CHAPTER 83 Malignancies of the Paranasal Sinus

• Malignant paranasal sinus tumors are rare and have relatively poor survival outcomes, often related to advanced stage at diagnosis.

• Symptoms suggesting sinus malignancy include nasal obstruction, pain, epistaxis, swelling, nasal discharge, epiphora, palate lesion, diplopia, cheek numbness, decreased vision, neck mass, proptosis, and trismus.

• Treatment for sinonasal malignant tumors varies widely according to histologic type, tumor location, and institutional experience.

• Advancements in imaging, experience with image-guided endoscopic surgery, and new chemoradiation therapy regimens have significantly altered attitudes regarding the optimal approach to sinus malignancy.

• Understanding the complex three-dimensional anatomy of the sinuses and surrounding structures is a crucial component of therapeutic decision-making.

In the first decade of the 21st century it is difficult to describe the “standard of care” for any malignant process. The rapid evolution of imaging combined with the emergence of effective nonsurgical treatment modalities and the expansion of image-guided surgical technology has created a dynamic therapeutic environment. Management of paranasal sinus malignancy exemplifies this situation, and treatment selection is additionally influenced by the proximity of the sinuses to the eyes and the brain, which limits the opportunity to achieve “clear surgical margins” for the majority of these tumors. Consideration of post-treatment quality of life has clearly influenced treatment selection in the past and is driving current management toward combined therapy and aggressive endoscopic debulking to achieve organ preservation while also preserving the probability of cure.

We present traditional topics in paranasal sinus malignancies and also discuss some of the emerging modalities, including endoscopic resection, concomitant chemoradiation therapy, and image-modulated radiation therapy. Benign lesions in the paranasal sinus and tumors of the nasopharynx are discussed elsewhere in this book.

Recognition of malignant lesions in the paranasal sinuses creates a challenge for patients and practitioners, given that the area is neither visible nor palpable and sinus malignancy initially mimics a benign process. The diagnosis usually becomes apparent only after the lesion has reached an advanced stage, thus explaining the relatively poor prognosis. The otolaryngologist has a unique obligation to be aware of these disorders and to understand the management of these complex tumors.

This chapter first reviews material common to all sinus tumors: anatomy, symptoms, physical findings, diagnostic assessment, and surgical options. Although these lesions are rare, numerous histologic subtypes must be discussed individually because management varies significantly among them (Fig. 83-1).

Figure 83-1. Evaluation and management of suspected paranasal sinus cancer. *Neutron irradiation is an option for adenoid cystic carcinoma. CT, computed tomography; MRI, magnetic resonance imaging.

Epidemiology and Survival

Malignant lesions within the sinuses are uncommon, accounting for less than 1% of all neoplasms, with a frequency of 0.1 to 0.3 per 100,000 during the first decade of life that rises to 7 per 100,000 by the eighth decade of life.¹ Several histologic subtypes have been described. In one of the most widely cited papers on the incidence of malignant sinonasal lesions, Jackson and colleagues² reviewed 115 patients between 1957 and 1974 and reported on the histologic distribution of their tumors (Table 83-1). These rates are similar to those in a review by Sisson and associates³ in 1989. In 2007, the Memorial Sloan-Kettering Cancer Center published their experience with paranasal sinus malignancies; the histologic prevalence remains similar to that described by Jackson 30 years earlier (see Table 83-1).⁴ The most common lesions reported were squamous cell carcinomas, with a frequency of less than 1 per 200,000 per year.

Table 83-1 Rates of Malignant Tumors

	Number Reported (%)
Histology	Jackson et al (1977)²	Hoppe et al (2007)⁴
Squamous cell carcinoma	61 (55)	42 (49)
Undifferentiated carcinoma	11 (10)	4 (5)
Adenoid cystic carcinoma	8 (7)	11 (13)
Adenocarcinoma	7 (6)	6 (7)
Melanoma	7 (6)
Olfactory neuroblastoma	5 (4)	7 (8)
Fibrosarcoma	3 (3)	9 (11)
Papillary adenocarcinoma	2 (2)
Transitional cell carcinoma	2 (2)
Plasmacytoma	2 (2)
Hemangiopericytoma	1 (1)
Lymphoma	1 (1)
Carcinosarcoma	1 (1)
Chondrosarcoma	1 (1)
Neuroendocrine	n/a	2 (3)

A large retrospective review of 220 patients with nasal and paranasal sinus malignancies showed a 5-year survival rate of 40%.¹ The National Cancer Institute created the Survival, Epidemiology, and End Result (SEER) database in 1973. This database enables accurate estimates for cancer incidence and mortality in the United States. In 2006, Davies and Welch⁵ used this database to compile recent incidence and mortality rates for head and neck cancer. The data suggest that mortality for sinus malignancies decreased by 11% between 1990 and 2001.

Numerous environmental agents are considered to have a causal relationship with carcinoma of the paranasal sinuses. The following are all associated with squamous cell carcinoma: aflatoxin, found in certain foods and dust; chromium, nickel, mustard gas, polycyclic hydrocarbons, and other organic chemicals, usually from manufacturing processes; and mesothorium (Thorotrast), a radiopaque dye used as a contrast medium within the antrum. Wood dust has a particular association with adenocarcinoma of the ethmoid sinus. Tobacco smoke, salted or smoked foods, woodworking, heavy metal exposure, and hydrocarbon refining have all been linked with a higher incidence of these malignancies. There is a 2 : 1 male to female preponderance, likely due to the higher rates of male workers in the occupations listed. Additionally, human papillomavirus, including subtypes 6, 11, and 18, has been identified in inverted papilloma and associated squamous cell carcinoma.⁶

Anatomy

Assessment and management of this group of tumors demands an intense personal effort to understand the complex anatomy of the sinuses and skull base. Such knowledge is best obtained through anatomic dissection and repeated review of the anatomy of the human skull. It may be helpful to have a skull available in the operating room for inspection during complex resections, especially those that approach or traverse the skull base.

Pertinent to this discussion are the structures contiguous to each of the sinuses, because they define the extent of disease and determine the design of therapy. It is important to realize that all of the sinuses have venous communications with intracranial veins, which provide an avenue for intracranial spread.

Maxillary Antrum

As noted by Ohngren,⁷ the most prognostically significant structures are above and behind the maxillary antrum. Superior to this structure are the orbit and the ethmoid sinus. The posterior boundary includes the pterygoid plates, the pterygoid space, and the infratemporal fossa. The other boundary areas are more easily resected en bloc and thus are less worrisome when involved. Anteriorly lies the canine fossa, inferiorly the alveolus, and medially the nasal cavity.

Ethmoid Sinus

As with the antrum, the least worrisome directions of spread from the ethmoid sinus are inferior into the antrum and medial into the nasal cavity. Of much greater concern is superior spread, in which the fovea ethmoidalis and the cribriform plate provide little barrier to intracranial spread. Evidence of intracranial spread requires consideration of an anterior craniofacial resection. The lateral wall consists of the thin bone of the lamina papyracea, which also provides little resistance to neoplastic invasion.

Sphenoid Sinus

Located in the center of the head, the sphenoid sinus is immediately adjacent to very complex and vital structures. For this reason, complete resection of a malignant tumor involving the sphenoid sinus is usually not possible. The optic nerve and the pituitary gland are superior to this sinus, and the internal carotid artery and the cavernous sinus sit laterally. The bone of the lateral wall is usually less than 0.5 mm thick and not uncommonly is dehiscent. Anterior to the sinus are posterior ethmoidal cells, and inferiorly are the vidian nerve, the clivus, and the nasopharynx.

Frontal Sinus

The sinus least frequently involved by malignant tumors, the frontal sinus, is bounded anteriorly by the soft tissues of the scalp, inferiorly by the dense frontal beak of the frontal bone, the ethmoidal cells, and the orbit, and posteriorly by the anterior cranial fossa.

Lymphatics

Ohngren ⁷ emphasized the importance of precise understanding of the lymphatic drainage of the nose and paranasal sinuses as a prerequisite to adequate therapy. He contested the established dictum that these tumors do not often metastasize. He held that they do metastasize but that clinical evidence of early metastasis is absent because the primary drainage is to the lateral and retropharyngeal nodes. He believed that the relative success of his therapy hinged on electrocoagulation and irradiation of the rich lymphatic channels passing from the nose and paranasal sinuses posteriorly to the retropharynx which remains an important therapeutic concept.

Symptoms

In the review of 115 patients by Jackson and colleagues,² the symptoms associated with malignancy were nasal obstruction (61%), localized pain (43%), epistaxis (40%), swelling (29%), nasal discharge (26%), epiphora (19%), palate lesion (10%), diplopia (8%), cheek numbness (8%), decreased vision (8%), neck mass (4%), proptosis (3%), and trismus (2%). As the disease becomes more advanced, the tumor infiltrates adjacent structures, giving rise to these symptoms as follows:

Diplopia or vision loss is most often a manifestation of tumor mass compressing or invading the orbit and may also result from direct involvement of the optic or oculomotor nerves at the orbital apex or the cavernous sinus.

Epiphora is caused by obstruction or infiltration of the lacrimal duct situated in the anteromedial aspect of the maxilla.

Facial swelling and malocclusion result from bone destruction and advancement of the tumor into the soft tissues of the face or mouth.

Trismus signals far-advanced tumor invading the muscles of mastication, most commonly the pterygoid muscles.

Neck mass; palpable metastatic adenopathy in the jugular chain is another sign of advanced disease.

Hearing loss usually results from nasopharyngeal extension of the tumor that obstructs the eustachian tube. This finding is important because nasopharyngeal extension of disease usually precludes a clear resection margin in this area.

Facial numbness is a manifestation of tumor invasion of portions of the trigeminal nerve.

Physical Findings

Until the tumor has infiltrated a cranial nerve or facial bones or has grown sufficiently to obstruct a sinus ostium, it silently advances in size and extent. Discovering an asymptomatic sinus tumor at an early stage is truly a rare and fortuitous event. The most common physical features are as follows:

Nasal, facial, or intraoral mass. The intranasal mass is often necrotic, but polypoid mucosa may obscure underlying tumor tissue in the nose. Facial swelling results when an antral tumor erodes into the soft tissues of the cheek. Widening of the upper alveolar ridge or development of loose, nonvital teeth may be the earliest sign of inferior bony invasion. A palatal mass and ulceration are evidence of more advanced disease.

Proptosis. Mild protrusion of the eye may be consistent with tumor compression of the periorbita without frank invasion, but often it reflects intraorbital tumor and implies advanced disease.

Cranial nerve deficits. The commonly involved cranial nerves are II, III, IV, V1, V2, and VI. Involvement of cranial nerves is a manifestation of advanced disease and indicates a poor prognosis.⁸

Diagnostic Assessment

The selection of diagnostic studies should be guided by thorough history-taking and head and neck examination, including a careful assessment of cranial nerve function. Once a working diagnosis of neoplasm has been inferred from the history and physical findings, subsequent studies are directed toward establishing the extent of the lesion and its histology.

Imaging Studies

Computed Tomography

Computed tomography (CT) is the initial imaging modality of choice for paranasal sinus disease. CT is superior to magnetic resonance imaging (MRI) for detection of bony involvement. CT scanning is also particularly helpful for evaluation of tumor involvement of the retroorbital and orbital apex region, where there should be a “central low density region of fat surrounding the optic nerve with a radial arrangement of the extraocular muscles.”⁹ Loss of this plane implies advanced disease because the veins in this area extend directly to the cavernous sinus. CT is also valuable in assessing infiltration of the nasopharynx, where normal anatomy consists of a very thin layer of mucosa over the medial pterygoid plate. Thickening in this region implies tumor infiltration.⁹ When evaluation of intracranial extension is necessary, contrast-enhanced CT may improve the definition of tumor from adjacent brain.

Biopsy

Often a paranasal sinus tumor presents a surface that lends itself to biopsy at the time of initial clinical presentation. In this instance a biopsy specimen obtained with use of local anesthesia and a biting forceps advances the diagnostic evaluation. Infiltration of epinephrine with a local anesthetic may decrease bleeding. Tumors contained within the sinus cavities are ideally sampled transnasally because transcutaneous or transmucosal approaches may breach the margins of a subsequent en bloc resection. One must be mindful of the possibility of a vascular tumor or an encephalocele during biopsy of unilateral nasal masses. Usually palpation of the tumor with an instrument demonstrates its solid nature. If the mass is soft or cystic, the patient should be asked to perform a Valsalva maneuver while the mass is being observed. Expansion of the mass implies an intracranial connection or a major venous connection. If doubt still exists, it is best to perform aspiration with a fine-gauge spinal needle before the biopsy. If the needle aspirate consists of cerebrospinal fluid or blood, CT, MRI, and, possibly, angiography should be considered before a biopsy is performed.

If none of the preceding methods achieves satisfactory material for histologic examination, the tumor must be approached directly. A surgical procedure appropriate to the lesion must be selected (such as frontal trephination, transseptal sphenoidotomy, intranasal antrostomy, or external ethmoidectomy). Performance of biopsy through a standard endoscopic sinus surgical approach is also appropriate when feasible. The operation should be designed to minimize disruption of later en bloc resection. Biopsy may be the only surgical procedure carried out during the patient’s treatment. In that case, wide drainage of the sinus should be carried out at the time of biopsy to allow for the discharge of necrotic debris that results from subsequent therapy.

Surgical Options

Extirpation of paranasal sinus tumors is a challenging exercise in surgical planning, which may be considered in three phases. First, one must assess the bony and soft tissue structures to be included for en bloc resection. Second, the approach must be designed to provide adequate exposure while preserving functional tissue and cosmetic integrity whenever possible. Third, the repair should be planned to use prosthetics or soft tissue techniques to the best advantage. See Figure 83-2 for anatomic reference.

Figure 83-2. Anatomic reference for the surgical options available for treatment of malignancies of the paranasal sinuses.

Rights were not granted to include this figure in electronic media. Please refer to the printed book.

Classic “Open” Procedures

External Ethmoidectomy

INDICATIONS

The most limited operation to be discussed, external ethmoidectomy, is appropriate for removal of small tumors of the ethmoidal region and as an approach to biopsy and drainage of tumors of the sphenoethmoid region and the medial orbit (Fig. 83-3).

Figure 83-3. External ethmoidectomy.

BONY EXCISION

The limits of bony resection are the medial orbital wall and the ethmoidal labyrinth.

SURGICAL APPROACH

The surgical approach is through an incision on the lateral wall of the nose.

BENEFITS

The external ethmoidectomy approach allows excellent cosmesis and preservation of functional tissue.

LIMITATIONS

External ethmoidectomy will not provide en bloc excision for any but the most limited tumors (middle turbinate). There is a tendency for fistula formation from the skin to the nasal cavity when this area is irradiated.

Inferior Medial Maxillectomy

INDICATIONS

Inferior medial maxillectomy is designed for resection of the medial wall of the antrum and the inferior turbinate. It is most often used for management of an inverted papilloma (Fig. 83-4).

Figure 83-4. Inferior medial maxillectomy.

BONY EXCISION

The margins extend laterally to the infraorbital foramen, inferiorly to the floor of the nose, superiorly to the lacrimal fossa and the middle meatus, and posteriorly to the dorsal end of the inferior turbinate.

SURGICAL APPROACH

A lateral rhinotomy incision is frequently used; the same bony excision may be achieved through a sublabial approach, although an en bloc resection may be more difficult with the latter approach.

BENEFITS

Inferior medial maxillectomy allows adequate exposure and resection for limited tumors while preserving functional tissue and providing a very acceptable cosmetic result.

LIMITATIONS

Inferior medial maxillectomy provides en bloc removal of a limited area.

Medial Maxillectomy

INDICATIONS

Medial maxillectomy may be used for larger benign or intermediate tumors involving the entire lateral nasal wall but without extension to the orbit, anterior cranial fossa, lateral maxilla, or alveolus (Fig. 83-5).

Figure 83-5. Medial maxillectomy.

BONY EXCISION

The block removed contains the lateral nasal wall, including all turbinate tissue, and the contents of the ethmoid and maxillary sinuses.

SURGICAL APPROACH

An extended lateral rhinotomy incision provides excellent exposure. In some instances the same procedure may be accomplished by combining an ethmoidectomy incision with a sublabial incision.

BENEFITS

An en bloc resection may be accomplished with little resulting cosmetic deformity.

LIMITATIONS

Postoperative epiphora due to division of the nasolacrimal duct is not uncommon. The posterior and superior margins of resection should be monitored by means of a frozen section when indicated. Removal of all turbinate tissue results in an abnormal nasal cavity, which may require long-term management of crusting.

Radical Maxillectomy

INDICATIONS

Radical maxillectomy is the standard operation for advanced carcinoma of the maxilla (Fig. 83-6). Considerable debate exists regarding the proper application of this procedure, because the amount of tissue to be removed depends on a careful assessment of the extent of tumor involvement. Another uncertainty relates to the establishment of proper indications for removal of the orbital contents in combination with radical maxillectomy.

Figure 83-6. Radical maxillectomy.

BONY EXCISION

Complete radical maxillectomy involves removal of the maxilla along with the nasal bone, the ethmoid sinus, and, in some instances, the pterygoid plates.

SURGICAL APPROACH

The Weber-Ferguson incision is used with extensions around the eyelids to preserve those soft tissues. A skin or dermis graft is used to line the defect.¹⁰ A preformed obturator can be of great assistance in the immediate postoperative period because it acts as a support for packing and provides immediate dental function.

BENEFITS

Radical maxillectomy is adequate treatment for malignant tumors confined to the maxilla and those with extension to the facial soft tissues, lateral palate, or anterior orbit but without invasion of the eye, ethmoidal roof, posterior orbit, or pterygoid region. Historically, when the procedure was supplemented by irradiation, a cure rate approximating 30% was expected.² However, in the update by Sisson and associates³ on their experience, patients who received multimodality therapy consisting of surgery, irradiation, and chemotherapy achieved a 5-year survival rate of 49%.³

There is little debate regarding the indications for resecting the orbital contents in patients with advanced paranasal sinus carcinoma.^10a As indicated by Ketcham and Van Buren,⁸ the decision to excise the orbital contents was made on the basis of achieving an en bloc resection of the tumor. However, those researchers implied that as imaging became more precise, more selective resection might be possible. Perry and colleagues¹¹ emphasized the impact of CT on surgical planning and documented that selective preservation of the orbital contents is appropriate and achieves adequate local control in properly selected cases.¹¹ This finding was consistent with the experience of Weymuller and coworkers,¹² who also could not demonstrate better local control or survival when the orbital contents were included in maxillectomy procedures. This position was later further supported by the experience of Larson and associates.¹³

LIMITATIONS

Even when orbital exenteration is included, a maxillectomy fails to provide an adequate resection when the tumor has escaped superiorly (ethmoidal roof) or posteriorly (orbital apex, pterygoid region). When these areas are demonstrably involved, one must decide whether to undertake craniofacial resection or use a regimen as described by Sakai and colleagues ¹⁴ that relies on chemotherapy, curettage, and irradiation.

Craniofacial Frontoethmoidectomy

INDICATIONS

Craniofacial frontoethmoidectomy is specifically designed to provide en bloc resection for tumors of the ethmoidal and frontal regions (Fig. 83-7). By including exposure of the anterior cranial fossa, the procedure makes possible complete resection of the ethmoid and frontal sinuses, and dural resection may be included when necessary. As previously noted, one or more margins of these extensive resections are often limited by proximity to the eye or brain, resulting in surgical margins that are “microscopically positive” for tumor.

Figure 83-7. Craniofacial frontoethmoidectomy.

BONY EXCISION

Resection may include the anterior cranium (including the frontal sinus), the floor of the anterior cranial fossa, the ethmoid labyrinth (and the eye when necessary), and the nasal septum. If the tumor crosses the midline, the procedure may be performed bilaterally.

SURGICAL APPROACH

The craniofacial frontoethmoidectomy approach described by Kaplan and associates ¹⁵ allows excellent exposure with preservation of the supraorbital rim to decrease the cosmetic impact of the procedure. Although removal of the eye is unpleasant to consider, Ketcham and Van Buren⁸ have documented an improved survival when orbital resection is combined with craniofacial resection (57%, vs. 26% without exenteration).

BENEFITS

Craniofacial exposure provides direct visualization of the cribriform plate and the fovea ethmoidalis and the potential for en bloc removal. It also offers wide exposure to allow effective repair of dural tears, thus decreasing the chance of a postoperative cerebrospinal fluid leak and intracranial infection.

An international collaboration reviewing 188 patients published a report in 2005. The reported mortality associated with craniofacial resection was 4.5%, and the complication rate was 33%.¹⁶ Surgical margins, histologic type, and intracranial extension were independent predictors of survival. Shah and associates¹⁷ reported their experience with 71 patients, which showed an overall 5-year survival of 56% and a median survival of 4.2 years. Like the international group, Shah and associates¹⁷ showed that intracranial involvement predicted significantly worse survival.

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Malignancies of the Paranasal Sinus

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