillectomy
radical maxillectomy +/- exenteration orbite
craniofacial resection
Contraindications to surgical
extension to frontal lobes
invasion of prevertebral fascia
optic nerve involvement
cavernous sinus extension
Most lesions present in advanced states and require multimodality therapy
Wegener`s granuloma is a systemic disease with both the upper and lower respiratory tract and kidney involved
Matar