Nose & PNS Malignancy
Current concepts

Balasubramanian Thiagarajan

Otolaryngology online

Drtbalu's otolaryngology online
Introduction
· Uncommon tumors - >1% of all neoplasms
· Diverse group – some unique to nose alone
· Produces very little symptoms
· Commonly mistaken for rhinosinusitis
· Average delay from first symptom to diagnosis is about 6 months
· Accurate staging is still not possible – Current staging system is only for maxillary & ethmoid sinuses
Reality
· Surgery & chemoradiotherapy main trt modalities available
· Treatment modalities inflict considerable morbidity
· Facial disfigurement / Interference with mastication / loss of sight
· Quality of life – considered while choosing treatment modality
Epidemiology
· Incidence – 1% per 100,000 / year
· Commonly develop during 5th – 6th decades of life
· Twice as common in men than women
· Common sino-nasal malignancy – Primary epithelial tumors followed by non-epithelial malignant tumors
· Tumors arising from nose 25% and tumors arising from sinuses 75%
· 60% of squamous carcinomas arise from maxillary sinus, 20% from nasal cavity rest from ethmoids. 1% arise from sphenoid
Common sinonasal malignancy
· Squamous cell carcinoma – commonest
· Adenocarcinomas
· Adenocystic carcinomas
· Undifferentiated carcinomas
· Non Hodgkin's lymphoma
· Melanomas
Adenocarcinoma
· Third most common epithelial malignancy next only to sinonasal gland carcinoma.
· 4 times frequent in men. ? Occupational exposure to wood dust.
· Commonly arise from olfactory cleft.
· Usually appears polypoidal
· Unilateral expansion of olfactory cavity and opacification in imaging - ? suspicion
Adenocarcinoma - Imaging
Etiology
· Exposure to carcinogen
· Smoking / alcoholism
· Viral
Carcinogen
· Wood dust
· Nickel
· Chromium
· Polycyclic hydrocarbon
· Aflatoxin
· Thorotrast – (watch dial makers)
Wood dust
· Adenocarcinoma
· Wood workers – 500 times common
· Exposure to hard wood dust – Ebony, mahogany, oak.
· Exposure threshold - > 5mg / m3 / day
· Chemicals used in wood processing have been eliminated as a cause
· Even short periods of exposure can cause adenocarcinoma (< 5 years)
Industrial risk
· Wood industry
· Textile industry
· Bakery
· Textile
· Nuclear industry
· Farming
· Construction
· Mining
Human papilloma virus
· HPV-6 / HPV-11 demonstrated in 10% of squamous cell carcinoma nose & pns.
· HPV – 16 / HPV – 19 are known to cause more virulent cancers.
· Presence of squamo columnar junctions in the nose predisposes to HPV induced cancers.
Tumor spread
· Local invasion
· Orbital spread common – thin walls, nerves and blood vessels cause dehiscence
· Roof of frontal sinus is thin – perforations + for olfactory nerves to pass
Ohngren's Line
· Line running from medial canthus to angle of
mandible
· Prognosis of suprastructure tumors worse (This
was before advent of craniofacial resection)
Lymphatic drainage
· Lymphatic drainage of this area is scanty.
· Anterior / Posterior pathway
· Anterior pathway – 1st echelon nodes (facial, parotid, submandibular nodes)
· Posterior pathway – 1st echelon nodes (retropharyngeal nodes)
· Anteroinferior nasal cavity, skin of nasal vestibule – anterior pathway
· Rest of nose and sinuses drain via posterior pathway
Tumor spread
Clinical features
· Oral symptoms – Pain, trismus, alveolar ridge fullness, erosion, loosening teeth, ill fitting dentures (2530%).
· Nasal symptoms – Obstruction, epistaxis, rhinorrhoea (50%).
· Ocular symptoms – Epiphora, diplopia, proptosis, blindness (25%).
· Facial signs – Paresthesias, asymmetry
Clinical
Radiology
MRI
· Differentiates tumor from soft tissue
· Differentiates secretions from tumor mass
· Demonstrates perineural spread
· Not affected by dental fillings
· Can be imaged in sagittal plane
· Coronal MRI – Foramen rotundum, vidian canal, foramen ovale and optic canal can be seen
Angiogram
· Tumors surround carotid artery
· Carotid artery needs to be sacrificed in order to obtain clear surgical margins
· Balloon occlusion tests should be performed to estimate the risk of cerebral infarction if carotid needs to be
sacrificed.
CT imaging
Squamous cell carcinoma
· Most common sinonasal malignancy.
· Common during 7th decade / males.
· Arises – lateral nasal wall. 50% arises from turbinates.
· 85% are well differentiated and keratinizing.
· 15% of inverted papilloma turns malignant
Adenocarcinoma
· Wood workers
· 9% of all sino nasal malignancies
· Common 6th – 7th decades
· Common in upper nasal cavity / ethmoidal sinuses
· Growth rate slow
· Metastasis uncommon
· Histological types: Papillary, sessile, mucoid, neuroendocrine, intestinal, undifferentiated.
Adenoid cystic carcinoma
· 5% of all sinonasal malignancies
· Slow growth, perineural spread, vascular spread
· Maxillary sinus commonly affected
· Long history of facial pain defying diagnosis
Olfactory neuroblastoma
· Arises from basal cells of olfactory epithelium
· 5% of sinonasal malignancies
· Bimodal distribution (20 and 50 yrs old peak).
· More common in women than men
· Paraneoplastic syndrome +
Kadish staging system
· Stage A – Tumor limited to nasal cavity
· Stage B – Tumor limited to nose and sinuses
· Stage C – Tumor extending beyond the confines of nose and sinuses
· Stage D – distant metastasis
ULCA Staging
Stage

Description

T1

Tumor involving the nasal cavity or
paranasal sinuses (excluding sphenoid) or
both, sparing the most superior ethmoidal
air cells

T2

Tumor involving the nasal cavity or
paranasal sinuses (including the sphenoid)
or both with extension to or erosion of the
cribriform plate

T3

Tumor extending into the orbit or
protruding into the anterior cranial fossa

T4

Tumor involving the brain
Undifferentiated carcinoma
· Anaplastic
· Aggressive tumor
· Produces fewer symptoms
· Chemoradiation +
Melanoma
· 4% of sinonasal malignancies
· Common in women than men
· Affects elderly
· Nasal cavity / septum common sites
· Polypoidal / ulceration
· Metastasis less frequently to nodes
· Lungs / brain metastasis common
Lederman's classification
· Lines of Sebileau
· Supra, meso and infrastructures
· Prognosis worsens from below upwards
Growth maxilla – staging TNM
T1

Tumor confined to antrum – No bone
erosion

T2

Tumor with bone destruction except
posterior wall of antrum

T3

Erosion of posterior wall /
infratemporal fossa / pterygoid plates /
orbit / ethmoid sinus

T4a

Anterior orbital contents / cribriform
plate / sphenoid / frontal sinus

T4b

Orbital apex / dura / brain / middle
fossa / nasopharynx / clivus
Ethmoid sinus - TNM
T1

Tumor confined to ethmoid / with
or without bone erosion

T2

Tumor extending into nasal cavity

T3

Tumor extending to anterior orbit /
maxillary sinus

T4a

Anterior orbital contents / Skin of
nose or cheek / minimal anterior
cranial invasion / pterygoid plates /
sphenoid / frontal sinus
Orbital apex / dura / brain / middle
cranial fossa / cranial nerves other
than V2 / nasopharynx / Nasal
cavity

T4b
Nasal cavity
Subsites recognized – septum / floor / lateral wall / vestibule
T1

Tumor involving one subsite

T2

Tumor involving two subsites / ethmoid

T3

Tumor eroding to anterior orbit / maxillary sinus

T4a

Anterior orbit / skin of nose and cheek / minimal anterior cranial
fossa extension / pterygoid plates / sphenoid / frontal sinus

T4b

Orbital apex / dura / brain / middle cranial fossa / nasopharynx /
clivus / cranial nerves other than V2
Treatment
· Surgery
· Radiotherapy
· ? Chemotherapy
· Combination
Irradiation
· Preop irradiation preferable
· Post op irradiation is suitable only for slow growing tumors
· 200 rads x 5 days a week – 6 weeks (6000rads)
Surgery
· Partial maxillectomy
· Total maxillectomy
· Extended maxillectomy
Medial maxillectomy
· Good access to nasal cavities / ethmoids /
nasopharynx / sphenoid / pterygopalatine fossa
· Moore's incision
· Incision may be continued into nasal cavity
Medial maxillectomy - osteotomy
Anterior craniofacial resections
· Type I – Craniofacial / transorbital resection. This procedure is extended medial maxillectomy with
resection of ethmoid roof and orbital periosteum
· Type II – Medial maxillectomy with window craniotomy using frown line incision
· Type III – Neurosurgeon helps. Transfacial with neurosurgical approach like frontolateral craniotomy
Thank you

Malignant tumors involving paranasal sinuses

  • 1.
    Nose & PNSMalignancy Current concepts Balasubramanian Thiagarajan Otolaryngology online Drtbalu's otolaryngology online
  • 2.
    Introduction · Uncommon tumors- >1% of all neoplasms · Diverse group – some unique to nose alone · Produces very little symptoms · Commonly mistaken for rhinosinusitis · Average delay from first symptom to diagnosis is about 6 months · Accurate staging is still not possible – Current staging system is only for maxillary & ethmoid sinuses
  • 3.
    Reality · Surgery &chemoradiotherapy main trt modalities available · Treatment modalities inflict considerable morbidity · Facial disfigurement / Interference with mastication / loss of sight · Quality of life – considered while choosing treatment modality
  • 4.
    Epidemiology · Incidence –1% per 100,000 / year · Commonly develop during 5th – 6th decades of life · Twice as common in men than women · Common sino-nasal malignancy – Primary epithelial tumors followed by non-epithelial malignant tumors · Tumors arising from nose 25% and tumors arising from sinuses 75% · 60% of squamous carcinomas arise from maxillary sinus, 20% from nasal cavity rest from ethmoids. 1% arise from sphenoid
  • 5.
    Common sinonasal malignancy ·Squamous cell carcinoma – commonest · Adenocarcinomas · Adenocystic carcinomas · Undifferentiated carcinomas · Non Hodgkin's lymphoma · Melanomas
  • 6.
    Adenocarcinoma · Third mostcommon epithelial malignancy next only to sinonasal gland carcinoma. · 4 times frequent in men. ? Occupational exposure to wood dust. · Commonly arise from olfactory cleft. · Usually appears polypoidal · Unilateral expansion of olfactory cavity and opacification in imaging - ? suspicion
  • 7.
  • 8.
    Etiology · Exposure tocarcinogen · Smoking / alcoholism · Viral
  • 9.
    Carcinogen · Wood dust ·Nickel · Chromium · Polycyclic hydrocarbon · Aflatoxin · Thorotrast – (watch dial makers)
  • 10.
    Wood dust · Adenocarcinoma ·Wood workers – 500 times common · Exposure to hard wood dust – Ebony, mahogany, oak. · Exposure threshold - > 5mg / m3 / day · Chemicals used in wood processing have been eliminated as a cause · Even short periods of exposure can cause adenocarcinoma (< 5 years)
  • 11.
    Industrial risk · Woodindustry · Textile industry · Bakery · Textile · Nuclear industry · Farming · Construction · Mining
  • 12.
    Human papilloma virus ·HPV-6 / HPV-11 demonstrated in 10% of squamous cell carcinoma nose & pns. · HPV – 16 / HPV – 19 are known to cause more virulent cancers. · Presence of squamo columnar junctions in the nose predisposes to HPV induced cancers.
  • 13.
    Tumor spread · Localinvasion · Orbital spread common – thin walls, nerves and blood vessels cause dehiscence · Roof of frontal sinus is thin – perforations + for olfactory nerves to pass
  • 14.
    Ohngren's Line · Linerunning from medial canthus to angle of mandible · Prognosis of suprastructure tumors worse (This was before advent of craniofacial resection)
  • 15.
    Lymphatic drainage · Lymphaticdrainage of this area is scanty. · Anterior / Posterior pathway · Anterior pathway – 1st echelon nodes (facial, parotid, submandibular nodes) · Posterior pathway – 1st echelon nodes (retropharyngeal nodes) · Anteroinferior nasal cavity, skin of nasal vestibule – anterior pathway · Rest of nose and sinuses drain via posterior pathway
  • 16.
  • 17.
    Clinical features · Oralsymptoms – Pain, trismus, alveolar ridge fullness, erosion, loosening teeth, ill fitting dentures (2530%). · Nasal symptoms – Obstruction, epistaxis, rhinorrhoea (50%). · Ocular symptoms – Epiphora, diplopia, proptosis, blindness (25%). · Facial signs – Paresthesias, asymmetry
  • 18.
  • 19.
  • 20.
    MRI · Differentiates tumorfrom soft tissue · Differentiates secretions from tumor mass · Demonstrates perineural spread · Not affected by dental fillings · Can be imaged in sagittal plane · Coronal MRI – Foramen rotundum, vidian canal, foramen ovale and optic canal can be seen
  • 21.
    Angiogram · Tumors surroundcarotid artery · Carotid artery needs to be sacrificed in order to obtain clear surgical margins · Balloon occlusion tests should be performed to estimate the risk of cerebral infarction if carotid needs to be sacrificed.
  • 22.
  • 23.
    Squamous cell carcinoma ·Most common sinonasal malignancy. · Common during 7th decade / males. · Arises – lateral nasal wall. 50% arises from turbinates. · 85% are well differentiated and keratinizing. · 15% of inverted papilloma turns malignant
  • 24.
    Adenocarcinoma · Wood workers ·9% of all sino nasal malignancies · Common 6th – 7th decades · Common in upper nasal cavity / ethmoidal sinuses · Growth rate slow · Metastasis uncommon · Histological types: Papillary, sessile, mucoid, neuroendocrine, intestinal, undifferentiated.
  • 25.
    Adenoid cystic carcinoma ·5% of all sinonasal malignancies · Slow growth, perineural spread, vascular spread · Maxillary sinus commonly affected · Long history of facial pain defying diagnosis
  • 26.
    Olfactory neuroblastoma · Arisesfrom basal cells of olfactory epithelium · 5% of sinonasal malignancies · Bimodal distribution (20 and 50 yrs old peak). · More common in women than men · Paraneoplastic syndrome +
  • 27.
    Kadish staging system ·Stage A – Tumor limited to nasal cavity · Stage B – Tumor limited to nose and sinuses · Stage C – Tumor extending beyond the confines of nose and sinuses · Stage D – distant metastasis
  • 28.
    ULCA Staging Stage Description T1 Tumor involvingthe nasal cavity or paranasal sinuses (excluding sphenoid) or both, sparing the most superior ethmoidal air cells T2 Tumor involving the nasal cavity or paranasal sinuses (including the sphenoid) or both with extension to or erosion of the cribriform plate T3 Tumor extending into the orbit or protruding into the anterior cranial fossa T4 Tumor involving the brain
  • 29.
    Undifferentiated carcinoma · Anaplastic ·Aggressive tumor · Produces fewer symptoms · Chemoradiation +
  • 30.
    Melanoma · 4% ofsinonasal malignancies · Common in women than men · Affects elderly · Nasal cavity / septum common sites · Polypoidal / ulceration · Metastasis less frequently to nodes · Lungs / brain metastasis common
  • 31.
    Lederman's classification · Linesof Sebileau · Supra, meso and infrastructures · Prognosis worsens from below upwards
  • 32.
    Growth maxilla –staging TNM T1 Tumor confined to antrum – No bone erosion T2 Tumor with bone destruction except posterior wall of antrum T3 Erosion of posterior wall / infratemporal fossa / pterygoid plates / orbit / ethmoid sinus T4a Anterior orbital contents / cribriform plate / sphenoid / frontal sinus T4b Orbital apex / dura / brain / middle fossa / nasopharynx / clivus
  • 33.
    Ethmoid sinus -TNM T1 Tumor confined to ethmoid / with or without bone erosion T2 Tumor extending into nasal cavity T3 Tumor extending to anterior orbit / maxillary sinus T4a Anterior orbital contents / Skin of nose or cheek / minimal anterior cranial invasion / pterygoid plates / sphenoid / frontal sinus Orbital apex / dura / brain / middle cranial fossa / cranial nerves other than V2 / nasopharynx / Nasal cavity T4b
  • 34.
    Nasal cavity Subsites recognized– septum / floor / lateral wall / vestibule T1 Tumor involving one subsite T2 Tumor involving two subsites / ethmoid T3 Tumor eroding to anterior orbit / maxillary sinus T4a Anterior orbit / skin of nose and cheek / minimal anterior cranial fossa extension / pterygoid plates / sphenoid / frontal sinus T4b Orbital apex / dura / brain / middle cranial fossa / nasopharynx / clivus / cranial nerves other than V2
  • 35.
    Treatment · Surgery · Radiotherapy ·? Chemotherapy · Combination
  • 36.
    Irradiation · Preop irradiationpreferable · Post op irradiation is suitable only for slow growing tumors · 200 rads x 5 days a week – 6 weeks (6000rads)
  • 37.
    Surgery · Partial maxillectomy ·Total maxillectomy · Extended maxillectomy
  • 38.
    Medial maxillectomy · Goodaccess to nasal cavities / ethmoids / nasopharynx / sphenoid / pterygopalatine fossa · Moore's incision · Incision may be continued into nasal cavity
  • 39.
  • 40.
    Anterior craniofacial resections ·Type I – Craniofacial / transorbital resection. This procedure is extended medial maxillectomy with resection of ethmoid roof and orbital periosteum · Type II – Medial maxillectomy with window craniotomy using frown line incision · Type III – Neurosurgeon helps. Transfacial with neurosurgical approach like frontolateral craniotomy
  • 41.