Familial Nasopharyngeal Carcinoma 6

Natural History, Presenting Symptoms, 4and Diagnosis of Nasopharyngeal CarcinomaSimon S. Lo and Jiade J. LuCONTENTS4.1 Introduction 414.2 Natural History 424.3 Presenting Symptoms and Signs 424.3.1 Cervical Mass 424.3.2 Nasal Symptoms 424.3.3 Ear Symptoms 424.3.4 Neurological Symptoms 424.3.5 Eye Symptoms 434.3.6 Headache 434.3.7 Paraneoplastic Syndrome 434.3.8 Miscellaneous Symptoms 434.4 Neurological Syndromes 444.5 Routes of Tumor Spread 444.5.1 Clinical Anatomy and LymphaticDrainage 444.5.2 Local Spread 454.5.3 Regional Nodal Spread 464.5.4 Distant Spread 464.6 Diagnosis 464.6.1 History and Physical Examination 474.6.2 Differential Diagnosis 484.6.3 Biopsy 484.6.4 Diagnostic Imaging Studies Required 494.6.5 Serology 494.7 Algorithm of a StandardizedDiagnostic Procedure 494.8 Summary 49References 50Simon S. Lo, MDDepartment of Radiation Medicine, Arthur G. James CancerHospital, Ohio State University Medical Center, 300 West 10thAvenue, Columbus, OH 43210, USAJiade J. Lu, MD, MBADepartment of Radiation Oncology, National UniversityCancer Institute, National University Health System, NationalUniversity of Singapore, 5 Lower Kent Ridge Road, Singapore119074, Republic of Singapore4.1IntroductionEarly diagnosis has been demonstrated to correlatewith favorable treatment outcomes in patients withnasopharyngeal carcinoma (NPC). Unfortunately, theclinical manifestation can often be very deceptive andconfusing until the disease progresses into a relativelyadvanced stage. Coupled with the difficulty associatedwith thorough examination of the nasopharynx,this disease presents a diagnostic challenge to physicians.Only about 10% of all new patients present withearly disease despite recent advances in diagnostictechniques such as fiberoptic examination, diagnosticimaging, and tumor serology. The presentingsymptoms are closely related to the location of thetumor in nasopharynx, the extent of local tumor invasion,and the degree of regional nodal metastasis.Because early symptoms are frequently minimal innature, they are easily ignored by the physician andthe patient. A high index of suspicion is crucial in atimely diagnosis of nasopharyngeal carcinoma, especiallyin endemic areas like Southeast Asia and areaswith a large number of Chinese immigrants. As mentionedearlier, the treatment outcomes of NPC dependon the stage of the disease, and delaying the diagnosisof NPC can potentially be detrimental.Apart from a correct diagnosis, detailed interpretationof the presenting symptoms can facilitate accuratedelineation of the extent of disease. Certain symptomsand signs will direct further detailed review of areas ofsuspected involvement, especially in the base of skullarea, on diagnostic imaging studies such as computerizedtomography (CT) and magnetic resonance imaging(MRI). A geographic miss can potentially occur ifthe target volume is underestimated, especially in themodern era where highly conformal radiation therapytechniques such as intensity-modulated radiationtherapy are used to minimize toxicities. This chapter