Unilateral nasal obstruction, due to the extension of the lesion into the nasal fossa through the ethmoidal infundibulum or by the erosion of the medial bony wall, is often the presenting complaint of a maxillary sinus carcinoma (Miyaguchi et al. 1990). Epistaxis and nasal discharge may be associated. Another frequent heralding symptom is facial pain, which may be localized to the orbit, the cheek, or the tooth, according to the local extent of the tumor (Miyaguchi et al. 1990).
Other signs and symptoms are related to the growth of the neoplasm according to different pathways. Anterior extension beyond the bony wall is suggested by a premaxillary swelling, which can be observed in up to 41.7% of patients (Paulino et al. 1998). Progression of the disease into the soft tissues may lead to skin fixation and ulcer. A lesion extending towards the orbit may be associated with symptoms related to involvement of the infraorbital nerve, such as pain, paresthesia and/or anesthesia of the skin covering the upper lip, the nasal pyramid, and the premaxillary region. When the mass pushes and dislocates the ocular bulb, proptosis and diplopia may appear. It is worth remembering that diplopia may also be a sign of extrinsic ocular muscles infiltration. Epiphora is caused by compression or infiltration of the lacrimal pathways or by an abnormal stimulation of the vidian nerve. Posterior extension towards the pterygopalatine fossa is suggested by ipsilateral tooth and facial pain due to maxillary nerve involvement; major palatine nerve involvement is associated with hemi-palate paresthesia and pain. Xerophthalmia suggests infiltration of the sphenopalatine ganglion. Trismus due to extension of the lesion into the masticatory space is usually a sign of an advanced-stage disease. The extension towards the underlying alveolar process may be accompanied by tooth protrusion and eventual unhealed tooth extraction (Paulino et al. 1998). Hard palate involvement is suggested by a submucosal swelling or by the appearance of an ulcerated lesion.
At presentation, cervical node metastases may be detected in 3% to 20% of patients; regional me-
tastases develop in 7.3% to 28.9% of patients after treatment (Rinaldo et al. 2002b). The occurrence of lymph node metastases is more common in tumors with oral cavity involvement (Kim et al. 1999a; Cantu et al. 2002). Levels I, II, and III are the most frequently involved, whereas the prevalence of retro-lateral-pharyngeal node metastases is not well established (Cantu et al. 2002; Rinaldo et al. 2002b).
Early predominant symptoms and signs of ethmoid malignancies are nasal obstruction and/or epistaxis (Wax et al. 1995); nasal discharge may be also present at diagnosis. When the nasal septum has been infiltrated and the contralateral nasal fossa has been filled by the tumor, bilateral nasal obstruction is detected (Biller et al. 1989; Salvan et al. 1998). Pain, either for direct neural infiltration or for the occurrence of an acute obstructive sinusitis caused by the tumor itself, may be the heralding symptom in about 11% of patients (Lund et al. 1998); the first trigeminal branch is the most frequently involved.
Though ocular complaints may reveal the existence of an ethmoid malignancy, their occurrence can be observed also when the lamina papyracea has been compressed and displaced but not yet invaded. Most ethmoid cancers, when diagnosed, abut the lamina papyracea. Once it has been eroded, there is still a barrier that prevents orbital fat invasion: the periosteum investing the orbital bones (Kraus et al. 1990; Salvan et al. 1998). Tumor extent through the periosteum may be micro- or macroscopic. In the first case, signs and symptoms of intraorbital extension are usually absent. Conversely, spread through the posterior lamina papyracea is associated with orbital signs like periorbital swelling, extrinsic ocular muscle impairment, orbital displacement, proptosis, or visual disturbance. Epiphora and recurrent dac-ryocystitis should suggest the possibility of an anterior ethmoidal adenocarcinoma infiltrating the lacrimal pathway.
Superior extension into the anterior cranial fossa through the ethmoid roof is observed in up to 50% of cases of ethmoid cancers (Bridger and Baldwin 1989; Kraus et al. 1990; Wax et al. 1995). Subsequent involvement of the olfactory tract may cause hypo- or anosmia. However, the same symptoms may occur also in presence of a neoplasm interfering with the air flow in proximity of the olfactory epithelium (Wax et al. 1995). Tumor extent beyond the ethmoid bony roof may cause dural dis placement, and eventually infiltration, either micro-or macroscopic. When the mass is extradural or has only a microscopic dural infiltration, all signs and symptoms suggestive for CNS involvement, apart for headache, could be absent. Massive transdural growth, with possible brain infiltration, may be instead associated with cerebrospinal fluid leakage, mental confusion and seizures.
Less frequently the tumor extends posteriorly towards the sphenoid, pterygopalatine fossa, and middle cranial fossa or into the optic canal through the posterior medial orbital wall (Cheesman et al. 1986).
Cervical node metastases are observed in less than 3% of patients with adenocarcinoma. A higher incidence, up to 46%, is associated with undifferentiated carcinoma (Cantu et al. 2002).
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