Neoplasms of the Nose and Paranasal Sinuses
Lee A. Zimmer
Ricardo L. Carrau
EPIDEMIOLOGY
Malignant tumors of the sinonasal tract constitute about 3% of tumors arising in the upper respiratory tract. They occur most commonly in whites, and the incidence in males is twice the incidence in females (1). Exposure to industrial fumes, wood dust, nickel-refining processes, and leather tanning has been implicated in the carcinogenesis of certain types of sinonasal malignant tumors. Other industrial exposures associated with an increased incidence of sinonasal cancer include mineral oils, chromium and chromium compounds, isopropyl oils, lacquer paint, soldering and welding, and radium dial painting. A recent report demonstrates a higher incidence of nasal cancers in cigarette smokers (1).
EVALUATION
Diagnosis
Tumors of the sinonasal tract commonly present with symptoms similar to those caused by inflammatory sinus disease, such as nasal airway obstruction, epistaxis, headache, facial pain, and nasal discharge, and are frequently asymptomatic in 9% to 12% of patients, contributing to a delay in the diagnosis and advanced stage of disease. Regional and distant metastases are infrequent despite the advanced stage of the primary tumor. The incidence of cervical metastases on initial presentation varies from 1% to 26%, with most series reporting less than 10%. In one large series, the 5-year incidence of neck metastasis is 4.3% for primary ethmoid malignancies and 12.5% for primary maxillary malignancies (2). This number decreases in patients treated with radiation to the neck. The presence of distant metastasis on initial presentation is even less common, with most authors reporting an incidence of less than 7%.
The physical examination should be thorough, with emphasis on the sinonasal region, orbit, cranial nerves, and should include nasal endoscopy. Although not pathonogmonic, numbness or hypoesthesia of the infraorbital (V2) or supraorbital (V3) nerve strongly suggests malignant invasion. Other findings such as proptosis, chemosis, extraocular muscle impairment, mass effect in the cheek, gingiva or gingivobuccal sulcus (e.g., ill-fitting dentures), and loose dentition also suggest the presence of a sinonasal tumor. Table 129.1 provides a summary of diagnostic techniques.
Radiologic imaging is essential for staging. Plain films may demonstrate bone destruction; however, a significant number will be interpreted as normal. A screening computed tomography (CT) scan is more accurate than plain films to evaluate the bony framework of the paranasal sinuses and compares favorably with the cost of plain films. High-risk patients with a history of carcinogen exposure, severe persistent pain, cranial neuropathies, exophthalmos, chemosis, and sinonasal disease and those with persistent symptoms after adequate medical treatment should be evaluated with an axial and coronal CT scan with contrast or magnetic resonance imaging (MRI) with contrast. CT scanning is superior for the evaluation of the bony confines of the sinonasal tract and skull base. The use of contrast provides an estimate of the tumor vascularity and its relationship to the carotid artery.
MRI differentiates adjacent tumor from soft tissue, differentiates secretions in an obstructed sinus from a spaceoccupying lesion, demonstrates perineural spread, suffers less artifact effect with dental fillings, offers the advantage of imaging in the sagittal plane, and does not involve exposure to ionizing radiation. Coronal MRI images are superior for the evaluation of the foramen rotundum, vidian canal, foramen ovale, and optic canal. Sagittal images are most useful to demonstrate the replacement of the normal low-intensity signal of Meckel’s cave and the high-intensity signal of fat in the pterygopalatine fossa (PtPF) by tumor signals similar to brain. MRI, however, is more expensive than CT scan, more prone to motion artifact, and less tolerated due to claustrophobia.
TABLE 129.1  DIAGNOSIS PARANASAL TUMORS TECHNIQUE | |||||||||||||||||||||||
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Positron emission tomography (PET) is commonly used for malignancies of the head and neck for staging and surveillance. The combined PET/CT scanner adds anatomical detail which aids in surgical planning by defining the extent of tumor. Although many reports have documented the use of PET in head and neck cancer, none have evaluated the use of this modality for malignant tumors of the nose and paranasal sinuses.
Angiography with carotid flow study is reserved for surgical candidates presenting with tumors that surround the carotid artery or when sacrifice of the vessel is anticipated to obtain clear margins. Balloon occlusion tests, used with SPECT, Xenon CT scan, or Transcranial Doppler, offer a reasonable estimate of the risk of ischemic brain infarction if the internal carotid artery is sacrificed. These tests, however, cannot predict ischemia at marginal (“watershed”) areas or embolic phenomena.
A CT scan of the chest and abdomen is recommended for patients presenting with tumors that metastasize hematogenously, such as sarcomas, melanoma, and adenoid cystic carcinoma. Metastatic evaluation is important if an extensive resection is considered. A lumbar puncture, brain and spine imaging are recommended for tumors that invade the meninges or brain.
Pathology
The sinonasal tract pathology, with certain important exceptions, reflects the pathology found in other areas of the head and neck (Table 129.2). We provide a brief description of the most common histologic diagnoses.
Benign Epithelial Tumors
Papillomas arise from squamous or schneiderian epithelium. The keratotic papilloma of the vestibule (vestibular wart) behaves like other cutaneous counterparts. It is easily treated by simple excision or cauterization.
Papillomas of the nasal cavity may be classified in three distinct categories (Table 129.3). Fungiform papillomas arise from the nasal septum, while inverted and cylindrical papillomas typically arise from the lateral nasal wall. Although benign in nature, extension beyond their site of origin can destroy bone, recur when not excised completely, and may be associated with malignant tumors (3,4). They are most commonly diagnosed in white males during the fifth to seventh decades (mean 50 years). En bloc resection has been the gold standard for the treatment of these lesions (Table 129.4). MRI, CT scanning, and nasal endoscopy permit an accurate preoperative mapping of these lesions, allowing a more conservative resection through less invasive approaches. During the last decade, various endoscopic, transnasal techniques for the resection of inverting papillomas have been reported (4,5). Transnasal techniques avoid the use of incisions and usually require a shorter hospital stay than external approaches. The endoscopic approach provides superior visualization of the posterior ethmoid cells, especially those that extend lateral to the sphenoid sinus or around the optic nerve (Onodi cells). In expert hands, resection with an endonasal, medial maxillectomy approach has equal recurrence rates as traditional open, en bloc resections (Table 129.5) (5).
Papillomas of the nasal cavity may be classified in three distinct categories (Table 129.3). Fungiform papillomas arise from the nasal septum, while inverted and cylindrical papillomas typically arise from the lateral nasal wall. Although benign in nature, extension beyond their site of origin can destroy bone, recur when not excised completely, and may be associated with malignant tumors (3,4). They are most commonly diagnosed in white males during the fifth to seventh decades (mean 50 years). En bloc resection has been the gold standard for the treatment of these lesions (Table 129.4). MRI, CT scanning, and nasal endoscopy permit an accurate preoperative mapping of these lesions, allowing a more conservative resection through less invasive approaches. During the last decade, various endoscopic, transnasal techniques for the resection of inverting papillomas have been reported (4,5). Transnasal techniques avoid the use of incisions and usually require a shorter hospital stay than external approaches. The endoscopic approach provides superior visualization of the posterior ethmoid cells, especially those that extend lateral to the sphenoid sinus or around the optic nerve (Onodi cells). In expert hands, resection with an endonasal, medial maxillectomy approach has equal recurrence rates as traditional open, en bloc resections (Table 129.5) (5).
TABLE 129.2 TUMORS OF THE SINONASAL TRACT | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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TABLE 129.3 PAPILLOMAS OF THE SINONASAL TRACT | ||||||||||||||||||||||||
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TABLE 129.4 OPEN MEDIAL MAXILLECTOMY RECURRENCE RATES FOR INVERTING PAPILLOMA | ||||||||||||||||||||||||||||||||||||||||||||||||
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Adenomas of the sinonasal tract arise more commonly in the nasal septum. Most are found during the fourth to the seventh decade, occurring with an equal sex distribution. The recurrence rate is low following complete removal (10%).
Malignant Epithelial Tumors
Squamous cell carcinoma (SCCA) is the most common tumor of the sinonasal tract. It is most commonly reported in white males in their fifth to sixth decade. The prognosis is related to the extent of the tumor and the site of origin.
TABLE 129.5 ENDOSCOPIC RECURRENCE RATES FOR INVERTING PAPILLOMA | ||||||||||||||||||||
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Adenocarcinomas make up 4% to 8% of all sinonasal tumors. They originate most commonly in the ethmoid sinuses and nasal cavity and are associated with exposure to hardwood dust. Adenocarcinomas may be divided into low and high grades according to their histologic characteristics and behavior. Low-grade tumors present a uniform glandular architecture and cytologic characteristics, with rare mitoses and seldom perineural invasion or distant metastases. Low-grade adenocarcinomas tend to recur locally. High-grade adenocarcinoma has a solid growth pattern with poorly defined margins, prominent pleomorphism, and large number of mitoses. One-third of patients with high-grade adenocarcinomas will present with distant metastases. Approaches to adenocarcinomas of the paranasal sinuses include anterior craniofacial resection, lateral rhinotomy, and endonasal techniques with or without radiotherapy. The 5-year disease-specific survival for patients with adenocarcinoma of the nose and paranasal sinuses following surgery and radiation therapy is 100% for T1, 93% for T2, 60% for T3, and 0% for T4 lesions (6). Disease extension to the orbit, infratemporal fossa, frontal sinus and dura are poor prognostic indicators.
Adenoid cystic carcinomas of the sinonasal tract comprise 14% to 20% of all the adenoid cystic carcinomas arising in the head and neck. They are characterized by early spread to neurovascular structures, submucosal spread, and advanced stage at the time of diagnosis. Lowgrade tumors are defined by histology with less than 30% solid architecture and include the cribriform and tubular patterns. High-grade tumors correspond to those with a histologic pattern with more than 30% solid cellular architecture. The incidence of perineural invasion is similar for both grades, but the incidence of local recurrence and metastases is higher in the solid type. High rates of recurrence (50% to 76%) are likely due to perineural spread and positive microscopic margins not identified at the time of surgery (7,8). The treatment of adenoid cystic carcinoma of the sinonasal tract is primarily surgical, although combined surgery and postoperative radiation therapy appear to yield better local control.
In the MD Anderson experience, among the 105 patients presenting with ACC of the sinonasal tract, most patients presented with locally advanced disease (8). Tumor extended to the skull base in 28% of patients and invaded the skull base and brain in 24%. Margins are difficult to clear even with extensive craniofacial resection due to the proximity to vital structures and the propensity for perineural spread, which in this series was 60% of patients. The survival rate at 2 years was 46% following primary treatment and 15% following salvage surgery. Patients recurred locally (31%), and 38% developed regional or distant recurrence. Overall 5-year survival was 50% for stage I to III and 20% for stage IV.
Melanoma of the sinonasal tract may be primary or metastatic. Although 20% of all melanomas originate in the head and neck, less than 1% arises from the sinonasal tract. They are most commonly found in the nasal cavity, followed by the maxillary sinus, ethmoid sinus, and frontal sinus, in descending order. Most patients present with disease confined to the site of origin but show a tendency toward early vascular and lymphatic invasion with a high incidence of local recurrence after surgical excision. Postoperative radiation therapy may be beneficial, although its impact on survival and local control has not been addressed in scientific trials. The median survival for patients presenting with sinonasal melanoma is 15 months with a 5-year overall survival of 23% (9). The most common cause of failure is local recurrence, whereas the most important factor in predicting survival is metastatic disease (9,10).
Olfactory neuroblastoma is a rare tumor arising in the olfactory epithelium. Patients often present with nasal obstruction and epistaxis. It has a bimodal frequency at 10 to 20 and 50 to 60 years of age, with a similar incidence in males and females. Its prognosis is related to the extent of disease and resectability on initial presentation. Most institutions have adopted combined therapies based on the Kadish staging system (Table 129.6). The UCLA classification (Table 129.7), however, provides better prognostication regarding local recurrences as factors such as intradural and orbital invasion are not considered in the Kadish staging system. The advent of open or endoscopic craniofacial resection with postoperative radiation to the primary site and neck has improved the therapeutic results (11). In a recent meta-analysis, the 5-year disease-free survival for all stages is 78% following combined surgery with radiotherapy and 47% for Kadish stage C (12).
TABLE 129.6 KADISH STAGING SYSTEM FOR OLFACTORY NEUROBLASTOMA | ||||||
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TABLE 129.7 UCLA STAGING SYSTEMS FOR OLFACTORY NEUROBLASTOMA | ||||||||||
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Sinonasal undifferentiated carcinomas are usually composed of small and medium-sized cells and must be differentiated from rhabdomyosarcomas, melanoma, olfactory neuroblastoma, lymphoma, and SCCA. The progression of symptoms is very rapid, and they usually present with very advanced stage involving multiple sinuses. Treatment includes trimodal therapy employing chemotherapy (cyclophosphamide, doxorubicin and vincristine), radiation therapy, and, in appropriate cases, surgery. Patients with intracranial disease fared poorly despite aggressive combination therapy.
Benign Nonepithelial Tumors
Fibro-osseous lesions, including osteomas, fibromas, and chordomas, are the most common benign tumors of the sinonasal tract. Their growth is usually slow and self-limited. Simple surgical excision is recommended when a histologic diagnosis is needed or to relieve obstructive symptoms.
Fewer than 4% of benign peripheral nerve sheath tumors of the head and neck arise in the nose and paranasal sinuses. They present as polypoid, slow-growing masses, reaching a very large size and often causing facial deformities and local destruction of adjacent structures. Ninety percent of the nerve sheath tumors show a benign histology. Two-thirds of these tumors are schwannomas and one-third neurofibromas. Unlike other regions, schwannomas of the nose and paranasal sinuses often lack tumor encapsulation, with neoplastic cells undermining adjacent respiratory mucosa (13). Treatment is by complete surgical excision, or partial removal for massive neurofibromas involving vital areas.
Malignant Nonepithelial Tumors
Neurogenic sarcomas are rare in the head and neck and commonly associated with neurofibromatosis. Neurogenic sarcomas are locally aggressive and frequently present with distant metastases. Surgery plays a primary role in their therapy; radiation and chemotherapy are usually reserved for incomplete removal, inoperable cases, or recurrences (14
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