Head and neck cancer

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]

Head and Neck cancer Microchapters
Patient Information
Overview
Classification Brain tumor Oral cancer Nasopharyngeal cancer Hypopharyngeal cancer Glomus tumor Salivary gland tumor Laryngeal cancer Thyroid cancer Parathyroid cancer Esophageal cancer
Causes
Differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]

Classification

• Head and neck cancers comprise of a group of malignancies arising from the oral cavity, pharynx and larynx, paranasal sinuses, nasal cavity or salivary glands with squamous cell carcinoma representing the most common histology.

'Head and neck squamous cell carcinomas (HNSCC's) make up the vast majority of head and neck cancers, and arise from mucosal surfaces throughout this anatomic region. These include tumors of the nasal cavities, paranasal sinuses, oral cavity, nasopharynx, oropharynx, hypopharynx, and larynx.

Oral cavity

Main article: Oral cancer

Squamous cell cancers are common in the oral cavity, including the inner lip, tongue, floor of mouth, gingivae, and hard palate. Cancers of the oral cavity are strongly associated with tobacco use, especially use of chewing tobacco or "dip", as well as heavy alcohol use. Cancers of this region, particularly the tongue, are more frequently treated with surgery than are other head and neck cancers.

Surgeries for oral cancers include

• Maxillectomy (can be done with or without Orbital exenteration
• Mandibulectomy (removal of the mandible or lower jaw or part of it)
• Glossectomy (tongue removal, can be total, hemi or partial)
• Radical neck dissection
• Moh's procedure
• Combinational e.g. glossectomy and laryngectomy done together.

The defect is covered/improved by using another part of the body and/or skin grafts and/or wearing a prosthesis.

Nasopharynx

Nasopharyngeal cancer arises in the nasopharynx, the region in which the nasal cavities and the Eustachian tubes connect with the upper part of the throat. While some nasopharyngeal cancers are biologically similar to the common HNSCC, "poorly differentiated" nasopharyngeal carcinoma is distinct in its epidemiology, biology, clinical behavior, and treatment, and is treated as a separate disease by many experts.

Surgeries for nasal cancer (cancer of the nose)

• Surgery to removal the entire nose or part of the nose. Removal of all of the nose is called a total rhinectomy, for part of the nose it is called a partial rhinectomy. Afterwards to cover the defect, a new nose can be made by using another part of the body and/or a nose prosthesis is made.

Oropharynx

Oropharyngeal cancer begins in the oropharynx, the middle part of the throat that includes the soft palate, the base of the tongue, and the tonsils. Squamous cell cancers of the tonsils are more strongly associated with human papillomavirus infection than are cancers of other regions of the head and neck.

Hypopharynx

The hypopharynx includes the pyriform sinuses, the posterior pharyngeal wall, and the postcricoid area. Tumors of the hypopharynx frequently have an advanced stage at diagnosis, and have the most adverse prognoses of pharyngeal tumors. They tend to metastasize early due to the extensive lymphatic network around the larynx.

Larynx

Laryngeal cancer begins in the larynx or "voice box." Cancer may occur on the vocal cords themselves ("glottic" cancer), or on tissues above and below the true cords ("supraglottic" and "subglottic" cancers respectively). Laryngeal cancer is strongly associated with tobacco smoking.

Surgeries can include partial laryngectomy (removal of part of the larynx) and total laryngectomy (removal of the whole larnyx). If the whole larynx has been removed the person is left with a permanent tracheostomy opening and learns to speak again in a new way with the help of intensive teaching and speech therapy and/or an electronic device.

Also anyone who has had a glossectomy (tongue removal) will be taught to speak again in a new way and have intensive speech therapy

Trachea

Cancer of the trachea is a rare malignancy which can be biologically similar in many ways to head and neck cancer, and is sometimes classified as such.

Most tumors of the salivary glands differ from the common carcinomas of the head and neck in etiology, histopathology, clinical presentation, and therapy, Other uncommon tumors arising in the head and neck include teratomas, adenocarcinomas, adenoid cystic carcinomas, and mucoepidermoid carcinomas. Rarer still are melanomas and lymphomas of the upper aerodigestive tract.

History and Symptoms

Throat Cancer usually begins with symptoms that seem harmless enough, like an enlarged lymph node on the outside of the neck, a sore throat or a hoarse sounding voice. However, in the case of throat cancer, these conditions may persist and become chronic. There may be a lump or a sore in the throat or neck that does not heal or go away. There may be difficult or painful swallowing. Speaking may become difficult. There may be a persistent earache. Other possible but less common symptoms include some numbness or paralysis of the face muscles.

Presenting symptoms include:

• A lump or sore that does not heal
• A sore throat that does not go away
• Trouble swallowing
• A change or hoarseness in the voice

Other symptoms may include the following:

• Oral cavity. A white or red patch on the gums, tongue, or lining of the mouth; a swelling of the jaw that causes dentures to fit poorly or become uncomfortable; and unusual bleeding or pain in the mouth.
• Nasal cavity and sinuses. Sinuses that are blocked and do not clear, chronic sinus infections that do not respond to treatment with antibiotics, bleeding through the nose, frequent headaches, swelling or other trouble with the eyes, pain in the upper teeth, or problems with dentures.
• Salivary glands. Swelling under the chin or around the jawbone; numbness or paralysis of the muscles in the face; or pain that does not go away in the face, chin, or neck.
• Oropharynx and hypopharynx. Ear pain.
• Nasopharynx. Trouble breathing or speaking, frequent headaches, pain or ringing in the ears, or trouble hearing.
• Larynx. Pain when swallowing, or ear pain.
• Metastatic squamous neck cancer. Pain in the neck or throat that does not go away.

These symptoms may be caused by cancer or by other, less serious conditions. It is important to check with a doctor or dentist about any of these symptoms.

Differentiating head and neck cancer from other diseases

Head and neck cancer must be differentiated from congenital abnormalities, and malignant lesions.

Category	Diseases		Benign/ Malignant	Clinical manifestation						Paraclinical findings			Gold standard diagnosis	Associated findings
				Demography	History	Symptoms		Signs		Lab findings	Histopathology	Imaging
						Pain	Dysphagia	Mass exam	Others
Neoplasm	Salivary gland neoplasm	Pleomorphic adenoma	Benign	More common in females Incidence increase with age Incidence: 2-3.5 cases per 100,000 population	History of swelling Dysphagia Hoarseness	−	+	Palpable mass of deep lobe of parotid gland Firm Mobile	−	−	Proliferation of epithelial cells and stromal matrix in the ducts	MRI: Homogenous on T1 Abundant myxochondroid stroma on T2	Biopsy	−
		Warthin's tumor	Benign	Male to female ratio: 4:1 More common in people aged 60-70 years old	History of swollen salivary gland Jaw pain Tinnitus	−	+	Non tender Mobile Firm Solitary	−	−	Papillae Fibrous capsule Cystic spaces	Neck CT: Cystic lesion posteriorly within the parotid gland MRI: B/L heterogeneous lesions	Biopsy	−
		Oncocytoma	Benign	Race: Caucasian patients predilection Gender: No gender preference Age: 50–70 years	Growing palpable painless mass Facial swelling Lymphadenopathy (if transformed to malignant)	±	±	Firm, multilobulated Mobile mass	Normal Redness Swelling Skin ulceration	Normal Anemia	Epithelial cells with eosinophilic Granular cytoplasm Rich in mitochondria	CT: Isodense expansive mass Enhancement after intravenous contrast Hypodense areas MRI: Isodensties on T1 Mass is hyperintense on T2 Enhancement on contrast	Incisional biopsy and histopathological examination	-
		Monomorphic adenoma	Benign or malignant	Age: 26-76 years Rare in children Gender: No predilection	Growing palpable painless mass on jaw or in oral cavity Facial swelling Lymphadenopathy (if transformed to malignant) Pain and ulceration (in later stage)	±	±	Nodular Fluctuant swelling	Normal Redness Skin ulceration May have lymphadenopathy	Normal	Straw colored fluid on aspiration	Ultrasound: Used to biopsy the lesion May show cystic an solid components CT: useful for lesions with calcification and venous phleboliths MRI: Test of choice Differentiate benign from malignant Defines tumor extent Shows perineural spread	Incisional biopsy and histopathological examination	-
		Mucoepidermoid carcinoma	Malignant	Age: Mean age of 59 Female predilection	Painlesss mass Swelling in oral cavity Lymphadenopathy	±	±	Cystic and solid mass	May have lymphadenopathy	−	Gross findings: Firm Tan-white to yellow Bosselated Cystic Microscopic findings: Encapsulated squamous and glandular components	Cystic and solid component with variable appearance on CT and MRI	Incisional biopsy and histopathological examination	Association with CMV
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings
Neoplasm	Salivary gland neoplasm	Adenoid cystic carcinoma	Malignant	Age: 40s-60s Gender: Female predominance	Slow growing rare tumor with low recurrence	±	±	Solid mass	Normal to ulcerated lesions May have lymphadenopathy	−	Gross findings: Tubular Cribriform Solid pattern of growth Microscopic findings: Components of large cells with pleomorphic nuclei Increased mitotic activity Focal necrosis	Imaging reveal dimensions of the tumor, local spread, and distant metastasis	Biopsy and histopathological examination	−
		Adenocarcinoma	Malignant	Age: young age predilection	Its a tumor of minor salivary glands May present as small ulceration or nodules in oral cavity	−	−	Small nodules in oral cavity With or without lymphadenopathy	Normal to ulcerated lesions May have lymphadenopathy	May be normal Or may show anemia and blood cell disorders with distant bone invasion	On histology it is confused with Adeocyctic carcinoma Components of gland and cyst formations More perineural invasion	CT and MRI MRI being more accurate for adjacent tissue involvement and lymphadenopathy	Biopsy and histopathological examination	−
		Salivary duct cancer	Malignant (Highly aggressive)	Incidence: 1-3% Gender: Male predilection Mean age: 55-61 years old	Rapidly growing mass with jaw involvement	±	±	Painless Hard Non-compressible mass	Ulceration of mucosa and skin May have lymphadenopathy facial paralysis in case of facial nerve involvement	Pathomorphologically tumor of salivary ducts resembles tumor of breast ducts	Gross findings: Firm mass Cystic component of variable size and dimension Microscopic finding: Resembling ductal carcinoma of breast Intraductal components invading surrounding tissue in several forms: cribriform papillary Solid with comedo-like central necrosis	Non-specific features on CT and MRI Shows neural and jaw involvement	Biopsy and histopathological examination	−
		Squamous cell carcinoma	Malignant	Incidence: rare Age: Old age , 61-68 years Male predilection	Present as painful growing mass on jaw	+	−	Teneder Firm Solitary swelling on jaw	Submandibular gland predilection Thinning and discoloration of skin	Past radiation exposure is a strong risk factor	Gross findings: Thinning of skin Microscopically findings: Nest and solid sheets of tumor cells arranged in glandular pattern Immunohistochemical staining can be used to mark the squamous and keratin component	Tumor dimension can be delineated using both CT and MRI	Biopsy and histopathological examination	−
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings
Neoplasm	Hypopharyngeal cancer		Malignant	More common in males Age: 55-65 years old Incidence: < 1/100,000 in U.S. More common in Japan, India, Iran	Tobacco use Abuse alcohol consumption HPV infection Lump in the neck Odynophagia Hoarseness	−	+	Non tender cervical node	Lymphadenopathy	−	Spindle cells Nuclear atypia Basaloid cells Abundant chromatin	Neck CT scan: Soft tissue mass Irregular thickening of mucosa Necrotic region MRI: Tumors are hypointense on T1 and hyperintense on T2	Biopsy	−
	Parathyroid cancer		Malignant	Incidence: Rare Mean age : 44-54 years old Gender: Female predilection	Presents with the hyperparathyroidism Bone pains Abdominal pain Nausea and vomiting Fatigue Confusion	+	+	Lower neck mass	Tachycardia Weight loss Sweating Neck swelling	Low TSH Increased T4 and T3 Hypercalcemia	Microscopic findings: Trabecular growth pattern High mitosis Surrounding thick fibrotic bands Capsular involvement Vascular invasion is common	CT and MRI shows more frequent lower lobe involvement, vascular involvement , lymph node metastasis, and perineural involvement Bone scan may show decreasing bone density	Biopsy and histopathological examination	Hereditary syndromes MEN-2B syndrome Neurofibromatosis type 1 VHL disease
	Carotid body tumors		Benign	Age: 26-55 years Male predominance	A slow growing neck mass	+	−	Mobile Non-tender neck mass (horizontally more than vertically) Pulsatile Bruit may be present	Change in voice Dizziness Tinnitus Headache	Rasised catecholamine levels	Microscopically they are extra-adrenal paragangliomas	Doppler ultrasound, CT, MRI and angiography is used to visualize the tumor Metaiodobenzylguanidine (MIBG) testing	Histopathology analysis and catecholamine levels	−
	Paraganglioma		Benign (Majority) Malignant (rare)	Age 50-70 years More in females	May be an accidental finding depending on their secretory nature or present with following symptoms: Palpitation Tremor Pulse-like vibratory sense Headache Change in voice Vertigo Catecholamine secreting paragangliomas presents with : Hypertension Headache Sweating Tachycardia	−	−	No visible mass Located deep in the the neck along the glossopharyngeal nerve	−	Biochemical testing may show catecholamine metabolites in serum or urine samples	Highly vascular tumors that involves nerves around vessels Gross findings: Fleshy tumor Pink to red-brown to gray in color Associated with hemorrhage or fibrosis Microscopic findings: Round or polygonal cells Nests or trabecular structures inside the capsule	Ultrasound Computed tomography Magnetic resonance imaging Angiography metaiodobenzylguanidine (MIBG) 18F-fluoro-2-deoxyglucose Positron emission tomography (FDG-PET)	Imaging and serum catecholamine analysis	−
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings
Neoplasm	Schwannoma		Benign	Rare tumor Incidence: 1-10%	Slow growing mass Localized neural deficit depending on the site of peripheral nerve involved Vagus nerve or superior cervical sympathetic chain involvement (most common locations)	+	±	Multiple Slow growing nodules on the skin	Vagal involvement: Hoarseness Dysphagia Sympathetic nerve involvement may present as Horner's syndrome: Dilated pupil Decrease sweating Dropping eyelid Vestibular nerve involvement & Hearing impairment (most common)	May be normal	Encapsulated neural tissue growth	Resembling Carotid body tumor on CT MRI and MRI angiography confirm the diagnosis	Imaging	neurofibromatosis type II
	Lymphoma		Benign or malignant	Age: Predilection for older age Mean age: 55	Insidious onset slow growing lymph nodes Non-specific systemic B symptoms: Fever Night sweats Weight loss) Rash Waxing and waning lymphadenopathy Abdominal fullness	−	±	Multiple chain lymphadenopathy Hepatosplenomegaly Ascites Crackles heard on chest auscultation	Rash and pruritus	Pancytopenia Hypercalcemia Hyperuricemia (increased cell turnover) Monoclonal immunoglobulin (M-spike) Raised LDH levels	On complete node analysis four patterns are described: Nodular/follicular Diffuse pattern Transition from a nodular to a diffuse pattern in adjacent nodes Transition from a lower to a higher grade of involvement within a single node	Imaging to stage the disease Positron emission tomography with computed tomography is preferred over MRI	Lymph node biopsy coupled with cytometry	Infections due to cytopenias With acquired form of C1 inhibitor deficiency patients may develop angioedema
	Liposarcoma		Malignant	Rare tumor Age: Relatively in older age Gender: No gender predilection	Mobile mass Few symptoms until they grow enough to compress the surrounding structures Symptoms of neural deficit, pain, tingling, or skin changes	±	−	Mobile soft mass Intact overlying skin Blue discoloration due to intra-lesion hemorrhage	Intact skin and normal color	Normal	Gross examination: Bulk of yellow colored fat tissue Microscopic features: Adipose tissue containing lipoblasts Atypical nucleus pushed to side by intracytoplasmic vacuoles Tissue biopsy may show histological sub-groups: Well-differentiated Myxoid/round cell Pleomorphic liposarcomas	Imaging not usually required for diagnosis May show deeper invasion Ultrasound shows homogeneous hyperechoic mass	Biopsy and histopathology analysis	−
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings
Neoplasm	Lipoma		Benign	Genetic predisposition Unspecific gender or age association	One or multiple soft, painless skin nodules May causes pain or compressive symptoms	±	−	Mobile soft nodule Intact overlying skin	Normal	Normal	Diagnoses is usually clinical Tissue biopsy may show: Bundle of well-demarcated lipocytes Single nuclei aligned to the side Intra-cytoplasimic fat granules	Diagnosis is usually clinical ultrasound is used to differentiate lipoma from other benign lesions such as: Epidermoid cyst A ganglion	Clinical evaluation	Multiple lipomas Associated with familial multiple lipomatosis
	Glomus vagale, glomus jugulare tumors		Benign	Rare tumor	Painless slowly enlarging mass in the neck	−	±	Non-compressible Firm Non-tender swelling No thrill or bruit Normal overlying skin	Secretory tumors May have compressive signs such as: Dysphagia Hoarseness Cranial nerves deficits Horner's syndrome	Normal	Glomus tumors arise from Non Chromaffin cells histopathology reveals "salt and pepper" chromatin On immunohistochemistry tumor cells show chromogranin and S-100 positivity	MRI (Imaging of choice): Typical appearance of the tumor along vagus nerve Ultrasonography (early stage of diagnosis): Isoechoic to hypoechoic well defined tumor CT: Vascularity of the tumor Biochemical testing: Shows secretary nature of the tumor	Imaging and Metaiodobenzylguanidine (MIBG) testing	−
	Metastatic head and neck cancer		Malignant	Depends on the nature of metastatic tumor	Asymptomatic Painless lymphadenopathy Supra clavicular fullness in case of stomach cancer metastasis	−	±	Non-tender mass in the neck Non-tender lymphadenopathy	Majority of metastatic head and neck cancer Metastatise from GIT and lungs	Vary depending on the underlying cancer	Histology of primary cancer	CT and MRI to see extent of tumor	Biopsy and histopathology of the primary site of tumor	−
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings
Other	Laryngeal cancer		Benign/Malignant	Older males Younger patients with HPV infection or smoking history	Neck mass Hoarseness Throat pain Snoring Obstructive sleep apnea	±	±	Examination of neck and oral cavity may show : mass lymphadenopathy Examination of laryngeal cancer is done using flexible laryngoscopy under anesthesia.	Smoking is the most common risk factor Smoking with alcohol increases the risk Oropharyngeal cancers presenting with neck masses are associated with human papillomavirus (HPV) infection	HPV testing may show HPV infection	FNA of neck mass Followed by biopsy of laryngeal cancer Show type cancer cells	CT, MRI and PET are used to see local infiltration by cancer Also to see distant metastases. Panendoscopy is done to see extent of the tumor.	Laryngoscopy and biopsy	−
	Arteriovenous fistula		Benign/Malignant	Depends on the risk factors	Expanding neck mass Headaches Dizziness Neurological sequels	−	−	Pulsating neck mass Bruit	May be associated with vasculopathies metastatic invasion of vessels and neck surgery		Varies depending on the etiology	MR angiography	MR angiography	−
	Thyroid nodule/ Goiter		Benign/ Malignant	Female predominance Young age (benign causes) Old age (malignant etiology)	Growing painless neck mass in front of neck Weight loss Palpitation Hoarseness Irritability	±	±	Painless Non-tender Asymmetrical neck mass in front of neck With smooth overlying skin Nodular surface Depending on the type: May be mobile Adherent to the underlying structure Lymphadenopathy in case of malignant features	Goiter is most commonly associated with iodine deficiency	Normal to low TSH levels in case of malignancy High TSH levels in case of goiter	FNA is done in case of goiter Core biopsy is performed if malignancy is suspected	USG: Shows nodular or non- nodular lesions in Thyroid US is better than CT. Thyroid radionuclide imaging: Shows radioiodine uptake Cold in case of malignancy Cold or hot in case of goiter.	Biopsy and histopathology of nodules	−
Category	Diseases		Benign	Demography	History	Pain	Dysphagia	Mass exam	Others	Lab findings	Histopathology	Imaging	Gold standard diagnosis	Associated findings

Primary Prevention

• Avoidance of recognised risk factors (as described above)is the single most effective form of prevention. Regular dental examinations may identify pre-cancerous lesions in the oral cavity.
• It will be interesting to see what effect the widespread use of HPV vaccines has on the incidence of HPV-related H&N cancers.
• People who have been treated for head and neck cancer have an increased chance of developing a new cancer, usually in the head and neck, esophagus, or lungs. The chance of a second primary cancer varies depending on the original diagnosis, but is higher for people who smoke and drink alcohol. Patients who do not smoke should never start. Those who smoke should do their best to quit. Studies have shown that continuing to smoke or drink (or both) increases the chance of a second primary cancer for up to 20 years after the original diagnosis.
• Some research has shown that isotretinoin (13-cis-retinoic acid), a substance related to vitamin A, may reduce the risk of the tumor recurring (coming back) in patients who have been successfully treated for cancers of the oral cavity, oropharynx, and larynx. However, treatment with isotretinoin has not yet been shown to improve survival or to prevent future cancers.

Case Studies

Case#1

Related chapters

• oral cancer
• cancer of the larynx
• thyroid cancer
• adenoid cystic carcinoma - a type of salivary gland cancer
• Burkitt's lymphoma - a type of lymphoma that affects the head and neck
• Dermatofibrosarcoma protuberans - a type of sarcoma that may involve the head and neck
• Hodgkin's disease - a lymphoma that often involves the lymph nodes in the neck
• paraganglioma - usually found in the head and neck region
• skin cancers - may involve the head and neck
• Bobby Hamilton - a NASCAR driver who died of head and neck cancer

External Links

• Head and Neck Cancer - Learn more from MedlinePlus
• Head and Neck Cancer Information
• Head and Neck Cancer: Questions and Answers
• Head and Neck Cancer: Treatment
• RadiologyInfo - The radiology information resource for patients: Head and Neck Cancer

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