Local Disease

A mass in the nasal cavity will produce unilateral nasal obstruction, discharge that is often bloodstained, a reduction in the sense of smell, and occasionally facial discomfort.1 Frank epistaxis is less common than might be supposed, although it typically occurs with vascular lesions such as juvenile angiofibroma, olfactory neuroblastoma, and malignant melanoma.

Tumors that arise in or involve the ethmoidal labyrinths obviously affect the nasal cavity at an early stage and may spread across the midline, for example, adenocarcinomas, but more often present with orbital symptoms due to early erosion of the lamina papyracea. These include proptosis, a sign that is almost always due to a mass either within or outside the orbit. The direction and degree of displacement will depend on the position of the mass and its speed of growth. This in turn may be associated with diplopia if displacement occurs quickly but may be absent if the change in position takes place slowly. Similarly, the optic nerve and its blood supply can tolerate impressive stretching if it occurs slowly, but vision may be lost rapidly when additional inflammation or infiltration is present. Diplopia may also occur as a consequence of direct muscle infiltration or involvement of the neural supply, especially in the cavernous sinus. Once at the orbital apex, whether extra- or intraperiosteal, the disease can spread into the middle cranial fossa. Tumors that invade or arise within the skull base may directly affect the optic nerve(s) and chiasm. This can occur with meningioma and chondrosarcoma and may tragically be bilateral. Visual loss can also result from chemosis due to corneal exposure, keratosis, and ulceration.

The incidence of orbital invasion by sinonasal malignancies will vary with the histology, but overall visual symptoms occurred in 50% of a cohort of 220 cases.1 This rose to 62% if the tumor arose in the ethmoid, compared with 46% with malignant tumors of the nasal cavity. However, it should be noted that the orbital periosteum, unlike the lamina papyracea, is relatively resistant to penetration by malignant tumors, so the presence of orbital symptoms does not indicate intraperiosteal spread per se, nor does the lack of orbital symptoms and signs necessarily mean that the eye has not been infiltrated. A recognition of this led to a change in strategy for the management of the orbit in the 1980s.2 It has been estimated that between 66% and 82% of patients with ethmoidal malignancy have erosion of the lamina, whereas the periosteum is involved in 30 to 50%.3–6

Epiphora due to nasolacrimal involvement is quite often encountered with anterior ethmoidal and maxillary lesions as a result of secondary compression or distortion of the system.

Superior extension of the tumor into the anterior cranial cavity can occur through the lateral lamella of the cribriform plate, along the anterior and posterior neurovascular bundles, or directly through the fovea, but this is generally asymptomatic. Classically this occurs with olfactory neuroblastoma. CSF leakage and meningitis are exceptionally rare and even when the dura has been breached and extensive frontal lobe infiltration occurs, any personality changes are usually too subtle to be noticed. Occasionally patients with direct involvement of the olfactory bulbs and tracts may develop reduction or distortion of the sense of smell, for example, in frontal meningioma, although this rarely attracts much attention.

Tumors arising within the anterior ethmoids/middle meatus can spread into the maxillary sinus and/or into the frontal sinus via the frontal recess, occasionally producing a mucocele, although this is a rare phenomenon in the presence of a malignant tumor. We have had three patients with sinonasal malignancies who have presented in this way (0.3%). It is not known why the frontal and sphenoid sinuses are rarely the site of primary malignant tumors and are more often involved by local spread or involvement from the surrounding bone. A frontal sinus tumor is most likely to present with swelling of the forehead, whereas sphenoid tumors produce orbital symptoms, in particular visual loss.

Malignancy of the maxillary sinus will spread medially into the nasal cavity, producing obstruction and serosanguinous discharge, as before, but may also spread superiorly, particularly into the infraorbital canal,7 producing paresthesia of the cheek as well as orbital symptoms. Direct anterior spread through bone or via the infraorbital foramen may produce a mass in the cheek, which in turn may ulcerate. Inferior spread from the maxilla produces a mass in the oral cavity, loosening of teeth, and/or a malignant oroantral fistula. Posterior spread into the pterygoid region and infratemporal fossa is associated with trismus and pain.

Metastatic Disease

Although it is mandatory to examine the neck, fewer than 10% of epithelial malignancies present with cervical disease, reflecting the paucity of lymphatic drainage from the sinuses. This is more often a concern with tumors of the nose and some of the lymphomas and sarcomas. The submandibular, jugulodigastric, prefacial and postfacial nodes are most commonly involved by tumors from the septum and in particular the columellar region; these sometimes spread bilaterally, which is invariably associated with a poor prognosis. However, careful examination may reveal more cervical disease than had hitherto been suspected, as with olfactory neuroblastoma, and the possibility of locoregional spread should always be considered during follow-up even in unusual locations such as the cheek.8

Systemic metastases are generally uncommon but may occur with longer follow-up and in the presence of uncontrolled local disease. Again this is particularly true of olfactory neuroblastoma and also of malignant melanoma. Adenoid cystic carcinoma is also known to spread along perineural lymphatics, either directly or by embolization, often presenting at some distance from the original tumor, although patients can survive for some time with disseminated disease. Patients may be unaware of systemic metastases for some time and it is a matter of debate how aggressively one should seek them out in the case of tumors where the therapeutic options may be limited. Notwithstanding this, particularly at presentation, complaints of an unresolving nonproductive cough, bone pain, or significant fatigue should prompt further investigation as this can have a direct bearing on the management of the primary lesion.