Philosophy of management

The management of benign sinonasal masses should follow a balanced algorithm of the need for resection weighed against the adverse effects of surgical removal. Lateral rhinotomy and subtotal maxillectomy for such conditions as IP, osteoma, or fibrous dysplasia (FD) may result in greater morbidity than observation alone, particularly if such lesions are asymptomatic. Conversely, surgical removal of benign sinonasal masses should be contemplated when they are causing symptoms or pending complications or when the suspicion of malignancy exists ( Box 1 ).

Complete tumor removal is always the desired treatment end point. Unlike malignancy, however, total en bloc excision with significant normal tissue removal can be excessive for some of these lesions and can result in unacceptable cosmesis, functional impairment, dental disruption, epiphora, orbital displacement, and nerve injury. These adverse effects may be acceptable for the management of malignant disease when the consequences of persistent local, regional, or distant disease are significant, but for benign disease, subtotal resection for the purposes of relieving the current symptomatology may be better practice when the potential for morbidity is high. When the suspicion for malignancy or potential malignant change is high (eg, IP), this morbidity needs to be balanced.

Common sites of tumor involvement for which this morbidity must be weighed are the dura and periorbita. There are many well described techniques for reconstructing these areas that are highly reliable, but resection of the dura or periorbita removes a natural barrier to tumor spread. Removal of soft tissues tumors from bone is significantly easier than direct dural or periorbital removal, and observation of residual benign tumor around the dura, periorbita, or carotid may be more prudent than a partial disruptive attempt at removal. Aggressive attempts to remove tumor from these soft tissue barriers are unlikely to result in wide negative margins, and a small breech may result in intracranial or orbital recurrence of benign disease that can subsequently require radical treatment with significant morbidity. In contrast to the surgical philosophy for sinonasal malignancies, the authors’ approach to confirmed benign disease involving these areas is more conservative to ensure that the surgical “cure” is not worse than the disease. A staged second resection may be more prudent.

Surgeons have always been cognizant of the need to keep morbidity low for benign conditions. The surgical management of benign conditions has commonly proceeded through limited approaches (eg, early intranasal procedures for IP with high recurrence rates), however. Limited approaches have often resulted in incomplete removal of disease at common sites ( Box 2 ). Lack of vision, heavy reliance on angled instruments, and “scraping” dissection techniques all increase the risk for recurrence at these areas. The authors typically approach these lesions with an en bloc resection philosophy but with strict attention to sites of tumor attachment even if tumors need to be debulked to visualize margins properly.

This article describes an aggressive surgical approach to these lesions while maintaining respect for their benign nature and the importance of preserving natural barriers to growth (spread), such as the dura and periorbita.

Symptoms

The growth of many benign masses within the paranasal sinuses is slow, and the clinical presentation is tempered by adaptation on the part of patients. Adaptation describes the phenomenon of decreased symptom awareness secondary to gradual impairment of function over time. Nasal obstruction is the most common presentation despite adaptation. Most patients (58%–76%) have unilateral nasal obstruction. The symptom duration may be misleadingly short, because many patients complain of a short symptom course with a concurrent viral upper respiratory tract infection. Although the patient history may be brief, the pathologic condition may have been present in considerable size for many months with adaptation to the functional component.

Epistaxis is also common for benign lesions (17%–21%). Initially, this symptom may seem to be more strongly associated with malignant disease. Benign lesions often cause disruption to nasal airflow, however, with subsequent turbulence, crusting, and small areas of mucositis and mucosal excoriation. Patients often blow the affected side of their nose with greater force to relieve symptoms, and self-digitalization is commonly performed to help clear crusting. These all increase the chance of nasal bleeding in benign disease.

Pain is commonly perceived in up to 18% of cases. The pain experienced is rarely severe, sharp, or debilitating. Subtle pressure and facial ache are more frequently encountered. This may be the result of ostial obstruction with changes in luminal pressure or inflammatory dysfunction of the sinuses. Additionally, it can be generated by dural or periorbital compression or contact. The ability of benign masses to cause persistent pain without evidence of sinus dysfunction is still debated. Many rhinologic surgeons have encountered patients, without surrounding sinus changes or dural or periorbital contact, with pain as their presenting feature. This situation is not uncommonly encountered, because many of these patients with headache of alternative origin have had a CT scan or other radiologic imaging and the subsequent finding of what might be an incidental lesion. After exclusion of other headaches causes, and especially in the presence of localization of pain, many of these patients have received benefit from tumor removal. The nature of pain generation may be based on periosteal reaction or localized osteitis.

Persistent sensation of postnasal drainage (PND) and bilateral nasal obstruction can occur in 12% and 14% of patients, respectively. PND is likely to be the result of mucociliary dysfunction, air turbulence, and subsequent changes in mucus rheology. Bilateral obstruction usually results from secondary septal deformity compromising the contralateral nasal cavity. Acute sinusitis is uncommon (9%). Mucociliary function is often retained until late in tumor growth, and bacterial infection of a compromised sinus is not common. The presentation of a nasal mass and epiphora also appears later in the disease progression. Olfactory loss is rare, because contralateral function usually prevents clinical symptoms. Eye symptoms can occur from growth into the orbit with proptosis, and then diplopia, as common orbital symptoms. Visual loss is extremely rare.

Symptoms

The growth of many benign masses within the paranasal sinuses is slow, and the clinical presentation is tempered by adaptation on the part of patients. Adaptation describes the phenomenon of decreased symptom awareness secondary to gradual impairment of function over time. Nasal obstruction is the most common presentation despite adaptation. Most patients (58%–76%) have unilateral nasal obstruction. The symptom duration may be misleadingly short, because many patients complain of a short symptom course with a concurrent viral upper respiratory tract infection. Although the patient history may be brief, the pathologic condition may have been present in considerable size for many months with adaptation to the functional component.

Epistaxis is also common for benign lesions (17%–21%). Initially, this symptom may seem to be more strongly associated with malignant disease. Benign lesions often cause disruption to nasal airflow, however, with subsequent turbulence, crusting, and small areas of mucositis and mucosal excoriation. Patients often blow the affected side of their nose with greater force to relieve symptoms, and self-digitalization is commonly performed to help clear crusting. These all increase the chance of nasal bleeding in benign disease.

Pain is commonly perceived in up to 18% of cases. The pain experienced is rarely severe, sharp, or debilitating. Subtle pressure and facial ache are more frequently encountered. This may be the result of ostial obstruction with changes in luminal pressure or inflammatory dysfunction of the sinuses. Additionally, it can be generated by dural or periorbital compression or contact. The ability of benign masses to cause persistent pain without evidence of sinus dysfunction is still debated. Many rhinologic surgeons have encountered patients, without surrounding sinus changes or dural or periorbital contact, with pain as their presenting feature. This situation is not uncommonly encountered, because many of these patients with headache of alternative origin have had a CT scan or other radiologic imaging and the subsequent finding of what might be an incidental lesion. After exclusion of other headaches causes, and especially in the presence of localization of pain, many of these patients have received benefit from tumor removal. The nature of pain generation may be based on periosteal reaction or localized osteitis.

Persistent sensation of postnasal drainage (PND) and bilateral nasal obstruction can occur in 12% and 14% of patients, respectively. PND is likely to be the result of mucociliary dysfunction, air turbulence, and subsequent changes in mucus rheology. Bilateral obstruction usually results from secondary septal deformity compromising the contralateral nasal cavity. Acute sinusitis is uncommon (9%). Mucociliary function is often retained until late in tumor growth, and bacterial infection of a compromised sinus is not common. The presentation of a nasal mass and epiphora also appears later in the disease progression. Olfactory loss is rare, because contralateral function usually prevents clinical symptoms. Eye symptoms can occur from growth into the orbit with proptosis, and then diplopia, as common orbital symptoms. Visual loss is extremely rare.

Pathologic findings encountered

IPs, juvenile nasopharyngeal angiofibromas (JNAs), hemangiomas, and fibro-osseous masses dominate the published literature. This does not necessarily reflect incidence but, as a result of clinical impact, challenges in management and difficulties in surgical removal. Table 1 presents a comprehensive but not necessarily complete list of benign neoplasms encountered in the nasal cavity and paranasal sinuses. Although it is important to have a structured comprehensive list (see Table 1 ) when unusual masses are encountered or for examination purposes, a common and practical grouping for these conditions is often structured as five simple groups: fibro-osseous (FD, chondroma, and osteoma), IP, neural-related (schwannoma, neuroma, and meningioma), vascular (JNA, hemangioma, and pyogenic granuloma), and odontogenic.

Inverted Papilloma

This is the most common epithelial tumor of the sinonasal tract. The incidence of IP has been documented at 0.2 to 0.6 cases per 100,000 people anually. It is synonymous with inverting papilloma, epithelial papilloma, papillary sinusitis, schneiderian papilloma, inverted schneiderian papilloma, soft papilloma, transitional cell papilloma, polyp with inverting metaplasia, and benign transitional cell growth. Although these names have been used interchangeably to describe this condition, its current description should be IP. Sinonasal papilloma was first described by Ward in 1854, but it was Hyams who helped to define the pathoclinical understanding of the condition. The subdivisions of inverted, septal (fungiform), and oncocytic (cylindric) are still widely accepted today. These lesions are the most common benign epithelial tumors of the nose. They comprise less than 5% of all tumors of the nose, but IP is frequently encountered in the clinical environment because of its potential for growth, malignant change, and high recurrence rate. IP occurs in the fifth to eight decade of life and is generally more common in men than in women. An association of IP with inflammatory polyps is well described, but most are isolated lesions. Only 4.9% of patients present with bilateral or multifocal disease. A human papilloma virus (HPV) origin has been widely investigated. The septal type is most commonly associated with HPV 6 and 11, but even with in situ hybridization techniques, HPV DNA has only been demonstrated in less than 50% of cases. The overall evidence for a causal link is not strong but may explain uncommon multifocal disease.

The classic clinical findings are of a nodular, papillomatous, unilateral mass ( Fig. 1 ). An endophytic or “inverted” growth pattern characterizes these tumors. There is a thickened squamous epithelial proliferation and a mixed chronic inflammatory infiltrate. A downward growth pattern into a fibromyxoid stroma is classic. There are two classic surgical features of IP. The presence of osteitis within the mass can often identify the site of attachment. Bony changes at the attachment site also lead to a rough base, and histologic examination has demonstrated tumor pseudopods extending into the bone, thus requiring bony resection at the attachment.

The classic appearance of IP arising in the left sphenoethmoidal recess.

Atypia, dysplasia, carcinoma in situ, and squamous cell carcinoma can all occur in papilloma. Malignant change within the inverted type of papilloma has been well documented. Krouse originally described a 9.1% rate of squamous cell carcinoma in his cohort. This has been supported by a recent meta-analysis of published case series that reported a synchronous carcinoma rate of 7.1% and a metachronous rate of 11%. It was widely speculated that the atypical and dysplastic changes were precancerous, but this is not supported by the literature. Degree of atypia, the mitotic index, and recurrence seem to have little correlation with carcinoma transformation. Carcinoma in situ should be closely followed, because these cases may represent precancerous change or undetected carcinoma from sampling errors.

Lateral rhinotomy for surgical removal was the standard of care until the past 2 decades. This was based on Weissler’s recurrence rates of 71% for the intranasal approaches, 56% for the limited Caldwell-Luc or external ethmoid approaches, and 29% for the lateral rhinotomy. The intranasal approaches were little more than a polypectomy in the pre-endoscopic era, however. Subsequent studies by Tufano and colleagues and Woodworth and colleagues and a literature meta-analysis have demonstrated a significant advantage to endoscopic resection. A collation of the literature reports recurrence rates of 12.8% for endoscopic resection, 17.0% for lateral rhinotomy, and 34.2% for other limited resections. Selection bias may exist for some of the published reports, but the Krouse staging system is well accepted in the literature to provide a comparison basis ( Table 2 ).

Table 2

Krouse staging for inverted papilloma

Stage
T1	Tumor isolated to one area of the nasal cavity without extension to the paranasal sinuses
T2	Tumor involves medial wall of the maxillary sinus, ethmoid sinuses, or osteomeatal complex
T3	Tumor involves the superior, inferior, posterior, anterior, or lateral wall of the maxillary sinus, frontal sinus, or sphenoid sinus
T4	Tumor with extrasinonasal extent or malignancy

Juvenile Nasopharyngeal Angiofibroma

JNA is an uncommon tumor (<1%) of the sinonasal system, but it has a disproportionately high representation in the literature because of its clinical impact, potential for morbidity, and difficulties in surgical resection. It is a misnomer, with the site of origin accepted as an area in the lateral nasal wall near the sphenopalatine foramen. The lesions occur almost exclusively in men and during the second and third decades of life. There is some debate about the potential for JNA to represent a vascular malformation rather than a tumor. Spontaneous regression has been reported. There is a familial predisposition to JNA, with the lesion being encountered 25 times more frequently in those carrying the familial adenomatous polyposis gene.

The tumor appears as a purple-gray pedunculated mass centered on the sphenopalatine foramen ( Fig. 2 ). Histologically, there is a vascular component of thin-walled vessels of varying sizes. They lack elastic fibers and usually have a partial or incomplete smooth muscle layer. A fibrous stroma extends throughout the lesion. Androgen receptors have been detected within the stroma. The classic radiologic feature of JNA is anterior bowing of the posterior maxillary sinus wall, the Holman-Miller sign. Intracranial extension of JNA may occur, with tumor spreading through well described routes to the skull base. This spread is dominated by an extradural pattern. The Andrew’s classification is currently used for staging ( Table 3 ).

The pale mucosal covering of a JNA occupying the posterior right nasal cavity. The contrasted CT images demonstrate its typical origin near the sphenopalatine foramen. The vascularity of the tumor, which is well shown in the CT images, is not immediately apparent on examination. An injudicious biopsy can result in heavy epistaxis.

Table 3

Andrew’s (modified Fisch) classification for juvenile nasopharyngeal angiofibroma

Type	Tumor Extent
I	Limited to the nasopharynx and nasal cavity, bone destruction negligible or limited to the sphenopalatine foramen
II	Invades the pterygopalatine fossa or the maxillary, ethmoid, or sphenoid sinus with bone destruction
IIIa	Invades the infratemporal fossa or the orbital region without intracranial involvement
IIIb	Invades the infratemporal fossa or orbit with intracranial extradural (parasellar) involvement
IVa	Intracranial intradural tumor without infiltration of the cavernous sinus, pituitary fossa, or optic chiasm
IVb	Intracranial intradural tumor with infiltration of the cavernous sinus, pituitary fossa, or optic chiasm

The endoscopic route has become widely accepted for managing most tumors. Even large tumors involving the middle cranial fossa can be successfully removed endoscopically ( Fig. 3 ). The midface degloving approach is occasionally used for extensive intracranial involvement. A careful devascularization of the tumor before removal is the key to successful endoscopic surgical management. This can be achieved by lateral ligation of the feeding arteries (see Fig. 2 ) or by preoperative embolization. Radiotherapy and hormonal therapy have little role in modern management.

Intracranial extension of a JNA often follows classic pathways. This lesion has developed through the pterygopalatine fossa, involving the foramen rotundum and the middle cranial fossa floor.

Hemangioma

Lobular capillary hemangioma (LCH) dominates the vascular neoplastic masses in the nose and paranasal sinuses. LCH is synonymous with pyogenic granuloma and vascular pregnancy tumor. Pyogenic granuloma is a poor name, however, because the condition is neither an infectious process nor a granulomatous one. These lesions are common throughout life but are uncommon in persons younger than 16 years of age. LCH presents as a red-purple, lobulated, smooth mass ( Fig. 4 ). It classically arises from the septum, but it also arises from the head of the inferior turbinate. Histologically, LCH is characterized by lobules of central capillaries in the submucosal space. There are surrounding feeding vessels. There is a prominent endothelial lining with tufting and mitoses. Atypical mitoses may be associated, but there is little evidence of transformation to angiosarcoma. Cavernous hemangiomas are much less common in the sinonasal tract than LCH. They are more frequently located in the middle turbinates ( Fig. 5 ), in the lateral nasal wall, and within bony structures.

( Left ) Large (1.5 cm) left anterior septal LCH. ( Right ) Excision is a simple zone 1 approach with low recurrence.

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