sino nasal malignancies
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Nasal Cavity
and
Para nasal Sinuses
Cancer
by
Osama El-Zaafarany
Incidence:
3% of aerodigestive malignancies
1% of all malignancies
Males : females = 2 : 1.
Sixth to seventh decades
The maxillary sinus is most commonly involved with
tumor, followed by the nasal cavity, the ethmoids, and
then the frontal and sphenoid sinuses.
Anatomy
Maxillary antrum:
Superior : orbit, ethmoids
Posterior : pterygoids,
infratemporal fossa.
Ethmoid sinus:
Superior : fovea, cribiform
Medial : lamina papyracea
Sphenoid sinus
Superior : optic nerve, pituitary
Lateral : ICA, cavernous sinus
Inferior : nasopharynx.
Frontal sinus
Inferior: orbit.
Posterior: anterior cranial fossa
Lymphatic Drainage
The anterior nose has the same lymphatic drainage as the external nose. These tend to spread to the submental or level I area.
The posterior nose tends to drain to the retropharyngeal nodes as well as the lateral pharyngeal nodes, which eventually drain into the level II.
Etiological Factors:
Exposure:
Wood, nickel-refining processes
Industrial fumes, leather tanning
Specific asssociations found include squamous cell
carcinoma in nickel workers and adenocarcinoma in
workers exposed to hardwood dusts and leather
tanning.
Cigarette and Alcohol consumption
No significant association has been shown
Pathologic classification: 1) Squamous cell carcinoma
2) Adenoid cystic carcinoma
3) Mucoepidermoid carcinoma
4) Adenocarcinoma
5) Hemangiopericytoma
6) Melanoma
7) Olfactory neuroblastoma
8) Sarcoma: osteogenic, fibrosarcoma, chondrosarcoma, rhabdomyosarcoma
9) Lymphoma: (NHL, NK/T-cell lymphoma=lethal midline granuloma).
10) Metastatic tumors (RCC is the most common).
11) Sinonasal undifferentiated carcinoma
Natural History:
Squamous cell carcinoma:
Most common tumor (80%), Males, Sixth decade.
Location:
• Maxillary sinus (70%)
• Nasal cavity (20%), lateral nasal wall is the most common site
88% present in advanced stages (T3/T4).
90% have eroded walls of sinuses.
Regional lymph node metastasis is about 10% to 20% of cases.
Local recurrence rate 30% to 40%.
Adenocarcinoma: 2nd most common, 5-20%
Ethmoids.
Strong association with occupational exposures.
High grade subtype: 30% present with metastasis
Adenoid Cystic Carcinoma:
3rd most common (3-15%).
occurs most frequently in women, and in the fifth and sixth decades.
Palate > major salivary glands > sinuses.
Neck metastasis is rare.
Multiple recurrences, distant mets.
Perineural spread
Resistant to tx.
Postoperative RTx is very important.
Long-term followup necessary
Olfactory Neuroblastoma
Esthesioneuroblastoma:
Originate from stem cells of neural crest origin that differentiate
into olfactory sensory cells.
Kadish staging system:
• A: confined to nasal cavity
• B: involving the paranasal cavity
• C: extending beyond these limits
Aggressive behavior
Local failure: 50-75%
Metastatic disease develops in 20-30%
Treatment is en bloc surgical (craniofacial) resection with
postoperative RTx.
Sinonasal Undifferentiated Carcinoma:
• It is rare type, believed to arise from Schneiderian epithelium, the Sionasal ectodermn.
• Risk factors: Smoking and radiation.
• The median age 6th decade, male predominance.
• Aggressive locally destructive lesion.
• Frequent orbital invasion and intracranial extension.
• Greater tendency to metastasize than squamous carcinoma.
• DD: melanoma, lymphoma, olfactory neuroblastoma, rhabdomyosarcoma, neuroendocrine carcinoma, and poorly differentiated squamous cell carcinoma.
• Prognosis is usually poor, with a median survival of 18 months. Overall survival is a bout 20% at 5 years.
• Preoperative chemotherapy and radiation may offer improved survival if combined with radical surgery.
Staging:
Ohngren’s Line
a line that is drawn from
the angle of mandible to
the medial canthus.
Ohngren indicated that
tumors that presented
above this line
(suprastructure); both
superiorly and
posteriorly, tended to
have a worse prognosis
Clinical Presentation: (delayed diagnosis)
Oral symptoms: 25-35%; Pain, trismus, alveolar ridge fullness, erosion
Nasal findings: 50% Obstruction, epistaxis, rhinorrhea
Ocular findings: 25% Epiphora, diplopia, proptosis
Facial signs: Paresthesias, asymmetry
Classic Triad of advanced disease: • facial asymmetry
• tumor bulge in oral cavity
• nasal mass
Diagnostic workup:
Physical exam
Nasal endoscopy
Biopsy
Radiography (CT, MRI).
Computed Tomography
Bone erosion
85% accuracy
Difficult to differentiate between: Tumor vs.
inflammation vs. secretions
MRI
94% accuracy
Inflammatory tissue & secretions: intense T2
Tumor: intermediate T1 & T2, Enhancement with Gadolinium
If there is a question of neural involvement, MRI is excellent for determining perineural spread, involvement of the dura, or involvement intracranially.
Treatment Recommendations:
Surgery: • Surgical resection is the primary treatment modality for cancers
involving the maxillary or ethmoid sinuses.
• Resection is often limited by tumor involvement of the base of skull
which can result in damage to critical structures such as brain, and the
cranial nerves.
• In the past, contraindications to surgical resection included tumor
extension to the lateral skull base, intracranial contents, or cavernous
sinus. However, with advances in surgical technique and
reconstruction, the decision of more extensive surgery, such as a
craniofacial resection via craniotomy or transglabellar/subcranial
approach can be considered in ethmoid sinus tumors involving
cribriform plate for example.
Unresectability:
extension to frontal lobes
invasion of prevertebral fascia
bilateral optic nerve involvement
cavernous sinus extension
Surgical approaches:
• Endoscopic
• Lateral rhinotomy
• Transoral/transpalatal
• Midfacial degloving
• Combined craniofacial approach
Surgical procedures:
The goal of surgery for nasal cavity and paranasal sinus
tumors is to achieve en bloc resection of all involved
bone and soft tissue with clear margins while
maximizing the cosmetic and functional outcome.
Limited nasal cavity lesions may be resected with
medial maxillectomy.
Ethmoid lesions usually require medial maxillectomy
and en bloc ethmoidectomy.
combined craniofacial procedure for lesions involving
the inferior surface of the cribriform plate and the roof
of the ethmoid.
The bony defect in the anterior cranial floor is closed
with a vascularized pericranial or temporal muscle flap.
maxillary antral cancers: radical maxillectomy that removes en bloc the entire maxilla and ethmoid sinus.
Suprastructure lesions may involve the orbit, necessitating orbital exenteration.
Resection of involved periosteum and frozen-section control of adjacent orbital contents with preservation of the eye may be possible in select lesions with involvement of the periorbita without intraorbital extension
Orbital preservation surgery in select patients with involvement of the bony orbit but not soft tissue does not appear to result in poorer survival or local control than those undergoing exenteration.
Inferior medial maxillectomy
Medial maxillectomy Radical maxillectomy
with excentration
Cranio-facial resection
Indications for orbital exenteration:
Involvement of the orbital apex
Involvement of the extraocular muscles
Involvement of the bulbar conjunctiva or sclera
Lid involvement beyond a reasonable hope for
reconstruction
Non-resectable full thickness invasion through the
periorbita into the retrobulbar fat
Reconstruction after surgery:
Surgery for sinonasal cancers
leaves major defects in the
skull and needs to be
reconstructed.
Advances in tissue transfer
techniques (particularly
microvascular free flaps)
provide reconstructive
options in addition to
maxillofacial prostheses.
Types (Stages) of obturator prostheses:
(I) Immediate (surgical) obturator prostheses: • initiated at the time of surgery
• fabricated on a cast obtained from an impression made at the time of the pretreatment dental examination.
• fabricated using autopolymerizing acrylic resin (methyl methacrylate)
• ligated into position following tumor resection but before flap closure.
(II) Transitional obturator prosthesis: • a minimum of 7 to 10 days after surgery.
(III) Definitive (permanent) obturator prosthesis: • begin once adequate healing has occurred, and radiation therapy is
completed (usually after three to four months).
Radiotherapy: Addition of Rtx. to surgery improve 5-years survival (44%)
when compared to RTx. alone (23%) or surgery alone.
Indications: • Adjuvant (standard of care). • Definitive: medically inoperable or who refuse radical surgery
pre- and postoperative radiation may result in similar control rates.
But post-operative RTx preffered: • Preoperative radiation increases the infection rate and the risk of postoperative
wound complications. • Preoperative radiation may obscure the initial extent of disease=surgery can not
remove the microscopic extensions of the tumor.
Postoperative radiation therapy is started 4 to 6 weeks after surgery.
Indications of elective nodal irradiation:
Not routinely recommended in nasal cavity nor ethmoid sinus tumors.
In maxillary tumors: include ipsilateral submandibular and subdigasteric nodes in:
• Squamous cell carc.
• Poor differ carc.
• T4 lesions.
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N.B. The neck is irradiated after neck dissection for nodal involvement at presentation according to the usual guidelines for postoperative neck irradiation in other head & neck cancers.
Target & Dose for 3D-CRTx.
(I) Definitive RTx:
Recommend 3D-CRT or IMRT planning to
increase sparing of normal structures.
GTV = clinical and/or radiographic gross disease.
CTV1 = 1 cm margin on primary and/or nodal GTV= 66-70 Gy;
(1.8-2Gy/Fx.)
CTV2 = high-risk regions (depending on the presence or absence of
anatomic boundaries to microscopic spread)= 60-63 Gy.
CTV3 = elective neck= 54-57 Gy
(II) Post-operative RTx :
A typical target volume in a postoperative
setting encompasses: 60-66 Gy
• Both halves of the nasal cavity.
• Ipsilateral maxillary sinus.
• If the tumor extends superiorly into the ethmoid air cells:
Ethmoid sinuses and the ipsilateral medial orbital wall are
included
• bony orbit after orbital exenteration
Field Margins:
a three-field technique for maxillary antrum: 1 anterior and 2 lateral fields.
Anterior field: superior border: above the crista galli to encompass the ethmoids.
in the absence of orbital invasion, at the lower edge of the cornea to cover the orbital floor.
inferior border: 1 cm below the floor of the sinus.
medial border: 1 to 2 cm (or more if necessary) across the midline to cover
contralateral ethmoidal extension.
lateral border: 1 cm beyond the apex of the sinus or falling off the skin.
Lateral fields: superior border: follows the floor of the
anterior cranial fossa.
anterior border: behind the lateral bony
canthus parallel to the slope
of the face.
posterior border: covers the pterygoid plates.
Simulation films of wedged-pair setup for a limited lesion
involving the maxillary antrum only.
The treatment volume includes the ipsilateral maxillary
sinus and the nasal cavity.
A, Anterior portal. B, Lateral portal.
OAR & possible complications of RTx.
Lens <10 Gy (cataracts).
Lacrimal gland <30–40 Gy. (dry eye syndrome)
Retina <45 Gy (blindness). incidence of visual loss with Rtx. 12-20% unilateral, 0-8% bilateral.
Optic chiasm and nerves <54 Gy at standard
fractionation. (Optic neuropathy)
Brain <60 Gy (necrosis).
Mandible <60 Gy (osteoradionecrosis).
Parotid mean dose <26 Gy (xerostomia).
Pituitary and hypothalamus mean dose <40 Gy.
Measures to protect the eye during treatment
planning for RTx.
Using advanced techniques; 3D-CRT, IMRT. can provide bilateral sparing of the globe for most patients, it may be more difficult to
spare optic nerves, especially on the ipsilateral side, Good fixation by immobilization devices.
Using nonaxial and noncoplanar fields.
The contralateral eye is blocked, and greater than two thirds of the ipsilateral eye are also blocked unless there is intraorbital infiltration.
With the four-field technique (with interorbital electron portal) the eyes are blocked from the anterior and lateral photon portals.
With the three-field technique, the anterior border of the lateral portal is placed at the bony canthus and the anterior portal is weighted more heavily (2 : 1 to 3 : 1).
Role of IMRT in sinonasal cancers
The dose delivered to the optic pathways can be
selectively reduced by IMRT, which has the potential to
preserve binocular vision, particularly for patients who
have extensive and large-volume disease in the
paranasal sinuses.
In a longitudinal analysis of 127 patients treated with
radiation therapy from 1960 to 2005 at the University
of California, San Francisco, the incidence of grade 3 or
greater late ocular toxicity among patients treated with
3D-CRT=9% and in IMRT=0%.
IMRT isodose plans of a patient with locally advanced paranasal sinus undergoing definitive radiotherapy. A: At the level of the maxillary sinuses/parotid glands;
B: at the level of the floor of the orbit/brainstem;
C: at the level of ethmoid sinuses/mid-orbit.
The bilateral eyes are nicely spared (<45 Gy isodose region) as are the brainstem (<45 Gy isodose region) and the parotid glands (<30 Gy isodose region).
Other RTx. Modalities:
Stereotactic Radiosurgery: could be ued for a
boost for gross residual disease in patients who
have small residual tumor volume at the skull
base
Proton Beam Radiation: for deep-seated
locations requiring high doses of radiation, but
no high level evidence for its use.
Role of Brachytherapy
For small lesions of nasal vestibule.
using a 192 Ir wire implant or intracavitary192 Ir mold.
The recommended doses for low-dose-rate brachytherapy
range from 60 to 65 Gy delivered during 5 to 7 days.
In patients with T1 or T2 a boost of 20 to 25 Gy (LDR)
over 2 days or 18 Gy (HDR; 3 Gy twice daily), following
EBRTx. after 50 Gy, if there is good reduction of tumor
volume.
This technique has been reported as yielding a
2-year local control of 86%.
Role of chemotherapy
Neoadjuvant chemotherapy is sometimes offered in
order to reduce tumor volume, which may permit
removal of tumor with a less morbid resection or
facilitate radiotherapy planning if shrinkage pulls away
tumor from critical structures.
chemotherapy may be given concurrent with
radiotherapy in the management of inoperable tumors
on the basis of improved results in more frequent head
and neck carcinomas.
Follow-up
H&P, labs, and CXR: • every 3 months for frst year,
• every 4 months for second year,
• every 6 months for third year,
• then annually.
Imaging of the H&N:
3 months post-treatment, then as indicated.
Inverted papillomas
47% of Schneiderian papillomas which derived from schneiderian mucosa (squamous) are inverting papillomas.
men, 6th-7th decades, unilateral.
lateral nasal wall.
Recurrence up to 80%.
malignant potential; associated with SCC in 2-13%.
Management:
• The gold standard was lateral rhinotomy with medial maxillectomy.
• Role of RTx.
absolute indication for radiation therapy is when an inverted papilloma is associated with squamous cell carcinoma.
those who had advanced incompletely resected or unresectable lesions that are biologically aggressive.
patients where morbidity in resection would be more pronounced that morbidity of tumor radiation.