Neoplasms of the nose and paranasal sinuses

Department of Maxillofacial SurgeryMedical University of Lublin

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Very rare 3%

Delay in diagnosis due to similarity to benign conditions

Nasal cavity◦ ½ benign

◦ ½ malignant

Paranasal Sinuses◦ Malignant

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Neoplasms of Nose and Paranasal Sinuses

Multimodality treatment

Orbital Preservation

Minimally invasive surgical techniques

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Neoplasms of Nose and Paranasal Sinuses

Predominately of older males

Exposure:◦ Wood, nickel-refining processes

◦ Industrial fumes, leather tanning

Cigarette and Alcohol consumption◦ No significant association has been shown

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Epidemiology

Maxillary sinus◦ 70%

Ethmoid sinus◦ 20%

Sphenoid◦ 3%

Frontal◦ 1%

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Location

Oral symptoms: 25-35%

◦ Pain, trismus, alveolar ridge fullness, erosion to oral

◦ cavity

Nasal findings: 50%

◦ Obstruction, epistaxis, rhinorrhea, discharge, stuffiness, congestion hard,

Ocular findings: 25%

◦ Epiphora, diplopia, proptosis, exophtalmus, fullnes of the lid

Facial signs

◦ Paresthesias ( infraorbital nerve), asymmetry, cheek swelling

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Presentation

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With advanced disease, the classic triad of findings for carcinoma of the nasal cavity and paranasal sinuses may be present. These include(1) facial asymmetry, (2) a visible or palpable tumor bulge in the oral cavity, and(3) tumor visible in the nose with anterior rhinoscopy.

All three will be present in 40 to 60% of patients, but at least one will be present in 90% at the time of diagnosis.

The diagnosis of sinonasal malignancies is challenging. Not only they are rare, but they are difficult to distinguish from their benign counterparts.

CT

◦ Bony erosion

◦ Limitations with periorbital involvement

◦ CT scanning is slightly more accurate than MRI in demonstration of orbital invasion due to its ability to evaluate both the bony orbital wall and adjacent fat

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Radiography

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MRI94 -98% correlation with surgical findingsInflammation/retained secretions: low T1, high T2Hypercellular malignancy: low/intermediate on both

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PETAn 18fluorodeoxyglucose–positron emission tomography scan generally does not play an important role in the initial assessment of sinonasal tumors. However, it is particularly

useful in posttreatment surveillance to diagnose viable tumors

Tissue diagnosis of a lesion of the nasal cavity or paranasal sinuses is required before undertaking definitive treatment, except in selected circumstances in which radiological characteristics may be sufficient to establish the diagnosis (e.g., angiofibroma). The approach to the biopsy of a sinonasal tumor is dictated by the anatomic location and radiological characteristics.

Transnasal endoscopy under general anesthesia is important for accurately delineating the extent of the tumor and obtaining a biopsy specimen for tissue diagnosis.

In addition, tissue diagnosis for otherwise inaccessible sinonasal tumors can be secured with a CT-guided needle biopsy.

Biopsy

Papillomas

Osteomas

Fibrous Dysplasia

Neurogenic tumors

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Benign Lesions

Vestibular papillomas

Schneiderian papillomas derived from schneiderian mucosa (squamous)◦ Fungiform: 50%, nasal septum◦ Cylindrical: 3%, lateral nasal wall/sinuses◦ Inverted: 47%, lateral nasal wall◦ Association with carcinoma is seen in 2 to 13% of

cases.◦ Recurrence rate varies widely from 0 to 80%

depending on the initial method of surgical control.

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Papilloma

4% of sinonasal tumors

Site of Origin: lateral nasal wall

Unilateral

Malignant degeneration in 2-13% (avg 10%)

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Inverted Papilloma

Initially via transnasal resection:◦ 50-80% recurrence

Medial Maxillectomy via lateral rhinotomy:◦ Gold Standard◦ 10-20%

Endoscopic medial maxillectomy:◦ Key concepts: Identify the origin of the papilloma Bony removal of this region

Recurrent lesions:◦ Via medial maxillectomy vs. Endoscopic resection◦ 22%

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Inverted PapillomaResection

Benign slow growing tumors of mature bone

Location:

◦ Frontal, ethmoids, maxillary sinuses

When obstructing mucosal flow can lead to mucocele formation

Treatment is local excision

They are most often seen in the 15 to 40 age group. Treatment if necessary is by local excision with a margin of normal bone

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Osteomas

Dysplastic transformation of normal bone with collagen, fibroblasts, and osteoid material

In the maxilla, it usually affects the canine fossa area or the zygomatic area. The radiographic picture is fairly typical and described as a ground-glass appearance.

Surgical excision for obstructing lesions

Malignant transformation to rhabdomyosarcoma has been seen with radiation

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Fibrous dysplasia

4% are found within the paranasal sinuses

Schwannomas

Neurofibromas

Treatment via surgical resection

Neurogenic Sarcomas are very aggressive and require surgical excision with post op chemo.

When associated with Von Recklinghausen’s syndrome: more aggressive (30% 5yr survival).

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Neurogenic tumors

Squamous cell carcinoma Adenoid cystic carcinoma Mucoepidermoid carcinoma Adenocarcinoma Hemangiopericytoma Melanoma Olfactory neuroblastoma Osteogenic sarcoma, fibrosarcoma,

chondrosarcoma, rhabdomyosarcoma Lymphoma Metastatic tumors Sinonasal undifferentiated carcinoma

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Malignant lesions

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

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Squamous cell carcinoma

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It is primarily a disease of males and presents most commonly in the sixth decade.Spread outside the sinuses is almost the rule at presentation. More than 90% will have invaded through at least one wall of the involved sinus when discovered.

88% present in advanced stages (T3/T4)

Surgical resection with postoperative radiation◦ Complex 3-D anatomy makes margins difficult

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Treatment

3rd most common site is the nose/paranasal sinuses

The paranasal sinuses comprise 14-17% of all cases

Perineural spread◦ Anterograde and retrograde

Despite aggressive surgical resection and radiotherapy, most grow insidiously.

Neck metastasis is rare and usually a sign of local failure

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Adenoid Cystic Carcinoma

Extremely rare

Widespread local invasion makes resection difficult, therefore radiation is often indicated

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Mucoepidermoid Carcinoma

2nd most common malignant tumor in the maxillary and ethmoid sinuses

Present most often in the superior portions

◦ Strong association with occupational exposures.Most are related to occupational exposures as it is believed that dust particles will travel along the middle turbinate and the larger particles will be deposited there.

◦ They present similarly to the squamous cell carcinoma and are divided histologically into high and low grades.

High grade: solid growth pattern with poorly defined margins. 30% present with metastasis

Low grade: uniform and glandular with less incidence of

perineural invasion/metastasis.

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Adenocarcinoma

Hemangiopericytoma

Pericytes of Zimmerman. More than 80% of these are said to involve the ethmoids.

Sinonasal hemangiopericytomas are considered neither malignant nor benign, but "intermediate" in behavior.

Metastases are rare. Present as rubbery, pale/gray, well circumscribed lesions

resembling nasal polyps Treatment is surgical resection with postoperative XRT for

positive margins

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0.5- 1.5% of melanoma originates from the nasal cavity and paranasal sinus.

Anterior Septum: most common site

The highest incidence is in patients in their fifth to eighth decades.

The nasal cavity is most frequently involved with the anterior septum most commonly seen.

The maxillary antrum is the most frequently seen sinus location.

Typically it is seen as a polypoid fleshy mass and its pigmentation varies. Initial metastasis to regional lymph nodes is not prevalent which is misleading as 5 and 10 year survival is on the order of 38 and 17%.

Treatment is wide local excision with/without postoperative radiation therapy

AFIP: Poor prognosis◦ 5yr: 11%◦ 20yr: 0.5%

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Melanoma

Originate from stem cells of neural crest origin that differentiate into olfactory sensory cells.

Kadish Classification◦ A: confined to nasal cavity

◦ B: involving the paranasal cavity

◦ C: extending beyond these limits

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Olfactory NeuroblastomaEsthesioneuroblastoma

These are rare lesions arising from in the upper part of the nasal cavity from stem cells of neural crest origin that differentiate into olfactory sensory cells.

Occurs in two age peaks, namely around 20 and 50.

It is a slow-growing tumor that forms an exophyticpolypoid or sessile mass with a congested appearance and usually a smooth surface. Larger tumors may be ulcerated. Initially it is unilateral but extension to the opposite side of the nasal cavity and paranasal sinuses occurs with growth.

Kadish proposed a clinical staging system with group A tumors being confined to the nasal cavity, group B involving the paranasal sinuses, and group C extending beyond these limits.

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Olfactory NeuroblastomaEsthesioneuroblastoma

Aggressive behavior

Local failure: 50-75%

Metastatic disease develops in 20-30%%.

Most often metastases are to regional lymph nodes, lungs, and bones.

Treatment:

◦ En bloc surgical resection with postoperative radiation

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Olfactory NeuroblastomaEsthesioneuroblastoma

Osteogenic Sarcoma◦ Most common primary malignancy of bone.◦ Mandible > Maxilla◦ Sunray radiographic appearance

Fibrosarcoma Chondrosarcoma-These are seen in the third to fifth

decades with an equal male:female incidence. Histologic differentiation between benign and malignant types can be difficult but the malignant variety predominates. Distant metastases are rare. Death is by slow erosive destruction of the skull base.

Treatment is often inadequate because of difficulty in establishing margins.

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Sarcomas

Most common paranasal sinus malignancy in children

They arise in the head and neck in 35 to 45% of cases.

8% of these are found in the sinonasal tract.

Three types are described: embryonal and alveolar, which are most common in children, and pleomorphic, more commonly seen in adults.

Triple therapy with surgery, chemotherapy, and irradiation is often necessary.

Aggressive chemo/XRT has improved survival from 51% to 81% in patients with cranial nerve deficits/skull/intracranial involvement.

Adults, Surgical resection with postoperative XRT for positive margins.

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Rhabdomyosarcoma

Sinonasal lymphoma has a bimodal presentation, affecting the very young but more frequently seen in men between the fifth and seventh decades.

They are usually of the non-Hodgkins type and 70% of the lesions are stage 4 at presentation.

Treatment is by radiation, with or without chemotherapy. Children do remarkably better than adults who suffer relapse frequently.

If there is recurrence, ultimate survival drops to around 10%.

Survival drops to 10% for recurrent lesions

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Lymphoma

Aggressive locally destructive lesion

Dependent on pathological differentiation from melanoma, lymphoma, and olfactory neuroblastoma

Preoperative chemotherapy and radiation may offer improved survival

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Sinonasal Undifferentiated Carcinoma

Renal cell carcinoma is the most common

Palliative treatment only

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Metastatic Tumors

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Staging of Maxillary Sinus Tumors

Öhngren described an imaginary plane defined by a line joining the medial canthus of the eye to the angle of the mandible. This plane divides the region of the nasal cavity and maxillary antrum in half . The anatomic region located anterior and inferior to this plane is called the “infrastructure,” and the region located posterosuperior to this plane is called the “suprastructure.” In patients with lesions arising from the infrastructure, symptoms generally develop early during the course of the disease and tumors are readily amenable to a satisfactory surgical resection with an excellent chance for local control.

On the other hand, in patients with lesions involving the suprastructure, symptoms develop late in the course of the disease. These tumors are technically difficult to resect because they often extend to the infratemporal fossa, pterygomaxillary fossa, orbit, base of the skull, and/or anterior cranial fossa. The potential for cure of these lesions is clearly less likely compared with tumors arising in the infrastructure.

Tumors of the infrastructure of the maxillary antrum may extend through the floor of the antrum into the oral cavity, through its medial wall into the nasal cavity, through its anterior wall to the soft tissues of the cheek, or through its lateral wall into the masticator space .

On the other hand, tumors of the suprastructure spread by extension through the posterior wall of the antrum into the pterygomaxillary space, infratemporal fossa, and the middle cranial fossa; through the roof of the antrum into the orbit; or via the ethmoid cavities to the anterior cranial fossa. Primary malignant tumors of the nasal cavity may invade the hard palate, maxillary antrum, ethmoid cavities, or orbit by local extension

Ethmoid tumors can extend to the sphenoid sinus, anterior cranial fossa, orbits, nasal cavity, or nasopharynx or into the maxillary antrum .

Although primary tumors of the frontal and sphenoid sinuses are uncommon because of local spread from these tumors into the cranial cavity with invasion of the dura and brain, they are rarely suitable for curative resection . Overall, dissemination to regional lymph nodes is relatively infrequent, occurring in less than 10% of all patients with

malignant tumors of the paranasal sinuses.

T1: limited to antral mucosa without bony erosion

T2: erosion or destruction of the infrastructure, including the hard palate and/or middle meatus

T3: Tumor invades: skin of cheek, posterior wall of sinus, inferior or medial wall of orbit, anterior ethmoid sinus

T4: tumor invades orbital contents and/or: cribriform plate, post ethmoids or sphenoid, nasopharynx, soft palate, pterygopalatine or infratemporal fossa or base of skull

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Staging of Maxillary Sinus Tumors

The surgical approach selected should provide adequate exposure for a safe and satisfactory surgical resection.

The surgical approach is dependent on the anatomic location, extent, and histology of the tumor.

Transnasal endoscopic resection is an emerging approach for management of limited, centrally located tumors. As experience with this approach increases, it is being used for a broader range of benign and even malignant tumors.

Endoscopic approaches require special instrumentation and considerable experience and thus should be performed only in established centers by persons with special expertise. In general, endoscopic resections are best reserved for tumors in the anterior nasal cavity and selected centrally located

tumors in the posterior nasal cavity.

Surgery

Malignant tumors of the “infrastructure” of the maxilla, including tumors of the upper gum, hard palate, or floor of the maxillary antrum, can be adequately excised by a transoral partial maxillectomy.

A sublabial degloving approach is suitable for larger tumors of the anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus, and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through the peroral approach.

This approach is often used for large

benign tumors of the nasopharynx,

such as angiofibrom

More extensive tumors require a facial incision to provide adequate exposure. Small tumors localized to the lower part of the nasal cavity and nasal septum that are inaccessible for endoscopic excision through the nasal vestibule are best approached via a lateral rhinotomy.

Although the “classic” Weber-Ferguson-Dieffenbach incision provides excellent exposure for resection of tumors of the maxilla, it can lead to an aesthetically unacceptable scar, distortion of facial contour and expression, and ectropion

Unresectable tumors:◦ Superior extension: frontal lobes

◦ Lateral extension: cavernous sinus

◦ Posterior extension: prevertebral fascia

◦ Bilateral optic nerve involvement

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Surgery

Surgical approaches:◦ Endoscopic◦ Lateral rhinotomy◦ Transoral/transpalatal◦ Midfacial degloving◦ Weber-Fergusson◦ Combined craniofacial approach

Extent of resection◦ Medial maxillectomy◦ Inferior maxillectomy◦ Total maxillectomy

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Surgery

Peroral Partial Maxillectomy (Infrastructure Maxillectomy)

Medial Maxillectomy

A medial maxillectomy is indicated for well-differentiated or low-grade malignant tumors, inverted papillomas, and other tumors of limited extent on the lateral wall of the nasal cavity or the medial wall of the maxillary antrum.

Subtotal Maxillectomy

A subtotal maxillectomy essentially removes the entire maxilla except the floor of the orbit and thus includes the infrastructure and suprastructure of the maxilla.

If the floor of the orbit is also resected, the operation is termed a “total maxillectomy.”

Complete removal of the maxilla becomes necessary when a primary tumor arising from the surface lining of the maxillary sinus fills up the entire antrum. Primary mesenchymal tumors arising in the maxilla such as soft tissue and bone sarcomas also require total removal of the maxilla to encompass the entire lesion.

Although the surgical approach for total maxillectomy is similar to that used for partial maxillectomy, a much wider exposure is necessary. With proper care and attention to detail, it usually is possible to remove the entire maxilla as a monobloc specimen for total removal of tumors contained within it.

In that setting, consideration should be given to reconstruction of the floor of the orbit to prevent ptosis of the globe

Total Maxillectomy with Orbital Exenteration

A radical maxillectomy with orbital exenteration is indicated when a primary tumor of the nasal cavity or paranasal sinuses extends into the orbit through the orbital periosteum. Orbital exenteration of a functioning eye with normal vision is considered only if the possibility of a curative resection exists.

Removal of a functioning eye for a palliative operation is not recommended.

Total Maxillectomy with Orbital Exenteration and Reconstruction with Free Tissue Transfer

When a radical maxillectomy with orbital exenteration requires sacrifice of the overlying skin of the face, the surgical defect becomes quite complex and difficult to repair without a microvascular free flap. Prosthetic restoration alone, without soft tissue support and skin coverage, leaves a significant functional and aesthetic debility and is not recommended.

130 maxillectomies only 7.7% required tracheostomy

Of those not receiving tracheostomy during surgery, only 0.9% experienced postoperative airway complications

Tracheostomy is unnecessary except in certain circumstances (bulky packing/flaps, mandibulectomy)

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Tracheostomy

Before 1970’s orbital exenteration was included in the radical resection

Preoperative radiation reduced tumor load and allowed for orbital preservation with clear surgical margins

Currently, the debate is centered on what “degree” of orbital invasion is allowed.

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Treatment of the Orbit

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

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Current indications for orbital exenteration

Radiation therapy

Primary large tumor only for palliation

10-15% improved 5 year survival

XRT = 23% vs. Surgery + XRT = 44%

preoperative vs. postoperative

protection of CNS and globe

◦ XRT 12-20% unilateral visual loss, 0-8% bilateral visual loss

Chemotherapy

Palliation, unresectable disease

(+) margins, perineural spread, surgical refusal,

Intraarterial chemotherapy◦ Robbins - 86% response of T4 lesions

◦ Lee - 91% satisfactory response

Neoplasms of the nose and paranasal sinus are very rare and require a high index of suspicion for diagnosis

Most lesions present in advanced states and require multimodality therapy

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Conclusions

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McCary S, Levine P, Cantrell R. Preservation of the eye in the Treatment of Sinonasal Malignant Neoplasms with Orbital Involvement: A Confirmation of the Original Treatise. Archives of Oto-HNS. Vol 122(6). June 1996. Pp 657-659.

Myers E, Suen J. Cancer of the Head and Neck, 3rd Edition: Neoplasms of the Nose and Paranasal Sinuses. W.B. Saunders Company. 1996.

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