eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Head & Neck Surgery

Malignant Nasopharyngeal Tumors

Author: Ho-Sheng Lin, MD, FACS, Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Faculty, Sleep Fellowship Program, Divison of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Wayne State University School of Medicine; Chief, Section of Otolaryngology, Department of Surgery, John D Dingell Veterans Affairs Medical Center
Coauthor(s): Willard E Fee Jr, MD, Edward C and Amy H Sewall Professor, Department of Otolaryngology-Head and Neck Surgery, Stanford University Medical Center
Contributor Information and Disclosures

Updated: Oct 16, 2009

Introduction

History of the Procedure

External beam radiation therapy is the primary mode of therapy for previously untreated nasopharyngeal carcinoma (NPC). Recurrent or persistent disease remains a challenge to clinicians. Typically, re-irradiation is advocated. In some institutions, salvage nasopharyngectomy is used for the treatment of recurrent disease.

In 1988, Fee and Tu published results of salvage nasopharyngectomy in a series of patients with recurrent NPC that failed previous treatment with radiation.1,2 The results were encouraging1 and inspired other investigators to start using surgery in the treatment of patients with recurrent NPC. Since then, various surgical approaches to the nasopharynx have been proposed. These include the transpalatal-maxillary-cervical, maxillary swing, transmandibular, transcervico-mandibulo-palatal, infratemporal fossa, lateral temporal, and endoscopic approaches.


Axial T2-weighted image shows a left-sided cervic...

Axial T2-weighted image shows a left-sided cervical nodal metastasis resulting from nasopharyngeal cancer.

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Axial T2-weighted image shows a left-sided cervic...

Axial T2-weighted image shows a left-sided cervical nodal metastasis resulting from nasopharyngeal cancer.


Frequency

Nasopharyngeal carcinoma (NPC) is a prevalent malignancy in Southeast Asia. In areas such as southern China, Hong Kong, Singapore, Malaysia, and Taiwan, the reported incidence rate ranges from 10-53 cases per 100,000 persons per year. The incidence is also high among Eskimos in Alaska and Greenland and in Tunisians, ranging from 15-20 cases per 100,000 persons per year. Although NPC is a relatively uncommon disease in Western countries (<1 case per 100,000 persons), it poses a significant health problem in regions of the United States with large Asian populations. The prevalence rate for people of Asian descent in the United States is 3.0-4.2 cases per 100,000 persons.

Etiology

A clear etiology for nasopharyngeal carcinoma (NPC) is still lacking. In general, NPC is thought to be the result of both genetic susceptibility and environmental factors such as carcinogens and infection with Epstein-Barr virus (EBV). Evidence in support of genetic factors is the association of NPC with genotypes HLA-A2 and HLA-Bsin2, which are prevalent in individuals from southern China but rare in whites. Furthermore, abnormalities of multiple chromosomes, including 1, 2, 3, 4, 5, 6, 8, 9, 11, 13, 14, 15, 16, 17, 22, and X, have been identified.

Possible environmental or cultural factors that may be associated with NPC include the ingestion of Cantonese-style salted fish and preserved foods that contain carcinogenic nitrosamines, especially during childhood. Evidence of EBV-DNA in almost all NPC cells that were studied supports the association of NPC with EBV. Further, the detection of clonal EBV-DNA in NPC suggests that the malignancy is a clonal expansion of a single EBV-infected progenitor cell. This finding indicates that EBV is present within the cell at the time of malignant transformation and suggests a role for the virus in contributing to the early transformation event. The contribution of both genetic factors and environmental factors for this disease is reflected in the observation that the incidence of NPC for American-born, second-generation Chinese individuals is lower than that for Chinese-born individuals in China but remains higher than that for white individuals in the United States.

Presentation

Although reported in all age groups, a bimodal peak incidence appears to occur in individuals aged 30-40 years and 50-60 years. Nasopharyngeal carcinoma (NPC) is observed predominantly in males, with a male-to-female ratio of 3:1. Clinically, NPC has few early warning signs, which often means late diagnosis. Early but nonspecific symptoms include nasal obstruction, blood-tinged sputum or nasal discharge, tinnitus, headache, ear fullness, and unilateral conductive hearing loss from serous otitis media or recurrent acute otitis media. In advanced cases, the tumor can invade the skull base and spread intracranially through one of the many nearby foramina. Evidence of cranial nerve involvement (III-VI), including diplopia and numbness of the face, suggests cavernous sinus invasion.

The abundant supply of regional lymphatic vessels in the nasopharynx contributes to the high prevalence of cervical metastasis. Approximately 44-57% of patients initially seek medical attention because of a metastatic lymph node that manifests as a neck mass. At the time of diagnosis, 60-85% of patients already have cervical metastasis.

Systemic dissemination also occurs more readily in NPC than in other head and neck cancers. The most frequently involved sites are bone, lung, and liver. Distant metastases are present in 5-10% of patients at the initial presentation.

Indications

Salvage nasopharyngectomy and neck dissection may be indicated in patients with nasopharyngeal carcinoma (NPC) that persisted or recurred locoregionally following prior treatment with radiation with or without chemotherapy. The proper selection of patients and surgical approach are essential for a successful outcome.

Relevant Anatomy

Nasopharynx

The nasopharynx is defined anteriorly by the posterior choanae, posteriorly by the clivus and the first 2 cervical vertebrae, superiorly by the floor of the sphenoid, and inferiorly by the level of the free border of the soft palate. The nasopharynx is divided into 3 subsites: the posterosuperior wall, the lateral walls, and the posterosuperior surface of the soft palate. The torus tubarius is the opening of the eustachian tube into the lateral nasopharyngeal wall. The fossa of Rosenmüller is the groove or recess posterior to the torus at the junction between the lateral and posterior walls. Nasopharyngeal carcinoma (NPC) most commonly occurs in this location.

The posterior and lateral nasopharyngeal walls are composed of 3 layers of tissue. The mucosal epithelium of the nasopharynx is complex, consisting mainly of pseudostratified columnar ciliated epithelium near the choanae and the adjacent part of the roof of the nasopharynx, a transitional epithelium in the roof and the lateral walls, and stratified squamous epithelium along the posterior and inferior portions of the nasopharynx. The superior constrictor muscle and the buccopharyngeal fascia surround the mucosa. Superiorly, the buccopharyngeal fascia unites with the pharyngobasilar fascia, which is attached to the skull base.

The buccopharyngeal fascia extends posterolaterally from the free edge of the medial pterygoid plate to the lateral border of the carotid artery. This fascia separates the nasopharynx from the parapharyngeal (paranasopharyngeal) space. A line joining the free edge of the medial pterygoid plate posterolaterally to the styloid process divides the paranasopharyngeal space into the prestyloid space anteriorly and the retrostyloid space (containing the carotid sheath and the cranial nerves) posteriorly. 

The paranasopharyngeal space is bound anteriorly by the pterygomandibular raphe, which joins the lateral pterygoid plate to the mandible. The retropharyngeal space contains the retropharyngeal lymph nodes and the node of Rouviere. This space is located posterior to the buccopharyngeal fascia and anterior to the prevertebral fascia; therefore, lesions that extend beyond the buccopharyngeal fascia posteriorly involve the retropharyngeal space, while lesions extending laterally beyond this fascia reach the parapharyngeal space.

The nasopharynx is an anatomically difficult area to expose surgically. This area is in close proximity to several foramina and associated vital neurovascular structures. These include the foramen ovale, the foramen spinosum, the foramen lacerum, the carotid canal, and the jugular foramen.

Neck

Ho originally described the supraclavicular fossa as a triangular region defined by 3 points: the sternal end of the clavicle, the lateral end of the clavicle, and the point where the neck meets the shoulder.3 This area is clinically significant in that any nodal involvement within this triangle is, by definition, an N3 lesion and, therefore, stage IV cancer.

Contraindications

Salvage nasopharyngectomy is contraindicated in patients with locally unresectable recurrent nasopharyngeal cancer and patients with distant metastasis.

More on Malignant Nasopharyngeal Tumors

Overview: Malignant Nasopharyngeal Tumors
Workup: Malignant Nasopharyngeal Tumors
Treatment: Malignant Nasopharyngeal Tumors
Follow-up: Malignant Nasopharyngeal Tumors
Multimedia: Malignant Nasopharyngeal Tumors
References
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Further Reading

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Keywords

malignant nasopharyngeal tumors, nasopharyngeal carcinoma, NPC, nasopharyngeal cancer, undifferentiated carcinoma, lymphoepithelioma, nasopharyngectomy, salvage nasopharyngectomy, head and neck cancer, head and neck carcinoma, cervical metastasis, EBV

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Contributor Information and Disclosures

Author

Ho-Sheng Lin, MD, FACS, Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Faculty, Sleep Fellowship Program, Divison of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Wayne State University School of Medicine; Chief, Section of Otolaryngology, Department of Surgery, John D Dingell Veterans Affairs Medical Center
Ho-Sheng Lin, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Sleep Medicine, American Association of University Professors, American College of Surgeons, American Head and Neck Society, Association of VA Surgeons, Chinese American Medical Society, Southwest Oncology Group, and Triological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Willard E Fee Jr, MD, Edward C and Amy H Sewall Professor, Department of Otolaryngology-Head and Neck Surgery, Stanford University Medical Center
Willard E Fee Jr, MD is a member of the following medical societies: American College of Surgeons, American Laryngological Association, American Laryngological Rhinological and Otological Society, American Medical Association, California Medical Association, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Accuray, Inc Ownership interest Consulting; Health Development Corp. Ownership interest Consulting; IRX Therapeutics Grant/research funds Other; NIH Grant/research funds Other

Medical Editor

Benoit J Gosselin, MD, FRCSC, Associate Professor of Surgery, Dartmouth Medical School; Director, Comprehensive Head and Neck Oncology Program, Norris Cotton Cancer Center; Staff Otolaryngologist, Division of Otolaryngology-Head and Neck Surgery, Dartmouth-Hitchcock Medical Center
Benoit J Gosselin, MD, FRCSC is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society, American Medical Association, American Rhinologic Society, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, College of Physicians and Surgeons of Ontario, New Hampshire Medical Society, North American Skull Base Society, and Ontario Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Nader Sadeghi, MD, FRCS(C), Associate Professor of Surgery, Director of Head and Neck Surgery, Division of Otolaryngology, George Washington University
Nader Sadeghi, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society, Federation of Medical Specialists in Quebec, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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