Uncommon cancers in the roof of the pharynx are called nasopharynx cancer and are discussed here.
Cancers in the sinuses in the face (nasosinus) including the maxilla and ethmoid sinus are discussed here.
There are other uncommon cancers in this area such as Olfactory neuroblastoma (esthesioneuroblastoma) and is discussed here.
NASAL VESTIBULE — The nasal vestibule is lined with squamous epithelium. As such, tumors arising there are most often squamous cell carcinomas (SCCA) and have a natural history similar to that of squamous cell skin cancer. Primary tumors of the nasal vestibule are usually relatively limited at presentation and exhibit a more favorable prognosis than tumors of the nasal cavity.
Overview — The nasal vestibule is a pear-shaped cavity which functions as the entrance to the nasal cavity. Its borders are the nasal septum and columella medially, the lower lateral (alar) cartilage laterally, and the pre-maxilla inferiorly. The vestibule terminates posteriorly at the limen nasi, the junction of the lower and upper lateral cartilages, as well as the transition from skin to mucosa. The vestibule is lined by skin bearing hair follicles, sebaceous glands, and sweat glands.
The male-to-female ratio of cancer of the nasal vestibule is 2:1, with an average age of 60 to 65 years. The development of nasal vestibule tumors has been associated with smoking tobacco. Although the majority of nasal vestibule tumors are squamous cell carcinomas, basal cell carcinomas are also seen
Early tumors present as superficial ulcerations or masses of the membranous septum or columella with crusting, scabbing, and occasional minor bleeding. More advanced lesions can perforate the septum or invade the alar cartilage and grow through the skin surface. There can also be extention inferiorly into the gingivo-labial sulcus and premaxillary soft tissues. Growth into the posterior nasal cavity occurs late or with recurrences.
The nasal vestibule drains primarily to the submandibular lymph nodes. The facial lymph nodes are second-echelon nodes, which may become involved with recurrent disease. Only about 5 to 10 percent of patients present with lymph node metastasis
History and physical exam, computed tomography (CT) scan, and biopsy for pathologic confirmation are usually sufficient for evaluation
Treatment — The goal of therapy is to achieve the best oncologic outcome with the least morbidity in terms of cosmesis and nasal function. For small T1 lesions, either surgical excision or definitive radiation therapy (RT) can be used. Large T1, T2, T3, and small T4 tumors are best treated with definitive RT, as surgical excision of these lesions usually produces significant cosmetic deformity. Extensive T4 tumors, however, require a combined modality approach consisting of both surgery and radiation therapy.
Because of the rare occurrence of nasal vestibule tumors, there are no prospective data to guide treatment recommendations; the majority of the literature is single-institution case series.
Early stage — T1 and T2 tumors are treated with definitive RT, with a five-year local control rate of 95 percent for T1 and T2 tumors and for all tumor stages a five-year cause-specific survival and overall survival of 90 and 76 percent, respectively. Surgery is a reasonable alternative if the lesion can be removed with a good functional and cosmetic result.
Definitive RT usually involves external beam RT (EBRT) followed by a boost, delivered via EBRT or interstitial brachytherapy one to two weeks after completing EBRT. EBRT can be delivered either with opposed lateral fields using megavoltage photons or a single anterior portal using a 4:1 mix of high-energy electrons and megavoltage photons. For both techniques, a tissue-equivalent wax bolus is placed over the entire nose to convert the irregular nasal surface to a box-like contour to improve the dose distribution. The dose delivered by the initial EBRT fields is usually 50 gray (Gy) given in once-daily 2 Gy fractions. This is followed by a boost to the tumor of 20 to 25 Gy. Serious complications with definitive RT are uncommon.
Although interstitial implants of the nasal vestibule are individualized, the implant generally consists of two to four planes of needles inserted through the dorsum of the nose. The source is iridium-192. When given as a boost after EBRT, the dose delivered by the interstitial implant is usually 25 Gy. Very early T1 tumors can be treated with interstitial brachytherapy alone, usually 55 to 75 Gy, depending on the tumor size
Surgical resection of early stage lesions in the nasal vestibule results in local control rates of 90 to 100 percent. Surgery can be a simple wide local excision with or without skin grafting, but can also involve the removal of cartilage (usually either columella or alar cartilage) and/or external nasal skin.
It is important that the surgeon assess the likelihood of achieving a complete resection; a lesion that is likely to result in close or positive margins with surgery would be better treated with definitive RT. The surgeon should also critically assess the tumor extent to understand which structures will need to be resected before embarking on the procedure. Nasal floor or lateral nasal wall tumors can be easily removed if there is not deep invasion into the nasal cartilages. Septal lesions can be removed with excellent cosmesis as long as the anterior septal support is not compromised. If needed, the external nose can be reconstructed but often requires multiple procedures by a nasal reconstructive surgeon and may be disfiguring.
Advanced stage — T3 and small T4 tumors with no bone invasion are usually treated with RT alone, with surgery reserved for radiation failure. RT is delivered as described above, with EBRT to about 50 Gy followed by an interstitial brachytherapy or EBRT boost for an additional 25 Gy.
Extensive T4 tumors should be treated with a combination of surgery and RT, as these tumors have a low rate of local control with either modality alone . In this case, when the treatment consists of both surgery and RT, RT can be delivered either pre- or postoperatively. Preoperative RT offers the possibility of shrinking the tumor to allow for a less extensive resection; postoperative RT results in less wound complications. The EBRT dose is usually 50 to 60 Gy delivered in 2 Gy fractions.
Resection of advanced stage lesions may require total rhinectomy. A prosthesis is usually created to cover the defect, as total nasal reconstruction is cosmetically unacceptable.
Little has been published about the use of chemotherapy in the treatment of nasal vestibule cancers.
Management of the neck — Since the risk of cervical metastasis is low, elective nodal treatment (either radiation or neck dissection) should be considered only for patients with T4 cancers, particularly those that are poorly differentiated and/or recurrent. Patients who present with lymph node-positive disease should be treated with radiation to the involved neck and/or neck dissection.
Nodal recurrence rates of 0 to 13 percent have been reported, with the majority of nodal failures occurring in patients with stage T4 and/or recurrent tumors Furthermore, salvage treatment of nodal disease is often successful. As an example, 75 patients with NO disease treated without elective neck irradiation achieved an initial nodal control rate of 87 percent and a five-year nodal control rate of 97 percent after salvage attempts
Prognosis — Five-year local control is 80 to 95 percent for early-stage lesions and 70 percent for advanced lesions. Reported five-year overall survival ranges from 58 to 76 percent. Regional and distant metastases are extremely rare. Factors associated with a worse prognosis include higher T stage, nodal involvement, and involvement of the lip, cartilage, or bone
NASAL CAVITY — Tumors arising in the nasal cavity, posterior to the limen nasi, have a natural history similar to tumors of the paranasal sinuses and are treated similarly.
Overview — Nasal cavity tumors are twice as common in men and usually occur between the ages of 55 and 65 years. Tobacco smoke has been associated with the development nasal cavity tumors Adenocarcinomas of the nasal cavity have been linked to multiple occupational exposures such as wood dust, formaldehyde, leather dust, and textile dust
The nasal cavity begins at the limen nasi and ends posteriorly at the choanae, which communicate directly with the nasopharynx. It is bounded inferiorly by the hard palate and superiorly by the base of skull. It is divided into right and left halves by the nasal septum. The lateral wall of each nasal cavity is composed of thin bony folds that project into the nasal cavity, called the inferior, middle, and superior nasal turbinates (concha). The nasal cavity communicates with the paranasal sinuses and other structures via multiple passages or meatuses
Histology — Cancers with a wide variety of histologies arise in the nasal cavity; these are similar to those that occur in the paranasal sinuses.
Patients with tumors of the nasal cavity usually present with a chronic history of sinus congestion and recurrent nasal obstruction. Many patients complain of nasal discharge and intermittent epistaxis. These symptoms can mimic those of chronic sinusitis or nasal polyps and may delay the diagnosis of cancer. However, since chronic sinusitis is most often associated with bilateral symptoms, unilateral symptoms should be used as an indication for further evaluation, such as nasal endoscopy and/or imaging.
The symptoms from more advanced lesions depend on the site of tumor spread: widening of the bridge of the nose from lesions invading the olfactory region, frontal headaches from extension through the cribriform plate or into the ethmoid sinus, and proptosis, diplopia, or a palpable orbital mass from invasion of the medial orbit.
The nasal cavity drains to the retropharyngeal, submandibular, junctional, and subdigastric nodes. Less than 10 percent of patients present with lymph node metastasis
A detailed history and physical examination should be performed with attention to signs and symptoms of orbital or cranial nerve involvement. Biopsy can usually be performed via nasal endoscopy. Both computed tomography (CT) with contrast and magnetic resonance imaging (MRI) are usually performed, CT for determining bone, nasopharyngeal, and lymph node invasion and MRI for evaluating intracranial, orbital, and perineural spread. MRI also helps determine whether the sinuses are opacified due to tumor, fluid, or secondary inflammatory mucosal changes
Treatment — Both early and moderately advanced tumors of the nasal cavity are treated with a combination of surgical resection and postoperative radiation. Very advanced (unresectable tumors) are treated with RT with or without concurrent chemotherapy.
No randomized studies have evaluated treatment options for patients with carcinoma of the nasal cavity. Most retrospective studies combine nasal cavity tumors with paranasal sinus tumors, making it difficult to make conclusions that are specific to nasal cavity cancers.
Early stage — For T1N0 tumors, surgery alone is a reasonable treatment option, resulting in an overall survival of 50 to 70 percent in selected patients However, most patients with early stage tumors are treated with surgery and postoperative RT, details of which are discussed below
Advanced stage — The standard treatment for advanced stage tumors is surgery followed by postoperative RT. Although preoperative RT has been used to minimize tumor bulk and limit the extent of resection, we favor postoperative RT to minimize the risk of wound complications. Very advanced (unresectable) tumors are treated with definitive RT. Based upon experience with analogous tumors of the sinus and other mucosal sites, chemotherapy, administered as induction or concurrently with RT, is used by some for unresectable and more extensive lesions, but there is little supporting data.
Local control following surgery alone is 50 to 60 percent at five years. The addition of postoperative radiotherapy improves five-year local control to 55 to 80 percent
The most common surgical techniques used for exposure of the nasal cavity are the lateral rhinotomy and the midface degloving procedures. The goal of both surgical techniques is to provide an en bloc resection of all involved bone and soft tissue, while preserving function and cosmesis. If the superior extent of the tumor involves the fovea ethmoidalis or cribriform plate, then a craniofacial resection is required
Endoscopic resection is gaining acceptance as an alternative to transfacial or craniofacial surgeries
There is no consensus on criteria used to define tumors as unresectable. Most agree that tumors that invade the cavernous sinus or optic chiasm or extend transdurally are not resectable without causing unacceptable morbidity and loss of function
RT is initiated four to six weeks after surgery to allow for healing. A dose of 60 Gy delivered in 2 Gy fractions daily is sufficient when negative margins are achieved. If surgery resulted in positive margins or gross residual disease, or if the tumor was removed piecemeal (ie, endoscopically), the dose used is 66 to 70 Gy in 2 Gy daily fractions. Hyperfractionated irradiation may decrease the risk of optic neuropathy if any part of the optic apparatus is likely to receive doses in excess of the tolerance dose
RT for nasal cavity cancer is almost always delivered using intensity-modulated radiation therapy (IMRT) rather than conventional EBRT. IMRT allows for improved target coverage while better sparing critical structures such as the optic apparatus. Included in the target volume are the tumor bed, any residual disease, and areas with a high likelihood of harboring microscopic disease. Preoperative diagnostic CT, MRI and/or positron emission topography (PET) scans are used when delineating the target volume. Low rates of late complications, including blindness, have been reported with IMRT
Very advanced or unresectable tumors are treated with high doses of RT, usually 70 Gy in 2 Gy daily fractions or 74.4 Gy in 1.2 Gy fractions delivered twice daily with a six-hour split. Outcomes in these patients are poor, with five-year overall survival of 15 percent reported Induction chemotherapy and/or concurrent chemotherapy is being adapted for use in nasal cavity and paranasal sinus cancers, despite scant evidence of chemotherapy efficacy in nasal cavity cancer
Management of the neck — Elective treatment of the N0 neck is controversial. Reported rates of nodal recurrence when the neck is not electively treated vary from 0 to 19 percent Some believe that there is no benefit to electively treating the N0 neck . Others recommend elective nodal treatment, either neck dissection or radiation, for patients with advanced-stage disease and/or poor prognostic factors. We suggest elective irradiation of the N0 neck in patients with T3 and T4 disease, tumor extension to areas rich in lymphatics (ie, the nasopharynx), and all esthesioneuroblastoma and sinonasal undifferentiated carcinomas (SNUC).
Patients who present with lymph node-positive disease should be treated with neck dissection and postoperative radiation.
Prognosis — Local failure is problematic in the treatment of nasal cavity tumors and adversely impacts survival; regional and distant metastases are rare. When treated with surgery and adjuvant RT, the five-year local control and overall survival is 50 to 80 percent and 50 to 70 percent, respectively. Factors associated with a worse prognosis include higher T stage, treatment with RT alone, lymph node involvement, SCCA and SNUC histologies, and invasion of the orbit, cribriform plate, or dura
POSTTREATMENT SURVEILLANCE — Regular posttreatment follow-up has become an essential part of the care of patients after potentially curative treatment of head and neck cancer (HNC). It is particularly important for nasal vestibule cancers because recurrences can often be salvaged successfully. Patients should be educated about possible signs and symptoms of tumor recurrence, including hoarseness, pain, dysphagia, bleeding, and enlarged lymph nodes.
In general, the intensity of follow-up is greatest in the first two to four years, since approximately 80 to 90 percent of all recurrences after curative intent treatment will occur within this timeframe. Continued follow-up beyond five years is generally suggested since the risk of recurrence remains elevated beyond the first five years, especially for cancers of the hypopharynx, larynx, nasopharynx, and salivary glands and for second primary malignancies. Because of the higher risk of recurrence and second primary malignancy in those who continue tobacco use, many schedule more frequent surveillance visits for these patients and continue for longer duration (ie, beyond five years).
Posttreatment surveillance of head and neck cancer, including screening for treatment-related complications, is reviewed separately.
SUMMARY AND RECOMMENDATIONS
Nasal vestibule — Carcinomas of the nasal vestibule are essentially skin cancers and have a good prognosis.
Nasal cavity — Tumors arising in the nasal cavity have a natural history similar to tumors of the paranasal sinuses. A wide variety of tumor histologies occur in the nasal cavity, with squamous cell carcinoma and adenocarcinoma predominating.