Keywords

1 Introduction

All of the nasal cavity and paranasal sinus tumors constitute approximately 3% of upper respiratory tract tumors and 1% of all tumors [1]. Tumors originating from the nasal cavity are common among sinonasal tumors [2, 3]. In advanced sinonasal tumors, it may be difficult to make a decision on where the tumor has originated from and for this reason, nasal cavity tumors are frequently grouped together with paranasal tumors. The close proximity of nasal cavity tumors to vital structures necessitates the treatment strategies to be accurately determined. Due to their rarity, and their vague symptoms that might have been confused with other rhinitis, making a diagnosis might not be possible. However, the increasing use of endoscopes for diagnostic purposes has enabled diagnosis of nasal cavity tumors at earlier stages, today. In this chapter, tumors originating from the nasal cavity will be discussed in all aspects.

2 Epidemiology

According to a study that reviewed the sinonasal tumor database between years of 1973 and 2006, tumors of nasal cavity origin constituted the largest share of sinonasal tumors, with a ratio of 43.9% [4]. In this study, female/male ratio was 1/1.8 and majority of the patients were diagnosed at ages of sixth decade or older. In another study, it was stated that 55% of sinonasal tumors originated from maxillary sinuses, 35% from nasal cavity, 9% from ethmoidal sinuses, and the remaining from frontal and sphenoid sinuses [5].

3 Risk Factors

Nickel refinery workers, wood and furniture workers, leather shoe workers, workers who have close contact with formaldehyde, and cigarette smokers are considered as high-risk groups [6,7,8].

In the study conducted on workers who had worked for at least 5 years in nickel refinery, it was found that the risk for development of nasal cancer had persisted for 15–42 years following exposure to nickel [9]. Nickel causes breakage in DNA, changes the DNA proteins, and inhibits the repair of DNA [10]. Nickel complexes lead to release of reactive oxygen radicals. Additionally, formaldehyde was found to have mutagenic effects in in vitro studies and cytogenetic effects in conducted in vivo studies [11].

In a study conducted on workers who were exposed to wood dust, an increase of micronucleus frequency was determined in nasal cells, together with chromosomal instability, which indicated increased risk in terms of nasal carcinomas [12].

Cigarette smoking was determined to increase the recurrence risk of inverted papilloma, following surgery [13].

It was suggested that HPV is important and adversely affects the expected survival rates in nasal cavity tumors such as nasal squamous cell carcinoma and inverted papilloma [14,15,16].

4 Anatomy

Knowing the anatomy of the nasal cavity well is important for staging the disease and planning the surgery. Nasal cavity is anatomically in close relation with oral cavity, paranasal sinuses, orbita, nasopharynx, pterygomaxillary fissure, pterygopalatine fossa, infratemporal fossa, skull base, and intracranial fossa.

Nasal cavity is the part located between nasal septum and lateral nasal wall, of the region between nostrils, named nares, anteriorly, and choana posteriorly. It is bounded by hard palate inferiorly, and skull base superiorly (frontal bone, cribriform plate of ethmoid bone, sphenoid bone). The lateral nasal wall, formed by frontal process of maxilla, ethmoid bone, lacrimal bone, and palatine bone, contains superior, middle, and inferior concha, together with meatuses. Nasolacrimal duct opens to inferior meatus, anterior sinuses (frontal, maxillary, anterior ethmoid) open to middle meatus, and posterior sinuses (posterior ethmoid, sphenoid) open to superior meatus.

5 Histology

Histology of the nasal cavity consists of neuroepithelium in olfactory mucosa, stratified squamous epithelium in respiratory mucosa, and keratinized squamous epithelium in nasal vestibule. Except the vestibule, entire nasal cavity and paranasal sinuses are lined by mucosa, and covered by mucus. While the mucosal layer creates a protective physical and immunological barrier, it plays a great role in heating the entering air. The most important layer forming the mucosa is pseudostratified ciliated columnar epithelium. The vestibule is the first 1–2 cm part of nasal cavity. It is covered by keratinized stratified squamous epithelium. It includes hair follicles, together with sweat and sebaceous glands. At the region between vestibule and the anterior edge of middle concha, stratified squamous epithelium transforms to transitional epithelium.

There is olfactory epithelium at the upper-rear part of each nasal cavity, localized in a 1-cm area, at the upper side of superior concha and roof of the septum. It contains non-ciliated, pseudostratified, columnar epithelium, bipolar olfactory nerve cells, and supporting cells. The olfactory epithelium settles on the lamina propria; it does not have a submucosa. Respiratory mucosa is lined with pseudostratified ciliated columnar epithelium. There are four types of cells: ciliated cubic cells, nonciliated cubic cells, goblet cells, and basal cells. Goblet cells within the epithelium and submucosal seromucous glands produce the mucus layer, covering the epithelium. Nasal mucus layer is 10–15 μm thick and it is continuously moved by cilia, with a speed of 6 mm/min.

5.1 Nasal Cavity Tumors

While the most common benign tumor is inverted papilloma, the most common malign histological type is squamous cell cancer [3, 17]. Malign melanoma, adenoid cystic carcinoma, and adenocarcinoma are the following tumors [3, 5].

5.1.1 The Benign Nonepithelial Mesenchymal Tumors of Nasal Cavity

Fibro-osseous tumors (osteoma, chondroma, ossifying fibroma, fibrous dysplasia): Osteomas are slowly growing benign masses. While they are commonly seen in frontal sinus, they are rarely manifested in the nasal cavity [18]. Chondromas are also very rarely reported in the nasal cavity [19, 20].

Ossifying fibromas and fibrous dysplasia are also rare tumors of nasal cavity and have some discriminative properties. Ossifying fibromas are masses, seen in the first and second decades of age [21], containing lamellar bone trabecula, which is separated by sharp boundaries, together with vascularized fibrous stroma. Fibrous dysplasias are enlargements of bone, having indistinct boundaries, and they are surrounded by a thin bony cortex [21]. While ossifying fibromas are observed as lesions surrounded by an expansile and thick bone density in computerized tomography, the boundaries in fibrous dysplasia are not distinct. Ossifying fibromas have an aggressive growth pattern. Thus, if they are not completely excised, recurrence may occur.

Neural tumors (schwannoma, neurofibroma, meningioma): Their detection rates in the nasal cavity are quite low. The majority of them have a benign character [22]. Schwannomas originate from the sheaths of Schwann cells. Since olfactory and ophthalmic nerves do not contain these cells, Schwannomas do not originate from these nerves [23]. It is suggested to originate from the cells located on the branches of trigeminal nerve in the nasal cavity. Its most common symptoms are nasal obstruction and epistaxis. Although schwannomas are benign, bone damage due to pressure effect may be detected in tomography (Fig. 58.1a, b). This is not an indicator for the presence of malignancy.

Fig. 58.1
figure 1

(a) Six-year-old girl’s left nasal cavity schwannoma. (b) The same case’s CT

Vascular tumors (hemangioma, juvenile nasopharyngeal angiofibroma, pyogenic granuloma): Lobular capillary hemangioma is also called pyogenic granuloma. It is frequently seen in pregnant women and is generally located at the anterior part of nasal septum [24]. Trauma and hormonal effects have been suggested as etiological factors. Systemic corticosteroids have been beneficial in their treatments [25]. Following corticosteroid treatment, the confined tumor can be surgically excised. Angiofibromas may rarely originate from nasal cavity [26]. By angiography, the vessels, feeding the tumor, may be detected and embolized preoperatively and blood loss may be minimized during the operation. Although it is histologically benign, due to its aggressive clinical behavior, expansive growth, and local extension, it is considered as clinically malignant (Fig. 58.2).

Fig. 58.2
figure 2

CT from the pyogenic granuloma of the right nasal cavity

5.1.2 Benign Epithelial Tumors of the Nasal Cavity

5.1.2.1 Nasal Cavity Papillomas

Nasal cavity papillomas are classified as inverted, fungiform, and cylindrical. While fungiform type originates from nasal septum, inverted and cylindrical types may originate from lateral nasal wall.

5.1.2.2 Inverted Papilloma

It is most commonly seen in ages of 50–60 years and predominantly in males. Unilateral nasal obstruction is the most frequent reason for presentation of the patient to the physician. It originates most commonly from the lateral nasal wall (89%). Nasal septum (9.9%), frontal sinus (6.5%), and sphenoid sinus (3.9%) are the other sites, which the tumor may originate from [27]. CT and MRI are extremely valuable in terms of diagnosis. The diagnosis may be confirmed by biopsy following radiological investigations. With CT, the site from where the tumor has originated can be shown, together with the sites of bone erosion and destruction [28] (Fig. 58.3a–c). It was reported that a solitary cone-shaped hyperostotic area in computerized tomography shows the site of origin for inverted papilloma and it is very important for planning surgical management [28] (Fig. 58.4). MRI shows the boundaries better, by discriminating the tumor tissue from the areas of inflammation. The convoluted cerebriform pattern was reported to be important for differential diagnosis of inverted papilloma from other malign tumors [29] (Fig. 58.5a, b)

Fig. 58.3
figure 3

Inverted papilloma’s hyperostosis origin: (arrow) (a) at the inferomedial orbita; (b) at the posterior nasal septum; (c) from frontal sinus to nasal cavity

Fig. 58.4
figure 4

Inverted papilloma excised with the hyperostosis bone (arrow) origin

Fig. 58.5
figure 5

(a) Coronal CT. Hyperostosis (arrow) at the left maxillary sinus lateral wall. (b) Same patient’s cerebriform pattern on MRI T2-weighted imaging

With routine histopathological examination of all nasal polypectomy specimens, inverted papillomas can be diagnosed earlier, in terms of both local invasive potential and malignancy risk. Its incidence to be seen bilaterally is reported between 2% and 7% [30]. Due to being multicentric and risk for leaving residual tumor following surgery being higher, they have a higher rate of recurrence [31]. The incidence to be associated with malignancy or development of a secondary malignancy was determined as 5–20% [32, 33]. Malign transformation generally occurs as squamous cell carcinoma [32]. However, even during the advanced stages of the disease, lymph node metastasis is rare [33]. These can be multicentric with a rate of 12–30% [34]. They should be excised completely; otherwise, recurrence is frequent. The rate of local recurrence was shown to be between null and 24% [35]. Nowadays, it is possible to excise the tumor by endoscopic approach in many patients. In endoscopic surgery, wide resection of the tumor origin throughout the subperiosteal plane, together with drilling the bone underneath is important for successful results. It has been stated that recurrence may occur if this procedure is not performed [36]. Following surgery, close endoscopic follow-up is important. Addition of radiochemotherapy to the treatment was determined to extend the survival period [32, 37]. In squamous cell carcinoma, which is formed by malign transformation, the survival rate is 72.5% and this rate is higher, when compared to primary squamous cell carcinoma [32].

Hamartomatous tumors (respiratory epithelial adenomatoid hamartoma): Although they are rarely seen in nasal cavity, when they are localized in this region, they are most frequently seen in posterior nasal septum and unilaterally [38]. Their etiology is not certain and has been considered to be correlated with smoking, asthma, nasal polyposis, chronic sinusitis, and inflammation [39]. Widening of the olfactory cleft and not destructing the bone are valuable assets in terms of differential diagnosis [40] (Fig. 58.6). Because they are self-limiting disorders, meticulous surgical excision is sufficient for their treatment.

Fig. 58.6
figure 6

CT from the respiratory epithelial adenomatoid hamartoma of the nasal cavity

5.1.3 Malign Epithelial Tumors of the Nasal Cavity

Squamous cell carcinoma: It is the most common malign epithelial tumor of the sinonasal tract. Since the risk for extending to facial soft tissues and then to the regional neck will increase if it stems from nasal vestibule or anterior nasal septum, the prognosis is worse. The 3-year and 5-year survival rates of squamous cell cancers of the nasal cavity were determined as 86% and 69%, respectively [41].

Adenoid cystic carcinoma: While it is most frequently seen in maxillary sinus within the sinonasal tract, it is the second most frequently localized in the nasal cavity [42, 43]. The lymphatic regional extension is rare. Lung metastasis is frequent [43]. It has cribriform, tubular, and solid subtypes. Solid type has the worst prognosis [44]. It can involve maxillary, mandibular, and vidian nerves, by extending early to the neurovascular structures. It can advance to the intracranial foramina. Its 5-year survival rate is 50–86% [42, 45]. Although there are studies showing that perineural invasion does not affect prognosis too much [46], there are also studies showing that it worsens the prognosis [47]. In a meta-analysis related to adenoid cystic carcinoma, no differences were found among the only surgery group, group with radiotherapy (RT) following surgery, and only chemoradiotherapy group, in terms of 5-year survival rate [43].

Adenocarcinoma: It is frequently originated from ethmoidal sinuses and nasal cavity. The prognosis varies according to the histological differentiation. While the prognosis is poor in sessile and alveolar mucoid types, papillary type has a better prognosis. It is treated by surgical excision.

Olfactoryneuroblastoma (Esthesioneuroblastoma): It constitutes 7–10% of sinonasal tumors. It is stained with neuroendocrine markers. Resection of the anterior skull base is suggested.

Mucosal melanoma: In most of the cases, it originates from the septum and lateral nasal wall. Due to its aggressive behavior and delay in diagnosis, its prognosis is poor. Among mucosal melanomas of the head and neck, nasal cavity is seen with such a high rate as 50% [48]. The prognosis of mucosal melanoma is worse than cutaneous melanoma. Caution should be exercised in terms of cranial nerve involvement and cervical lymph node metastases during the diagnosis. While it is observed with an aggressive osteolytic structure in tomography, it is characterized with heterogeneous contrast enhancement in magnetic resonance imaging [48]. It should be excised, including a surgical margin of 1.5–2 cm [49]. Although its radiosensitivity is quite poor, RT is recommended following surgery [50].

5.1.4 Malign Nonepithelial Tumors of the Nasal Cavity

Neurogenic sarcoma: It is rare and generally associated with neurofibromatosis. Its prognosis is poor.

Rhabdomyosarcoma: The rhabdomyosarcomas originating from sinonasal tract have an aggressive clinical course; systemic dissemination and regional extension is frequent. RT is required in addition to surgery.

Fibrosarcoma: Although it is rare in head and neck region, it is the second most frequently seen in nasal cavity following maxillary sinus, when sinonasal region is taken into consideration [51]. Surgery alone, or associated with RT, has more successful results when compared to RT alone [51]. The definitive diagnosis can be made histopathologically.

Chondrosarcoma: It is the most common soft tissue tumor in children under 15 years of age. Only 10% are located at the head and neck region [52]. Since it is a low-grade tumor, surgery is sufficient for treatment [53]. Because it is relatively resistant to chemotherapy and RT, these treatments can only be preferred in cases with insufficient surgery [54].

Hemangiopericytoma: It is rarely met in nasal cavity. It is seen as a mass having uniformly high density and significant contrast enhancement in computerized tomography [55] (Fig. 58.7). Its prognosis is closely associated with the histological grade. It has histopathological characteristics similar to glomus tumor [56].

Fig. 58.7
figure 7

CT from the hemangiopericytoma of the left nasal cavity

Lymphoma: It has homogeneous density, together with soft tissue edema and less bone destruction in computerized tomography. Malign lymphomas of the nasal cavity are usually NK/T-cell lymphomas, whereas B-cell lymphoma is seen in paranasal sinuses [57].

Extramedullary plasmacytoma: It occurs with monoclonal proliferation of plasma cells [58]. Its treatment involves surgery and RT. It is a quite radiosensitive tumor [58]. It may show progression to multiple myeloma and in this situation, its prognosis is worse [59].

Metastatic tumors: The frequently determined primary sites are kidneys, breasts, and lungs.

6 Clinical Presentation

The most commonly seen clinical presentations of nasal cavity tumors are nasal obstruction (71%) and epistaxis (42%) [6]. However, since the symptoms of nasal cavity cancers are similar to common disorders such as sinusitis, delay in diagnosis may occur. In advanced disease, symptoms according to the involved regions may be added to the clinical features. Due to symptoms such as nasal obstruction and epistaxis, nasal cavity tumors can be diagnosed earlier, when compared to paranasal sinus tumors. Nine to twelve percent of the patients may be asymptomatic [60]. Particularly, patients with unilateral sinonasal symptoms should be carefully evaluated. Unilateral facial swelling, unilateral proptosis, diplopia, and cranial neuropathies should serve as alerters. Regional and distant metastases are seen quite rarely even in advanced stages of the disease [61]. Examination of the cranial nerves and oral cavity, together with nasal endoscopy, should be performed meticulously. When the infraorbital V2 branch of the maxillary nerve is involved, numbness or hyperesthesia may occur. In orbital involvement, ocular pain, fullness in the eye, unilateral epiphora, diplopia (related to involvement of periorbital mass), and proptosis may be seen. The tumor may show advancement to anterior cranial fossa, through the cribriform plate. When the tumor advances to nasopharynx, clinical features of serous otitis media may emerge. When it advances toward the palate, palatal fullness, ulceration, and tenderness may occur. When the tumor advances to pterygopalatine and/or infratemporal fossa, it may lead to trismus, facial tenderness, and severe epistaxis.

7 Radiological Evaluation and Diagnostic Procedures

Computerized tomography (CT) and magnetic resonance imaging (MRI) offer enormous amount of useful information. CT is valuable for evaluation of bony structures and skull base. Bone erosions, destructions, and sclerotic areas can be detected. In nasal polyposis, CT reveals widening of the infundibulum, opacification of sinuses, together with thinning of the sinus walls and nasal ethmoidal septa. In nasal cavity tumors, the most common finding in CT is bone destruction. While bone involvement is seen as erosion, infiltration, and destruction in malign tumors, it is seen as expansion in benign tumors.

In juvenile angiofibroma, CT reveals deformity at the base of ipsilateral medial pterygoid lamina, together with forward bowing (Holman-Miller sign) at the posterior wall of maxillary sinus [62].

When CT findings and histopathological data are compared, the rate of false-positive findings has been found to be high [63]. Faulty evaluations about involvements of ethmoid, orbita, soft palate, and nasopharynx have led to misinterpretations of the tumor stage [63].

With MRI, the details of soft tissues are better evaluated. It is important for the assessment of perineural spread. The involvements of dura, orbita, and brain parenchyma can be detected. With MRI’s diffusion-limitation property, the benign-malign discrimination of tumors can also be predicted (Fig. 58.8a–c). Additionally, with MRI, by differentiating tumor tissue from inflammatory areas, the borders of the mass can be better identified [63]. The high water content of the inflammation manifests increased signal in T2 images. This is an important superiority of MRI, when compared to CT. Moreover, MRI is superior to CT in terms of showing the site of tumor origin [63]. In addition, with MRI, the hemorrhagic areas inside the mass can be discriminated from the mass itself. The evaluation of tumor blood flow by noninvasive perfusion in MR imaging was suggested to be useful in differential diagnosis of squamous cell carcinoma and inverted papilloma [64].

Fig. 58.8
figure 8

Squamous cell carcinoma of the nasal cavity MRI: (a) T1 weighted; (b) short inversion time inversion recovery (STIR) image; (c) T1-weighted postcontrast

Especially in tumors of the face having soft tissue involvement, sarcomas with high risk of hematogenous dissemination, and malign melanomas, scanning for distant metastases by positron emission tomography (PET/CT) should be performed.

Biopsy, performed following radiological investigations, will shed light on the pathological diagnosis. However, due to other pathologies such as edema in the nasal cavity, polyp, or inflammation, accompanying tumors, nasal biopsies obtained from wrong sites at the preoperative period can be misleading. In cases of clinical suspicion, recurrent biopsies might be needed.

8 Staging

T Staging

T1

Tumor is confined to only one substructure, +/− bone invasion.

T2

Confined to two substructures at the same region or extending to nasoethmoid complex, +/− bone invasion.

T3

Tumor with involvement of medial wall or base of orbita, maxillary sinus, palate, cribriform plate.

T4a

Tumor with involvement of anterior orbital structures, nasal skin or cheek, minimal in anterior cranial fossa, pterygoid plate, sphenoidal or frontal sinus.

T4b

Tumor with involvement of orbital apex, dura, brain, middle cranial fossa, cranial nerves except V2, nasopharynx, or clivus.

AJCC Cancer Staging Manual, Seventh Edition (2010) Springer New York, Inc.

In TNM classification made by using CT and MR imaging techniques, 50% of sinonasal cavity tumors were determined to be at advanced stage (stage IV) [3].

9 Treatment

Since many retrospective studies have covered the treatment results of all tumors belonging to the nasal cavity and paranasal sinuses, it is difficult to evaluate the results of the nasal cavity distinctively. Most of the nasal cavity tumors are treated by surgery and RT, with a multidisciplinary approach [65, 66]. While treatment by surgery alone is possible in carefully chosen T1N0 patients, RT is recommended following surgery in other patients. Among patients having advanced staged nasal cavity tumors, the survival rate of patients who had been treated by RT in addition to surgery was determined to be better than those who had received RT only [67]. Therefore, in advanced staged and squamous cell nasal cavity tumors, in addition to surgery, RT following surgery is suggested [67, 68]. Treatment with RT alone is recommended in early stage, poorly differentiated tumors, and adenocarcinomas [67, 68]. By reducing the tumor volume in unresectable tumors, induction chemotherapy can lessen the damage of RT to the eye and brain.

9.1 Surgery

Besides the main purpose being total excision of the tumor, preservation of as much as possible functionality, together with a good cosmetic result should also be aimed. Due to anatomical limitations, it is difficult to achieve wide negative margins. The most preferred surgical techniques for a good exposure of nasal cavity are lateral rhinotomy and midface degloving. Modifications have been made in midface degloving techniques, in terms of making no incision on the face [69]. If fovea ethmoidalis, cribriform plate, and intracranial structures have been involved by the tumor, craniofacial resection may be performed. If periorbital structures are invaded by the tumor in advanced staged tumors, orbital exenteration, partial or total maxillectomy may be required. Following surgery, the reconstruction of the defects can be made by locoregional flaps in several stages, or by osteocutaneous forearm free flaps in a single stage [70]. Moreover, free jejunal flap with non-vascularized bone was also reported for repair of the defect [71]. Today, with improvements in surgical techniques, endonasal endoscopic approaches can be used alone or combined with craniotomy. In suitable patients with sinonasal tumors, oncological outcomes of endoscopic approaches without addition of craniotomy have been found to be similar to those in which craniotomy was added [72]. During surgery, histopathological examination by frozen section technique may be carried out, in order to achieve clean margins. In nasal cavity tumors, as a surgical principle different from other tumors, debulking can initially be performed and surgery can be continued thereafter. The most important reason for treatment failures is not achieving the control of the primary area, and therefore providing the local control has utmost importance [73]. In a study comparing the therapeutic benefits of endoscopic and open surgical approaches in sinonasal malign melanoma, the disease-free survival periods were found to be similar [74]. In endoscopic approaches, the skull base defects occurring after surgery can be repaired by nasoseptal flap [75].

9.2 Radiotherapy

RT can be administered alone in medically inoperable patients or patients with unresectable tumors. RT is given following surgery. If the tumor is T1N0, histological grade is low, and perineural invasion and close or positive surgical margins are not present, RT may not be given. With new RT techniques, the damage to the normal tissues has lessened to a minimum. However, due to the close anatomical relationships, serious damage can occur in lacrimal apparatus, cornea, retina, optic nerve, and optic chiasma following RT [76]. In histological types such as esthesioneuroblastoma, RT improves survival significantly [77]. Stereotactic RT with CyberKnife® is used successfully and damage to the surrounding organs is significantly reduced in malign melanomas of the nasal cavity [78].

9.3 Chemotherapy

In sinonasal tumors, chemotherapy is given when the disease is locally advanced or as a palliative treatment, when surgery and RT are contraindicated in metastatic patients [79]. Chemotherapy and radiotherapy may be simultaneously administered when gross residual tumor is present during RT, if the tumor is unresectable or its histological grade is high, when surgical margin is positive for tumor cells, there is extracapsular nodal spread or if there are multiple positive lymph nodes. Neoadjuvant chemotherapy may be used in tumors with very advanced stage, prior to surgery or RT, in order to shrink the tumor [80]. In inoperable patients with advanced staged tumors, another alternative treatment is administration of the chemotherapeutic agents intraarterially, via internal maxillary artery, facial artery, and transverse facial artery [66].

9.4 Approach to the Neck

Neck dissection is performed if positive lymph nodes are present during physical examination or imaging. Elective neck dissection is not advised in the early period; however, it is recommended in patients with T3–T4 N0M0 squamous cell or other high-grade tumors [81].

9.5 Patient Follow-Up

Follow-up examination is recommended every 3 months for 2 years, since 80–90% of recurrences would occur during this period. CT or MRI is performed following treatment, for using as reference points during the next follow-up examination. In every follow-up examination, physical examinations of head and neck are performed, together with detailed endoscopic evaluation.

9.6 Prognosis

In nasal cavity tumors, the most important prognostic indicator is locoregional control. Although distant metastasis is not frequent, it may be seen in patients having adenoid cystic carcinoma, undifferentiated histological findings, or in whom lymph node positivity is present. In a study on 60 patients with nasal cavity carcinoma, it was found that, while histological type and clinical stage were correlated with prognosis, cervical metastasis and treatment type were not significantly effective in determining the prognosis [82]. The 5-year survival rate was determined as 56.7% in 783 patients with nasal cavity cancer [17]. Moreover, in this patient group, increased age, male gender, advanced T stage, advanced N stage, and increasing tumor grade, together with the histologic type being malign melanoma, were determined to reduce the survival rate [17]. In another study conducted with 32 patients having nasal cavity cancer, 5-year survival rate was reported as 50% [83]. While surgical margin is an important prognostic indicator in head and neck cancers, it cannot be widely used in nasal cavity, due to anatomical difficulties encountered during working on bone margins.

10 Tumors of the Nasal Vestibule

Nasal vestibule was added to this section, because it forms the entrance site of the nasal cavity. It is the region limited with septum and columella medially, inferior lateral cartilage laterally, and underlying premaxilla, below. It ends at the junction of superior and inferior lateral cartilages, the transition site from skin to mucosa. For this reason, it can be incorporated into the tumors of the nasal skin. Vestibule involves cutaneous hair follicles and sebaceous glands. Cancers of this region are generally seen between 60 and 70 years of age [84]. Smoking is an important risk factor. Squamous cell carcinoma is frequent (Fig. 58.9a, b). They are generally self-limiting tumors and have better prognosis than nasal cavity cancers. They usually drain into the submandibular lymph nodes. The American Joint Committee on Cancer 2010 TNM Classification included these tumors into the same group with nasal cavity tumors; however, in Wang’s classification, they were divided into three groups [85].

Fig. 58.9
figure 9

(a) Squamous cell carcinoma of the nasal vestibule. (b) The same patient’s reconstruction

T1 lesions are superficial and limited to the nasal vestibule.

T2 lesions are lesions extending from nasal vestibule to nasal septum, upper lip, and nasal skin, but not fixed to the bone.

T3 lesions extend to the hard palate, gingivobuccal sulcus, upper nasal septum, conchae, paranasal sinuses, together with deep muscles and bone.

If a good functional and cosmetic result will be achieved, surgery is preferred. If it is not possible to achieve such a result, radiotherapy may be preferred at early stages. According to the spread of the tumor, excision of cartilage and nasal skin may also be required. In reconstruction, repairs of the intranasal portion, the cartilaginous roof, and the nasal skin are planned. Local pedicle skin flaps may be used together with free cartilage grafts. If excisions of caudal part of septum and anterior nasal spin are required, it may lead to collapse. For frontal support, costal graft may be necessary. In tumors with advanced stage, RT is recommended following surgery [86].

If nodal involvement is present, neck dissection is suggested. If there is no nodal involvement, elective neck dissection or RT is suggested only if the tumor is in advanced stage; otherwise, they are not recommended, because the incidence of lymph node involvement during the first presentation of the patient is 6% [87].