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.

Benign tumors

  • 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.

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

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

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%

Rhabdomyosarcoma

  • 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
  • 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