You are in: eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > HEAD AND NECK ONCOLOGY Cancer of the HypopharynxArticle Last Updated: Feb 7, 2007AUTHOR AND EDITOR INFORMATIONAuthor: David Goldenberg, MD, Associate Professor of Surgery, Director of Head and Neck Surgery, Department of Surgery, Division of Otolaryngology - Head and Neck Surgery, Penn State College of Medicine, Milton S Hershey Medical Center David Goldenberg is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery and American Head and Neck Society Coauthor(s): Joseph A Califano, MD, Associate Professor, Department of Oncology, Division of Head and Neck Cancer Research, Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University Editors: Benoit J Gosselin, MD, FRCSC, Associate Professor of Surgery, Dartmouth Medical School, Dartmouth College, Hanover, NH. Director, Comprehensive Head and Neck Oncology Program, Norris Cotton Cancer Center, Lebanon, NH. Staff Otolaryngologist, Division of Otolaryngology-Head and Neck Surgery, Dartmouth-Hitchcock Medical Center, Lebanon, NH; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Karen Hall Calhoun, MD, Chair, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Missouri; Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders; Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine Author and Editor Disclosure Synonyms and related keywords: cancer of the hypopharynx, hypopharyngeal cancer, laryngeal cancer, hypopharynx, laryngopharynx, larynx, cancer, carcinoma, metastasis, neck cancer, throat cancer, postcricoid cancer, Plummer-Vinson syndrome, Paterson-Kelly syndrome, dysphagia, hypopharyngeal webs, esophageal webs, staging, cancer staging, laryngopharyngectomy, esophagectomy, postcricoid carcinoma, pyriform apex lesion, pyriform sinus lesion, partial laryngopharyngectomy, PLP, partial pharyngectomy, laryngectomy, hypopharynx cancer INTRODUCTIONCancer that arises in the hypopharynx represents approximately 7% of all cancers of the upper aerodigestive tract. An estimated 3000 new cases of hypopharyngeal cancer were diagnosed per year in the United States between 1987 and 1991 (Hoffman, 1997). The incidence of laryngeal cancer is 4-5 times that of hypopharyngeal cancer. The biological behavior of carcinoma of the hypopharynx differs greatly from that of carcinoma of the larynx. Carcinomas of the hypopharynx are usually poorly differentiated, and early presentations are unfortunately uncommon. In fact, T1 N0 cases (see Staging) represent only 1-2% of all patients seen. The rate of metastases is high, with nodal involvement present in 50-70% of cases at presentation. The frequency of distant metastases is also among the highest of all head and neck cancers (Garden, 2001). The prognosis of hypopharyngeal cancer is poor, with most series reporting a 5-year survival rate of less than a 25%. ProblemCarcinomas of the hypopharynx are often poorly differentiated. Early presentations are uncommon. FrequencyApproximately 2500 new cases of hypopharyngeal cancer are diagnosed in the United States each year (Arriagada, 1983). EtiologyPatients diagnosed with hypopharyngeal cancer are typically men aged 55-70 years with a history of tobacco use and/or alcohol ingestion. The combined use of tobacco and alcohol has a synergistic effect on the incidence of hypopharyngeal cancer. One exception is an increased incidence of postcricoid cancer in women aged 30-50 years with Plummer-Vinson or Paterson-Kelly syndrome. This syndrome includes dysphagia, hypopharyngeal and esophageal webs, weight loss, and iron deficiency anemia. Currently in the United States, because of the reduced incidence of Plummer-Vinson syndrome, postcricoid carcinoma is more common in men. Asbestos may pose an independent risk for the development of hypopharyngeal cancer (Marchand, 2000). PathophysiologyIn the United States and Canada, 65-85% of hypopharyngeal carcinomas involve the pyriform sinuses, 10-20% involve the posterior pharyngeal wall, and 5-15% involve the postcricoid area (Pignon, 2000). ClinicalSymptoms of hypopharyngeal cancer include dysphagia, chronic sore throat, and foreign body sensation in the throat or referred otalgia. (Otalgia is pain referred to the ear via the Arnold nerve, a division of the 10th cranial nerve. It suggests an underlying malignancy.) For hypopharyngeal cancer, a metastatic node in the neck is often the presenting symptom. An asymptomatic mass in the neck, usually a jugulodigastric or jugulo-omohyoid lymph node, is present in 20% of patients. The average duration of symptoms before presentation is 2-4 months. Other symptoms, which usually develop later, include weight loss, hemoptysis, and hoarseness when the vocal cord becomes affected by direct extension into the arytenoid cartilage or the recurrent laryngeal nerve. Of patients with hypopharyngeal cancers, 70% have stage III disease at presentation. Physical examination Assessment begins in the office with a thorough head and neck examination. Flexible fiberoptic endoscopic examination is important to attempt to localize and stage the primary tumor. Hypopharyngeal cancer is typically advanced at presentation, and an obvious abnormality is usually present in either the pharynx or the neck. Occasionally, only subtle signs such as submucosal fullness or unilateral pooling of saliva are present. During the flexible laryngoscopy, the assessment of vocal cord mobility or fixation is important for staging purposes. The patient who puffs out his or her cheeks or performs a Valsalva maneuver may distend the pyriform fossae for inspection. The neck should be examined in a systematic fashion. Any lymph nodes should be assessed with regard to size, location, and mobility. On neck examination, loss of the grating sensation (laryngeal crepitus) of the laryngeal cartilages over the prevertebral tissues may indicate deep pharyngeal wall involvement. INDICATIONSEarly hypopharyngeal cancer Early carcinomas of the hypopharynx are not common. Small lesions, particularly of the lateral or posterior wall, may be amenable to partial pharyngectomy or partial laryngopharyngectomy (PLP). In these cases, radiation may be the therapy of choice, offering better functional outcome and the ability to address occult cervical nodal disease (Garden, 2001). Pyriform fossa cancer Lesions that do not extend into the apex of the pyriform fossa, the posterior wall, or the postcricoid may be resected while preserving the larynx, but extension to the apex or involvement of the postcricoid area indicates advanced (T3 or T4) disease and dictates laryngopharyngectomy. Tumors isolated to the lateral wall of the pyriform fossa may be treated with a partial pharyngectomy and resection of the upper thyroid ala. Extension to the medial wall of the pyriform fossa without vocal cord fixation may be managed with a PLP. Postcricoid cancer Most patients with postcricoid cancer develop symptoms at a later stage of their disease. Thus, tumors in this site are usually large and require a laryngopharyngectomy. Extension into the esophagus necessitates a pharyngoesophagectomy and reconstruction. Paraesophageal and paratracheal lymph node dissection with hemithyroidectomy is usually also indicated. Posterior wall cancer Tumors in this site can be localized and may present an opportunity for wide excision through a suprahyoid pharyngotomy. On the other hand, submucosal spread and fixation to prevertebral structures complicate resection. If the involvement of the prevertebral structures is extensive, the patient may not be a suitable surgical candidate. RELEVANT ANATOMYThe hypopharynx, or laryngopharynx, is the longest of the 3 segments of the pharynx. It is wide superiorly and progressively narrows toward the level of the cricopharyngeal muscle. The hypopharynx is a continuous area; the oropharynx is above it and the cervical esophagus through the cricopharyngeal sphincter is below it. This region is known as the pharyngoesophageal junction or postcricoid area. It is bounded anteriorly by the posterior face of the cricoid cartilage. The parts of the hypopharynx that lie partly to each side of the larynx form the pyriform sinuses or fossae, so named for their pear shape. The pyriform sinuses are bound laterally by the thyroid cartilage and medially by the lateral surface of the aryepiglottic fold, arytenoids, and cricoid cartilages. The pyriform sinuses extend from the glossoepiglottic folds to the upper esophagus. The superior laryngeal nerve lies deep to the mucosa of the lateral wall of the pyriform fossa. The constrictor muscles, covered with mucous membrane, form the posterior wall of the hypopharynx. It extends from the level of the floor of the vallecula to the level of the cricoarytenoid joint. The anterior wall of the hypopharynx is bounded by the larynx. The pharyngeal plexus of nerves, which receives contributions from the glossopharyngeal and vagus nerves, supplies the innervation of the hypopharynx. The vagus nerve supplies motor innervation to the constrictors. Sensory information from the hypopharynx travels along the glossopharyngeal nerve and the internal laryngeal branch of the superior laryngeal nerve, which arises from the vagus nerve. Lymphatic drainage of the hypopharynx is extensive. The pyriform sinuses are drained by a network of lymphatics, which drain primarily to the upper and middle jugular nodes, posterior cervical nodes, and retropharyngeal lymph nodes. Lymphatics of the posterior wall of the hypopharynx drain to the jugular nodes and retropharyngeal nodes. Postcricoid lymphatics drain to the middle and lower jugular nodes and to the paratracheal nodes (Million, 1994). The intimate association between the hypopharynx and the larynx, oropharynx, and esophagus provide for certain dissemination routes of malignant disease. Pyriform sinus carcinomas may spread submucosally into the posterior wall of the hypopharynx, the postcricoid region, or the aryepiglottic fold. Large tumors also extend up into the paraglottic fat, the pre-epiglottic fat, and the base of the tongue. Tumors that arise from the lateral wall or apex of the pyriform sinus often have already invaded the thyroid cartilage. Lesions of the medial wall of the pyriform sinus may spread along the aryepiglottic fold into the false vocal cord and arytenoid cartilage. Medial wall lesions occasionally invade paraglottic and pre-epiglottic fat. They may also grow posteriorly into the postcricoid region and then cross the midline to involve the contralateral pyriform sinus. CONTRAINDICATIONSSee Surgical therapy. WORKUPLab Studies
Imaging Studies
Diagnostic Procedures
StagingThe American Joint Committee on Cancer (AJCC) has designated staging by Tumor, Node, Metastases (TNM) classification (also see Image 2).
TREATMENTMedical therapyNo single therapeutic regimen offers a clear-cut superior survival advantage over other regimens. Although the literature highlights various therapeutic options, it contains few reports that present any valid comparative studies. Laryngopharyngectomy and neck dissection followed by radiation therapy have been the most frequently used surgical therapies for hypopharyngeal cancers. Combined chemotherapy and radiation therapy directed at the primary tumor are the most common nonsurgical approaches for advanced tumors, with radiation alone for early tumors without cervical nodal involvement. Prior to treatment, the risks and benefits of treatment options should be frankly discussed with the patient. In cases of hypopharyngeal cancer, this should specifically address possible severe swallowing and speech dysfunction. A speech language pathologist should evaluate the patient before treatment. Before a treatment option is chosen, the patient's psychosocial factors should be considered.
Surgical therapy
Hypopharyngeal tumors metastasize to the neck early. This is most likely in pyriform fossa tumors and least likely in postcricoid tumors. Bilateral neck metastasis may develop because of rich submucosal lymphatics, which cross the midline. In addition, many patients who have no clinical findings for the neck are found to harbor occult metastasis. Because of the high incidence of clinically positive nodes with hypopharyngeal carcinoma, treating both neck nodes at the time of treatment of the primary tumor is prudent. The site and stage of the patient's tumor and the method of treatment of the primary disease dictate the mode of treatment for the neck. Preoperative detailsSome surgeons consider prevertebral musculature or c-spine involvement, massive mediastinal nodal enlargement, and carotid artery involvement to be contraindications to surgery. These cases usually represent advanced and aggressive disease. COMPLICATIONSCertain factors, such as prior radiation therapy, poor general health, chronic malnutrition, alcoholism, diabetes mellitus, advanced age, and systemic illness, all increase the chance of surgical complications. Intraoperative complications may include hemorrhage, carotid sinus reflux, and damage to cranial nerves. Unilateral resection of the hypoglossal nerve is usually well tolerated without serious sequelae; however, bilateral hypoglossal nerve resection causes severe disability with serious difficulties in feeding, swallowing, and speaking. Resection of the vagus nerve, which carries motor and sensory branches to the larynx and pharynx, causes vocal cord paralysis. Damage to the accessory nerve causes denervation of the trapezius muscle, which is one of the most important shoulder abductors. Damage to the accessory nerve also causes destabilization of the scapula, with progressive flaring of this bone at the vertebral border. Injury to the brachial plexus and phrenic nerve are complications that should be avoided with proper identification of anatomic planes. Postoperative complications are not uncommon after extensive head and neck surgery. In the immediate postoperative period, the patient is at greatest risk for pulmonary embolism, gastric ulceration, hemorrhage, and aspiration pneumonia. Other more specific early complications include infection, fistula formation, and subsequent mediastinitis. When a fistula or infection in the neck is detected, the wound should be opened widely and packed. The tract should be diverted away from the carotids and microvascular pedicle. Later complications include pharyngoesophageal stenosis and stricture. A stricture that causes dysphagia may develop some weeks or months after surgery or after radiotherapy is complete. Other late complications include chronic pharyngocutaneous fistula and functional deficits in swallowing. OUTCOME AND PROGNOSISPatients in whom lesions are detected early and who have negative nodes have 5-year survival rates higher than 70% (Martin, 1980). Most cases are, however, advanced, and the overall survival rate rarely exceeds 25% in any series. Postcricoid carcinomas and pyriform apex lesions fare more poorly than superiorly based pyriform sinus lesions. Postoperative irradiation improves the results of surgery. Despite a good local control rate, the 5-year survival rate does not exceed 35%; most patients succumb to distant metastases, intercurrent diseases, or second primaries. In the past, chemotherapy was used for palliative purposes because of poor outcomes in the treatment of cancer of the head and neck. With the introduction of platinum-based chemotherapeutics, either as a single agent or in combination with other drugs, partial response rates and complete response rates have improved. Of late, concomitant chemoradiotherapy has been show to result in a significant increase in survival. Altered fractionation radiation has been explored in advanced head and neck cancers. The largest randomized trial was a 4-arm trial carried out by the RTOG (RTOG 90-03) evaluated the effectiveness of conventional irradiation, a split course accelerated fractionation, delayed concomitant boost and hyperfractionated irradiation. In this study, only 13% of patients had hypopharyngeal cancers. Both hyperfractionated irradiation and concomitant boost showed significantly better locoregional control, disease-free survival, disease-specific survival, and overall survival. Importantly, these trials and studies do not specifically address hypopharynx cancers. Thus, this site is rarely studied separately; in case of mixed sites, the hypopharynx represents just a few cases. Little is known about the selection of patients with cancer of the hypopharynx (detailed locoregional extension, performance status), and no information is provided about the possibilities of surgical salvage. MULTIMEDIA
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Cancer of the Hypopharynx excerpt Article Last Updated: Feb 7, 2007 | |||||||||||||||||||||||||||||||||