Author(s): Christian Custodio, MD, Darcey Hull, DO, MA
Originally published: September 20, 2013 Last updated: June 30, 2022
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Disease/ Disorder

Definition

Head and neck cancers (HNC) include cancer that arises in the nasal cavity, sinuses, oral cavity (lips, mouth, tongue, hard palate, gums) salivary glands, pharynx (nasopharynx, oropharynx including base of tongue, tonsils, soft palate and hypopharynx) or larynx.1

Epidemiology

Globally, there are over 850,000 new cases each year of head and neck cancer affecting the oral cavity, lip, salivary glands, oropharynx, nasopharynx, hypopharynx, and larynx, and over 450,000 deaths in 2018 due to these cancers.2, 3 There were over 350,000 new cases of oral cavity cancer and over 120,000 new cases of nasopharyngeal cancer in the world in 2018.3 Males have a much higher incidence of these cancers and mortality rates from these cancers than do females.3

Head and neck cancer accounts for slightly less than 3% of all cancers in the United States, with the most commonly diagnosed types of head and neck cancer involving the tongue, tonsils and oral cavity.4,5 The overall incidence of head and neck cancer remained stable from 1987-1991 to 2002-2006 in the United States, vacillating between 10 to 12 cases per 100,000 people.4,5 In 2018, there were an estimated 64,000 newly diagnosed oral cavity, pharynx, and laryngeal cancers, and 13,000 deaths.6

Stage IV HNC incidence, however, has increased from 2004 to 2015 in the US, particularly OP, NP, OC.  Incidence of stage IV laryngeal HNC continues to decline, and stage IV hypopharyngeal cancer incidence has remained stable.  Stage I-III HNC incidence has remained stable or slightly decreased over the same timeframe (Harvey et al, rising incidence of late stage HNC US).

The incidence of head and neck cancer diagnosis increases after the age of 45.4 Men are more than twice as likely to be diagnosed with head and neck cancer compared to women.4 The lifetime risk of developing oral cavity or pharyngeal cancer in the United States is approximately 1%.5 The overall incidence of HNC in African American patients has decreased from 2005 to 2014, though African Americans have the highest incidence of stage IV HNC across all racial groups.7

The five year survival rate improved from 52.7% in 1982-1986 to 65.9% from 2002-2006 for all types of head and neck cancer.4 As of 2013, the five year survival rate for oral cavity and pharyngeal cancer in the United States was 67.8%.5 Survivability of particularly tongue and tonsil carcinoma for age 55-64 year-olds and oral cavity carcinomas for 15-44 year-olds has improved the most. Only patients aged over 74 years old have not had ameliorated survivability of HNC. 4 All-cause mortality, HNC-specific mortality, and stage of presentation were consistently associated with race, sex, insurance, marital status, and SES, with uninsured non-Latino, single Black males with very low SES faring least well.  Black patients with HNC from 2007-2016 had the lowest 5-year survival of 46%.8

It is unclear as to why the five year survival rate has improved, though this may be linked to the fact that HNC carcinoma caused by HPV has been increasing. From 2001-2017, there was an annual increase in HPV related HNC at 2.71% per year. For men, 81% of HPV related cancers were oropharyngeal.9 Roughly 70% of oropharyngeal cancers are caused by HPV in the United States10, and HPV related HNC has augmented survival rates as compared to non-HPV related HNC24. These factors may play a role in why the five year survival rate has improved in recent years.

Risk factors and Prevention

The use of tobacco products and alcohol contribute to a significantly large proportion of head and neck cancer cases.11 Tobacco products and cigarette smoking have a known dose-response relationship contributing to the development of head and neck cancers.12 Alcohol and tobacco use have a multiplicative relationship for the development of head and neck cancer.13 Certain types of human papillomavirus (HPV), especially HPV types 16 and 18, are responsible for over 50% of oropharyngeal cancers.11

Additional risk factors that have been identified include:

  • Premalignant lesions such as leukoplakia and erythroplakia, and possibly lichen planus14
  • Inherited conditions such as Fanconi anemia, Li-Fraumeni syndrome, ataxia telangiectasia and Bloom’s syndrome14
  • Epstein Barr virus in nasopharyngeal carcinomas15
  • Occupational exposure such as formaldehyde in nasopharyngeal carcinomas16
  • Sunlight exposure17
  • Genetics/Family history of HNC17, 18
  • Low body mass index18
  • Higher number of sexual partners18
  • Use of betel quid17
  • Socioeconomic status (unmarried males with less than high school education, family income less than $20,000)19

There are no current screening tests for head and neck cancers that are routinely used.11,17

Preventative methods for head and neck cancer include:

  • Diet, including increased fruit and vegetables20
  • Good oral hygiene21
  • Tobacco cessation17
  • Public and professional education17
  • Vaccination against HPV to prevent Oropharyngeal Cancer22

Pathophysiology

Most HNC are pathologically identifiable as squamous cell tumors that are positive for keratin.21 These types of HNC follow a pattern of hyperplasia, dysplasia, in situ carcinoma followed by invasive cancer, and may be preceded by erythroplakia or leukoplakia.21 Variants of squamous cell HNC include verrucous carcinoma (low-grade, usually in oral cavity), basaloid carcinoma (aggressive and associated with HPV), spindle cell, adenoid and small cell.21

For nasopharyngeal cancers, there are three main types: keratinizing squamous cell carcinoma, nonkeratinizing carcinoma and lymphoepitheliod/undifferentiated carcinoma; conversely, there are a number of different histologic types of sinus and salivary gland cancers.21

Disease Presentation

There are a number of symptoms and signs associated with head and neck cancer. The presenting complaints are dependent on the location of the primary tumor. Hoarseness can suggest laryngeal cancer and dysphagia can suggest pharyngeal cancer.18 Common symptoms and signs of head and neck cancer include18

  • Hoarseness18
  • Sore throat18
  • Stridor18
  • Dysphagia18
  • Dysphonia18
  • Neck mass18
  • Ear pain or effusion18
  • Nasal or ear congestion21
  • Oral, non-healing ulcer18, 21
  • Mouth lesion that is white or red in color18
  • Cranial nerve palsy18

The most common metastases sites are bone, lung and liver.21

Staging head and neck cancer is dependent on the type of tumor, extent of disease, and the presence of nodal or metastatic disease; the formal tumor staging of this information is based upon the combination of this information.21 The American Joint Committee on Cancer (AJCC) and Union for International Cancer Control (UICC) published an updated 8th edition staging system in 2017 for head and neck cancer based upon unique tumor (T), nodal (N), metastatic (M) characteristics, extranodal and depth of extension, and p16 status of oropharyngeal carcinoma.23, 24 Staging is important for prognostication, such that all patients in the same prognostic group should have similar survival rates.18, 24 Patients with HPV positive oropharyngeal squamous cell carcinomas have favorable prognoses in contrast to those with HPV negative OPSCC.24

Secondary or associated conditions and complications

  • Pain
  • Lymphedema of the neck, face, or upper extremities
  • Mucositis
  • Xerostomia
  • Trismus
  • Osteoradionecrosis
  • Dysphonia
  • Dysphagia
  • Facial nerve palsy
  • Other cranial nerve palsy
  • Musculoskeletal neck and shoulder impairments, including cervical dystonia, dropped head/neck extensor weakness, shoulder dysfunction and pain, scapular winging
  • Neuromuscular impairments including myopathy, radiculopathy, plexopathy and neuropathy.
  • Fatigue
  • Radiation fibrosis

Essentials of Assessment

History

A comprehensive history should be taken from an individual with head and neck cancer. An oncologic history, including date of diagnosis, type of cancer, location and extent of cancer and prior surgeries including lymph node removal should be obtained. This history should also include dates and types of chemotherapy along with the total radiation dose, fractions, location and timing of radiation. Functional and social history by a physiatrist should investigate whether impairments with speech, swallowing, pain, difficulty with mouth opening, mouth dryness, nasal stuffiness or discharge, facial swelling, or difficulty with neck or shoulder movement or activities of daily living exist. Physiatrists also need to inquire about issues regarding social isolation, vocational difficulties, home environment and individual support systems.

Physical examination

  • Comprehensive examination of the head, neck and oral cavity, including status of oral cavity/dentition, pharynx and larynx, presence of oral lesions, skin lesions, tongue mobility and strength, masseter and temporalis pain or spasticity, and lymphadenopathy25
  • Interincisor distance should be measured post-treatment. Normal is 35-40 mm.
  • Complete neurological exam, focusing on cranial nerve examination for presence of nerve palsy. The motor and sensory examination need to pay special attention to the face, shoulders and neck.
  • Neck, shoulder, upper extremity musculoskeletal exam, focusing on range of motion for flexion, extension, abduction, rotation
  • Presence of lymphedema of the upper extremities post-treatment

Functional assessment

  • Formal swallowing evaluation
  • Nutritional level and appropriateness of diet should undergo assessment
  • Psychosocial issues need to be addressed given possible disfigurement.

Laboratory studies

Initial work up of head and neck cancer should include a complete blood count and electrolyte analysis. Liver function tests and measurements of nutritional status (such as prealbumin) and thyroid function tests are important to ascertain in pre-treatment planning.25

Patients can be tested for HPV or EBV. Depending on tumor location and extension, biopsy can be performed under local anesthetic or fine needle aspiration of a suspicious lymph node.25

Imaging

Initial imaging studies should include a computed tomography (CT) of the skull base through the diaphragm.16 Magnetic resonance imaging (MRI) can be performed for oral cavity and oropharyngeal tumors and for investigation tumor extension through the laryngeal cartilage.16 Direct visualization of the pharynx and larynx can be achieved with direct laryngoscopy followed by flexible fiberoptic or rigid telescope examination.25

Under the direction of an oncologist, additional imaging studies such as positron emission tomography (PET) scans can be performed for staging.16 Imaging for biopsy with ultrasound or a tracer can also be used when necessary.18

Supplemental assessment tools

Objective measures to assess swallowing can be used in dysphagia or odynophagia, such as fiberoptic endoscopic evaluation of swallowing (FEES) or videofluroscopic swallow study (VFSS). In certain circumstances electrodiagnostic studies, including nerve conduction studies and needle electromyography can be used to evaluate for spinal accessory nerve, facial nerve, or trigeminal nerve impairment and recovery prognosis.

Early predictions of outcomes

Anatomically, the head and neck region consists of several distinct structures and tumor sites. The goal of treating HNC is to maximize disease control, improve survival and limit functional impairment.21 Surgery aims to resect tumor with negative margins while radiation doses can be > 70 Gy to the primary tumor and in lesser amounts after surgery or to other nodal regions.19 Surgery, radiation and chemotherapy, while intended to provide adequate and successful disease control, can result in a number of side effects which will be discussed later.21

Survival is clearly dependent on initial staging of the disease along with primary tumor site and histology. Earlier tumor stages (stage I and II) are associated with better survival in general.21, 26 HPV positive cancers tend to have a better prognosis.27 However, patients with HPV positive neoplasms are at higher risk of later contracting a second primary cancer than those with neoplasm that is not HPV positive.28

Social role and social support system

Most large head and neck oncology programs are associated with support groups. A newly diagnosed head and neck cancer patient will likely benefit from the ability to meet with other patients who have undergone similar treatment programs.

Like most patients with a diagnosis of cancer, head and neck cancer patients undergo a variety of emotions and physical challenges. Surgery, radiation and chemotherapy cause fatigue and additional impairments, including those of cosmesis. During and after treatment, adjustments in societal and familial roles may occur.

After treatment completion of head and neck cancer, the return to work rate is 61.9% of those employed at the time of diagnosis.29 Fatigue was listed as the most frequent reason to not return to work; however, of those who discontinued work after treatment, 40.7% returned to work within one year of treatment.29

Professional Issues

Physiatrists should counsel and educate the patient and family about the disease, its treatment and expected impairments. Advanced directives may need to be discussed depending on prognosis.

Rehabilitation Management and Treatments

Impairment and Treatment Overview

The aim of treatment for HNC is to maximize locoregional control and survival while minimizing functional and cosmetic alteration.21 Surgery and radiation therapy aim to provide curative control while chemotherapy is used as a combined modality treatment.21

The goal of rehabilitation is to reduce the different impairments, which may include:

  • Pain: There is a high prevalence of pain in head and neck cancer patients before and after surgery, radiation and chemotherapy30
  • Odynophagia:31 Many patients experience painful swallowing for up to 3 months or possibly more after treatment.
  • Difficulty chewing32
  • Dysphagia:31 Difficulty swallowing, or fatigue with swallowing can occur pre-treatment or post-treatment with surgery, radiation and chemotherapy, resulting in aspiration
  • Xerostomia: There is a high prevalence after chemotherapy, radiation and surgery resulting in sticky saliva and dysphagia32
  • Mucositis32
  • Impaired dentition:32 tends to significantly worsen by 3 years after chemotherapy and radiotherapy treatment31
  • Decreased or altered sense of taste32
  • Trismus: Reduced mouth opening impairs oral hygiene and speech, nutrition, and other aspects of swallowing.  Trismus was least likely to improve with time after chemoradiation therapy31. Treatment approaches include jaw stretching in therapy, exercises, or with devices.33, 34 Devices such as TheraBite or Dynasplint can also increase mouth opening to various degrees.35, 36
  • Malnutrition: Oral and oropharyngeal cavity cancer patients have high rates of xerostomia, pain, and dysphagia, and in one study 40% of these patients not receiving nutritional supplements had >10% weight loss pre- to post-treatment.37
  • Loss of voice: Commonly seen post-surgically; in one study, 52% of patients were affected32, 38
  • Lymphedema: Increased head, facial and extremity swelling occurs as a result of damage to the lymphatic system during treatment; can treat with manual drainage, massage and bandaging and compression garments.39
  • Speech dysfunction, Dysarthria
  • Chemotherapy-induced peripheral neuropathy39
  • Neck and shoulder dysfunction: Radical neck dissection can involve multiple structures including the sternocleidomastoid, spinal accessory nerve, jugular veins, and anterior and posterior triangle lymphatic groups.39 Neck and shoulder weakness and stiffness can be improved with physical therapy to lengthen muscles, improve range of motion and strength and reduce pain.37 Neck disability due to chemoradiation treatment is also correlated with reduced quality of life.40 
  • Difficulty with ADLs: 30% of patients in one study required assistance with “everyday functioning”,38 which can be treated with occupational therapy.
  • Sarcopenia41
  • Reduced Physical Activity42

Quality of Life and Functional Assessments

There are a number of HNC-related quality of life questionnaires, and no gold standard questionnaire exists; for example, there are 19 questionnaires that address functional status and well-being while 11 questionnaires assessed the treatment-related effects of surgery, chemotherapy and radiation.43

As a result, a recent systematic review recommended that researchers consider the psychometric and study design/objectives when selecting the desired quality of life questionnaire for the head and neck cancer population.43 For example, HNC survivors were found to have ameliorated quality of life with higher levels of physical activity, this was measured with the health-related quality of life (HRQoL) questionnaire.42

Pharmacologic Treatments

While treatment of HNC related pain remains symptom-based, there are several stereotypical pain-generators and causes of discomfort in HNC which can be addressed.  These are xerostomia, trismus and radiation fibrosis, chemotherapy induced peripheral neuropathy (CIPN), and other musculoskeletal pain which may be based on operative positioning, radiation treatments, imaging, as compensation to new oropharyngeal mechanics secondary to oncologic progression or treatment.  To treat xerostomia, saliva production can be augmented with agents such as pilocarpine, cevimeline, and over the counter agents such as Biotene mouthwash may also be used as well.  Trismus and radiation fibrosis pain may be partially prevented by using agents such as vitamin E and pentoxifylline.  Neuropathic pain caused by chemotherapy, tumor invasion into nerves, and nerve compression or traction injuries may be addressed using neuromodulatory agents such as gabapentin, pregabalin, duloxetine, tricyclic antidepressants.  Musculoskeletal pain may be addressed using agents such as NSAIDs, opioids, and alternating acetaminophen and NSAIDs. 

Coordination of care

A multi-disciplinary team should manage each patient individually. Team members should include medical, surgical and radiation oncologists, cancer physiatrists, physical and occupational therapists, speech language pathologists, dentists, social workers, psychologists, vocational rehabilitation, and other specialists as necessary.  Some inconsistencies have been noted in the literature in terms of which patients receive multi-disciplinary care, with some association with patient age and staging of cancer noted. These teams typically consist of surgical oncology, medical oncology, radiation oncology, and occasionally speech pathology.44 The rehabilitation specialist can be instrumental in encouraging and reinforcing physical activity and other rehabilitative-therapeutic activities to augment QOL for patients with HNC. 

Patient & family education

Patients with lower health literacy have been found to have lower levels of self-management behaviors, increased fear of recurrence, and reduced functional HRQOL.45 Patients should be counseled to quit tobacco and alcohol use. Patients and family members should also be educated about the need for follow up, possible impairments, and the rehabilitative strategies available to them for treatment. Patients should also be counseled that higher levels of physical activity are positively associated with improved QOL42 and also associated with improved functional capacity.46

On patient survey, a paucity of patients (only 31%) reported having been counseled about possible shoulder-neck dysfunction.47 Patients may benefit from improved counseling on neck and shoulder dysfunction prior to radiation treatment. 

Support groups can be valuable for peer-to-peer education and support.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Monitored exercise is beneficial in the treatment of cancer-related fatigue and should be recommended to HNC patients. Poor functional performance (<4METs) along with higher overall comorbidity severity, preoperative weight loss and higher TNM tumor stage all corresponded with higher 30 day unplanned readmissions, 90 day medical complications, and worse overall survival rates after surgical treatment of HNC.48

Preoperative assessments of nutritional and functional status as well as BMI and sarcopenia are beneficial in reducing postoperative debility and possibly overall survival as well. Obese and overweight patients had significantly better overall survival compared to normal weight patients; and sarcopenic patients had significantly poorer survival than non-sarcopenic patients.49 Continued evaluation of handgrip strength, skeletal muscle mass with bioelectrical impedance, and Timed Up and Go performance measures along with SARC-F scores to assess for sarcopenia concomitant with chemoradiation therapy could help to guide nutrition and therapeutic exercise plans as sarcopenia was found to develop during CRT.41

Preoperative assessment of cervical, oral and shoulder range of motion and education on exercises can help mitigate postoperative impairments.  Cervical range of motion and swallowing exercises had positive associations with subjective reports of eating though did not correlate with significant changes in penetration or aspiration scores.  Head and neck cancer survivors may perceive improved diet and swallowing skills through these exercises.50

In addressing trismus, while there may not yet be an established optimal intervention, adherence to a particular intervention protocol may positively affect oral aperture. Patients with follow-up reminders had significant improvement in mouth opening measurements.51

Psycho-educational intervention programs with support groups and peer counseling have been suggested by researchers of QOL and HNC.52

Cutting Edge/ Emerging and Unique Concepts and Practice

Constant changes in the approach to cancer patients using targeted-based therapy will impact the impairments seen in the head and neck cancer population and will require adjustments to managing these patients. Minimally invasive surgeries as well as improved radiation strategies will hopefully reduce the numbers of impairments in the HNC population. Facial reanimation surgeries are done in the setting of facial nerve palsy and subsequently require rehabilitation management.

Gaps in the Evidence-Based Knowledge

Preventative methods to reduce impairments resulting from treatments, including surgery, chemotherapy and radiation, require further research.  Study of optimal prehabilitation, nutritional supplementation, rehabilitation concomitant with chemoradiation and ideal timing and dose of therapies would be of great value in addressing treatment-specific complications such as sarcopenia, dysphagia, malnutrition, reduced QOL and function.53 Consistent use of multidisciplinary teams including physiatry and therapeutic services in the longitudinal treatment of patients with HNC is another gap in the literature.

Rehabilitation studies focusing on the impact of rehabilitation interventions on outcomes related to activity and participation (as opposed to impairment-driven interventions) would address a gap in the current evidence.54

Most actionable health literacy components to help improve patient outcomes with HNC in terms of QOL and functional well-being remain unidentified.  How to better meet needs of HNC survivors is a bourgeoning area of research as well.55

Research into prevention and treatments for radiation fibrosis (such as pentoxifylline, vitamin E, and hyperbaric oxygen) would address further gaps in research evidence.

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Original Version of the Topic

Nandita S. Keole, MD. Rehabilitation management of head and neck cancers. 9/20/2013

Previous Revision(s) of the Topic

Christian Custodio, MD, Sasha Knowlton MD. Rehabilitation management of head and neck cancers. 4/3/2017

Author Disclosure

Christian Custodio, MD
Nothing to Disclose

Darcey Hull, DO, MA
Nothing to Disclose