Sino nasal malignancies

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.

ــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ

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.