Malignancies of the Paranasal Sinus

CHAPTER 83 Malignancies of the Paranasal Sinus




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).




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.






Lymphatics


Ohngren7 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.





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.




Classic “Open” Procedures






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.





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




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.





SURGICAL APPROACH

The craniofacial frontoethmoidectomy approach described by Kaplan and associates15 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.


Jun 5, 2016 | Posted by in OTOLARYNGOLOGY | Comments Off on Malignancies of the Paranasal Sinus

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