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 660,000 new cases each year of head and neck cancer affecting the oral cavity, lip, salivary glands, oropharynx, nasopharynx, hypopharynx, and larynx, and over 325,000 deaths in 2020 due to these cancers.2 Males have a much higher incidence of these cancers and mortality rates from these cancers than do females.2,3
Head and neck cancer accounts for slightly less than 3% of all cancers in the United States, and less than 2% of cancer deaths.4 Classically, head and neck cancer was usually diagnosed in older adults whom have a history of heavy alcohol and tobacco use. Globally head and neck cancers have been decreasing due to decreasing rates of tobacco use; but, there has been an increase in the cases of HPV-associated oropharyngeal cancer mainly in younger generations in North America and Northern Europe.4 HPV has been found to make up 71% of oropharyngeal cancers in the United States.1 The use of the current prophylactic HPV vaccine is better delineated for anogenital and cervical cancers than for oropharyngeal cancers; where a potential decrease in incidence of oropharyngeal cancers may not be seen until after 2060. Despite the rising incidence of HPV-associated oropharyngeal cancer, it does carry a more favorable prognosis as it responds better to treatments such as chemotherapy and radiotherapy when compared to HPV-negative oropharyngeal cancers.4
The incidence of head and neck cancer diagnosis increases after the age of 50.2 Men are more than two to four times as likely to be diagnosed with head and neck cancer compared to women.2 The 5-year survival rate for oral cavity and pharynx cancers is 64%, while it is 61% for larynx cancers. Most cancer recurrences and cancer-related deaths occur after the first 2 to 3 years after diagnosis, while those reaching survivorship after 5 years are generally considered cured.5
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.6 Roughly 70% of oropharyngeal cancers are caused by HPV in the United States,7 and HPV related HNC has augmented survival rates as compared to non-HPV related HNC.11 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.Tobacco products and cigarette smoking have a known dose-response relationship contributing to the development of head and neck cancers. Alcohol and tobacco use have a multiplicative relationship for the development of head and neck cancer.5 Certain types of human papillomavirus (HPV), especially HPV type 16, are responsible for over 50% of oropharyngeal cancers.2,4
Additional risk factors that have been identified include
- Premalignant lesions such as leukoplakia and erythroplakia, and possibly lichen planus8
- Inherited conditions such as Fanconi anemia, Li-Fraumeni syndrome, ataxia telangiectasia and Bloom’s syndrome8
- Epstein Barr virus in nasopharyngeal carcinomas11
- Occupational exposure such as formaldehyde in nasopharyngeal carcinomas11
- Genetics/Family history of HNC12
- Low body mass index11
- Higher number of sexual partners1
- Use of betel quid1
- Socioeconomic status (Low educational levels and low income)1
There are no current screening tests for head and neck cancers that are routinely used where testing should be performed by the primary Oncologist to make shared treatment decisions.
Preventative methods for head and neck cancer include
- Diet, including increased fruit and vegetables
- Good oral hygiene
- Tobacco cessation
- Public and professional education
- Vaccination against HPV to prevent Oropharyngeal Cancer1
Pathophysiology
Most HNC are pathologically identifiable as squamous cell tumors that are positive for keratin.11 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.11 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.11
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.11
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. Common symptoms and signs of head and neck cancer include:
- Hoarseness11
- Sore throat11
- Stridor11
- Dysphagia11
- Dysphonia11
- Neck mass11
- Ear pain or effusion11
- Nasal congestion11
- Oral, non-healing ulcer11
The most common metastases sites are bone, lung and liver.11
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.11 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.4,24 Staging is important for prognostication, such that all patients in the same prognostic group should have similar survival rates.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 lymphadenopathy14
- 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.14
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.14
Imaging
Initial imaging studies should include a computed tomography (CT) of the skull base through the diaphragm.11Magnetic resonance imaging (MRI) can be performed for oral cavity and oropharyngeal tumors and for investigation tumor extension through the laryngeal cartilage.
Under the direction of an oncologist, additional imaging studies such as positron emission tomography (PET) scans can be performed for staging.11 Imaging for biopsy with ultrasound or a tracer can also be used when necessary.11 Continued imaging for surveillance with a PET is usually performed 8 to 10 weeks following treatment to decrease the amount of false positive uptake due to post-treatment inflammation.11
Supplemental assessment tools
Objective measures to assess swallowing can be used in dysphagia or odynophagia, such as fiberoptic endoscopic evaluation of swallowing (FEES) or videofluoroscopic 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.4 Surgery aims to resect tumor with negative margins while resulting in a total lower dose of radiotherapy to the primary tumor to help mitigate adverse treatment related events.4 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.4,11
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.4,11 HPV positive cancers tend to have a better prognosis.4 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 Greater than 60% of patients with SCC of the neck have an initial presentation of stage III-IV disease. As this is more advanced, it has a 15-40% chance of high-risk local recurrence and distal metastasis; as well as having an overall poor prognosis <50% five year survival rate.4,11
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. A systemic review looking at employment outcomes of head and neck cancer survivors post-treatment identifying factors associated with their return to work. It has been found that rates of return to work among survivors varied from 32% to 90%, with factors such as professional roles and supportive environments positively influencing return to work. Some of these factors included employer and co-worker support, work flexibility, meaningful work, paid sick leave, and job security.16
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.11 Surgery and radiation therapy aim to provide curative control while chemotherapy is used as a combined modality treatment.11
The goal of rehabilitation is to reduce the different impairments, which may include
- Pain
- Odynophagia
- Difficulty chewing
- Dysphagia:
- Xerostomia
- Mucositis
- Impaired dentition
- Decreased or altered sense of taste
- Trismus
- Malnutrition
- Loss of voice
- Lymphedema
- Speech dysfunction, Dysarthria
- Chemotherapy-induced peripheral neuropathy
- Neck and shoulder dysfunction
- Difficulty with ADLs
- Sarcopenia
- Reduced Physical Activity
Quality of life and functional assessments
There are a number of HNC-related quality of life questionnaires, and no gold standard questionnaire exists. Due to the great variation of quality of life and functional assessments available; it is best practice to find which tool best serves the population you are evaluating and fits best into your clinical and/or research needs.
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.17 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.18 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.
Due to the complex nature of head and neck cancer, these patients might also need further treatment such as feeding tubes, tracheostomies, or complicated treatment plans. Competent care of these delivery systems requires in-depth training and resources for patients, families, and caregivers, which can be negatively impacted by social risk factors. For example, inadequate health literacy has been associated with heightened distress among head and neck cancer patients. This deficiency can affect their ability to comprehend the risks and benefits of treatments and hinder effective symptom management.11
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
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. This is still an area that effects many patients, and needs further research aimed at improving these gaps of care.
Research into prevention and treatments for radiation fibrosis (such as pentoxifylline, vitamin E, and hyperbaric oxygen) would address further gaps in research evidence.
References
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- Gormley, M., Creaney, G., Schache, A. et al. Reviewing the epidemiology of head and neck cancer: definitions, trends and risk factors. Br Dent J 233, 780–786 (2022). https://doi.org/10.1038/s41415-022-5166-x
- Bray FF, Ferlay J, Soerjomataram I, Siegel RL, Torre LA, Jemal A. Global cancer statistics 2018: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries (vol 68, pg 394, 2018). Ca-a Cancer Journal for Clinicians. 2020 Jul 1;70(4):313-.
- Chow LQM. Head and Neck Cancer. N Engl J Med. 2020 Jan 2;382(1):60-72. doi: 10.1056/NEJMra1715715. PMID: 31893516.
- Du E, Mazul AL, Farquhar D, Brennan P, Anantharaman D, Abedi-Ardekani B, Weissler MC, Hayes DN, Olshan AF, Zevallos JP. Long-term Survival in Head and Neck Cancer: Impact of Site, Stage, Smoking, and Human Papillomavirus Status. Laryngoscope. 2019 Nov;129(11):2506-2513. doi: 10.1002/lary.27807. Epub 2019 Jan 13. PMID: 30637762; PMCID: PMC6907689.
- Liao, Caesar, Chan, Richardson, Kapp, Francoeur, Chan. HPV associated cancers in the US over the last 15 years: Has screening or vaccination made any difference?. J Clin Oncol 2021 39:15_suppl, 107.
- National Cancer Institute, HPV and Cancer. https://cancer.gov/about-cancer/causes-prevention/risk/infectious-agents/hpv-and-cancer. Accessed: June 24, 2022
- Shaw R, Beasley N. Aetiology and risk factors for head and neck cancer: United Kingdom National Multidisciplinary Guidelines. The Journal of Laryngology & Otology. 2016 May;130(S2):S9-12.
- Tobias JS. Current issues in cancer: Cancer of the head and neck. Bmj. 1994 Apr 9;308(6934):961-6.
- Johnson S, McDonald JT, Corsten MJ. Socioeconomic factors in head and neck cancer. Journal of Otolaryngology–Head & Neck Surgery. 2008 Aug 1;37(4).
- Locati L, Lim SH, Patel S, Pfister DG. Evaluation and treatment of head and neck cancer. In: Stubblefield MD, O’Dell MW, eds. Cancer Rehabilitation Principles and Practice. New York, NY: Demos; 2009:291-301.
- Osazuwa-Peters N, Graboyes EM, Khariwala SS. Expanding indications for the human papillomavirus vaccine: one small step for the prevention of head and neck cancer, but one giant leap remains. JAMA Otolaryngology–Head & Neck Surgery. 2020 Dec 1;146(12):1099-101.
- Glastonbury CM. Critical changes in the staging of head and neck cancer. Radiology: Imaging Cancer. 2020 Jan;2(1).
- Hamoir M, Poorten VV, Chantrain G, Van Laer C, Gasmann P, Deron P. Initial work-up in head and neck squamous cell carcinoma. B ENT. 2005 Jan 1:129.
- Shen J, Zhou H, Liu J, Zhang Z, Fang W, Yang Y, Hong S, Xian W, Ma Y, Zhou T, Zhang Y. Incidence and risk factors of second primary cancer after the initial primary human papillomavirus related neoplasms. MedComm. 2020 Dec;1(3):400-9.
- Zecena Morales C, Lisy K, McDowell L, Piper A, Jefford M. Return to work in head and neck cancer survivors: a systematic review. J Cancer Surviv. 2023 Apr;17(2):468-483. doi: 10.1007/s11764-022-01298-6. Epub 2022 Nov 17. PMID: 36396907.
- Hansen CC, Egleston B, Leachman BK, Churilla TM, DeMora L, Ebersole B, Bauman JR, Liu JC, Ridge JA, Galloway TJ. Patterns of multidisciplinary care of head and neck squamous cell carcinoma in medicare patients. JAMA Otolaryngology–Head & Neck Surgery. 2020 Dec 1;146(12):1136-46.
- Clarke N, Dunne S, Coffey L, Sharp L, Desmond D, O’Conner J, O’Sullivan E, Timon C, Cullen C, Gallagher P. Health literacy impacts self-management, quality of life and fear of recurrence in head and neck cancer survivors. Journal of Cancer Survivorship. 2021 Dec;15(6):855-65.
- Sindhar S, Kallogjeri D, Wildes TS, Avidan MS, Piccirillo JF. Association of preoperative functional performance with outcomes after surgical treatment of head and neck cancer: a clinical severity staging system. JAMA Otolaryngology–Head & Neck Surgery. 2019 Dec 1;145(12):1128-36.
- Fattouh M, Chang GY, Ow TJ, Shifteh K, Rosenblatt G, Patel VM, Smith RV, Prystowsky MB, Schlecht NF. Association between pretreatment obesity, sarcopenia, and survival in patients with head and neck cancer. Head & neck. 2019 Mar;41(3):707-14.
- Silbergleit AK, Schultz L, Krisciunas G, Langmore S. Association of neck range of motion and skin caliper measures on dysphagia outcomes in head and neck cancer and effects of neck stretches and swallowing exercises. Dysphagia. 2020 Apr;35(2):360-8.
- Chee S, Byrnes YM, Chorath KT, Rajasekaran K, Deng J. Interventions for trismus in head and neck cancer patients: a systematic review of randomized controlled trials. Integrative cancer therapies. 2021 May;20:15347354211006474.
- Bhardwaj T. Quality of Life of Head and Neck Cancer Patients: Psychosocial Perspective using Mixed Method Approach. Indian Journal of Palliative Care. 2021 Apr;27(2):291.
- Loewen I, Jeffery CC, Rieger J, Constantinescu G. Prehabilitation in head and neck cancer patients: a literature review. Journal of Otolaryngology-Head & Neck Surgery. 2021 Dec;50(1):1-1.
- Parke SC, Langelier DM, Cheng JT, Kline-Quiroz C, Stubblefield MD. State of Rehabilitation Research in the Head and Neck Cancer Population: Functional Impact vs. Impairment-Focused Outcomes. Current Oncology Reports. 2022 Feb 19:1-6.
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
Christian Custodio, MD, Darcey Hull, DO, MA. Rehabilitation Management of Head and Neck Cancers. 6/30/2022
Author Disclosure
Toure Barksdale, MD
Nothing to Disclose