Familial Nasopharyngeal Carcinoma 6

42 S. S. Lo and J. J. Lureviews the natural history, presenting symptoms, anddiagnosis of NPC.4.2Natural HistoryAs originally described by Ho from Hong Kong, thereare three clinical types of NPC (Ho 1970). The typesinclude: (1) the mainly invasive type, (2) the mainlymetastatic type, and (3) mixed type. In the mainlyinvasive type, the main pattern of disease progressionis local invasion. Metastasis to cervical lymph nodes iseither absent or insignificant. However, hematogenousspread to distant sites such as the spine, the lungs, andthe liver can occur when the tumor invades the basalvenous sinus. In the mainly metastatic type, thereis early metastasis to the cervical lymph nodes.Hematogenous spread usually occurs after cervicallymph node metastasis. The most common sites ofmetastases are bone, lung, liver, and superior mediastinaland hilar lymph nodes. In the mixed type,invasion of adjacent structures by the NPC and metastasesmay occur concurrently or sequentially.4.3Presenting Symptoms and SignsThe presenting symptoms and signs of NPC can beclassified according to the involved anatomic regionsand they include cervical mass, nasal symptoms,ear symptoms, neurologic symptoms, eye symptoms,headache, paraneoplastic syndrome, and miscellaneoussymptoms.4.3.1Cervical MassThis is the most common presenting symptom promptingthe patient to seek medical evaluation and 43% ofpatients present with unilateral or bilateral cervicalmass on physical examination (Skinner et al. 1991). Innearly all cases, the upper cervical nodes enlarge beforethe middle and lower cervical nodes (Sham et al. 1990).The upper cervical nodes are usually more bulkythan the lower cervical nodes, indicative of an orderlyspread in the craniocaudal direction. In most cases, thecervical node enlargement is unilateral, but it is notuncommon to see bilateral cervical adenopathy. Thecervical node enlargement is usually painless unlessit is accompanied by concurrent inflammatory orinfectious process.4.3.2Nasal SymptomsThe most common nasal symptoms include bloodstainednasal discharge, unilateral or bilateral nasalobstruction, and posterior nasal discharge, which isblood-stained, and approximately 30% of patientspresent with these symptoms (Skinner et al. 1991).Some patients may experience epistaxis or hawkingand coughing up of blood-stained sputum in themorning secondary to postnasal dripping of bloodstaineddischarge down the airway at night. Becauseof the nasal blockage, patients frequently speak witha nasal twang. Many of these symptoms can be confusedwith those caused by sinusitis or rhinitis. Whensuch symptoms are present, it is crucial to examinethe nasopharynx thoroughly.4.3.3Ear SymptomsThe most common ear symptom is conductive hearingloss as a result of middle ear effusion causedby the blockage of the Eustachian tube. The hearingloss is usually unilateral (Skinner et al. 1991). Sinceserious otitis media is relatively rare in adults whencompared with that in children, the new onset of unilateralconductive hearing loss can be a warning signfor nasopharyngeal carcinoma. Therefore, a thoroughexamination of the nasopharynx is necessary ifserious otitis media does not clear out in 2–3 weeksin an adult patient. Other common symptoms includetinnitus, which can occur in approximately 1/3 ofpatients with NPC. Again, tinnitus in the setting ofNPC is usually unilateral. In cases where there isinfiltration of the glossopharyngeal nerve by NPC,severe unilateral otalgia can occur.4.3.4Neurological SymptomsNeurological symptoms are usually indicative oflocally advanced disease. Depending on the extent ofinvolvement by the primary tumor, any of the cranialnerves, the upper cervical sympathetic nerves, thelesser occipital nerve, and the greater auricular nerve