Head and Neck Tumors

The presence of tumors in the neck is common. These are abnormal lesions that are located under the jaw, above the clavicle and deep in the skin. They may be visible, palpable, or seen on imaging studies.

Discerning the pathology underlying these tumors is usually not easy and can be quite problematic. They can develop from infectious, inflammatory, congenital, traumatic diseases, benign or malignant processes.

Unlike children, where the most common cause is infection, in adults, the most common cause is malignancy. In fact, there is abundant literature suggesting that the persistence of a tumor in the neck in an adult should be considered malignant until proven otherwise. Therefore, in adults, further investigation is paramount because it may be the only manifestation of a head and neck malignancy.

The location of the mass, imaging findings, and historical information are important to differentiate the etiology.

A complete medical history can provide important information for the diagnosis of a neck mass. Key details to determine are:

• Age – Provides important information about possible causes. It is one of the most significant predictors of malignancy.

• Characteristics of the tumor : information related to the duration, growth pattern, and presence of pain may provide clues to the cause.

• Associated symptoms : the presence of hoarseness, stridor, dysphagia, odynophagia, otalgia and epistaxis suggest cervical metastases from a primary malignant neoplasm of the upper aerodigestive tract. Doctors should also ask about systemic symptoms and classic “B signs” of lymphoma, which include fever, chills, night sweats, and unintentional weight loss.

• Social history : includes tobacco use (amount, duration, and method), alcohol use, intravenous drug use, contact with animals, and recent travel.

A complete examination of the head and neck can provide additional details about the cause. Key components include:

> Characteristics of the tumor

•  Size

•  Location

•  Quality (soft, fluctuating, rubbery, firm)

•  Mobility (mobile, hypomobile or immobile)

•  Sensitivity

•  Skin changes

> Head and neck examination

•  Skin : evaluate the face and scalp for lesions, ulcerations, erythema.

•  Oral cavity and oropharynx : examine tonsils, palate, posterior pharynx, tongue, tongue mobility, oral mucosa and gum. Dentures or other dental appliances should be removed. Take into account the presence of erythema, ulcerations, decreased movements or asymmetries. Palpation of these structures may reveal occult lesions.

•  Nose: examine the external part of the nose, nasal mucosa, septum and turbinates and evaluate the sensitivity of the paranasal sinuses.

•  Ear: evaluate decreased hearing and the presence of effusions through otoscopic examination.

•  Larynx: palpation during swallowing and evaluation of laryngeal crepitation may reveal underlying pathology

> Cranial nerves

Suspected malignancy requires images to be requested. The 2 main imaging modalities are those performed with contrast, computed tomography (CT) or magnetic resonance imaging (MRI):

> CT : it is the most commonly used imaging modality for head and neck tumors and is the initial diagnostic test of choice for patients with a persistent tumor in the neck. CT has several advantages including wide availability, rapid acquisition, and low cost. It is an excellent initial imaging test due to its ability to characterize the tumor in relation to other structures of the head and neck and evaluate the involvement of the cervical deep spaces. Although CT uses ionizing radiation, it is considered acceptable in the adult population.

>  MRI is similar to CT and allows precise tumor localization and can accurately characterize tumors and inflammation. Although both studies are effective for oncologic evaluation, MRI provides superior visualization of soft tissues and possible perineural extension.

The advantages of MRI are the lack of radiation exposure and image quality. It is reserved for patients with dental restorations such as crowns, posts or implants. However, MRI is more expensive, difficult for patients with claustrophobia, takes longer (about 30 minutes), and is excluded in patients with certain implantable medical devices such as a pacemaker. Regardless of the imaging modality selected, the addition of contrast is essential unless there is a contraindication, such as an allergy to the substance or kidney failure. There is rarely any additional benefit to ordering a non-contrast scan and therefore it should be avoided.

Contrast improves tumor characterization, maps edges, and may better identify the relationship of the neck mass to major vessels. Another frequently used diagnostic modality is ultrasound, which represents the least invasive imaging technique and can provide real-time evaluation of the tumor. Furthermore, it allows image-guided sampling and can adequately characterize benign, vascular, inflammatory, and malignant lesions, and is the gold standard for evaluating the thyroid. However, there are several disadvantages to using this tool.

Ultrasound is limited because its evaluation of the deep spaces of the neck is highly operator dependent. Therefore, it is not recommended as a first option. The few exceptions to this are delay in obtaining a CT or MRI, contraindication to the use of contrast, or the need for an adjunct to expedite a fine-needle aspiration biopsy (FNAB).

When the diagnosis of a cervical tumor remains uncertain, a biopsy should be performed. FNA represents the gold standard and should be the initial test for histological evaluation. FNAC is a procedure by which a small-gauge needle (25 or 27) is inserted into the tumor to obtain a sample. small.

It can be done without presurgical evaluation and does not expose patients to the risks of anesthesia. It can be performed with and without ultrasound or tomographic guidance. It is highly accurate, safe, cost-effective and provides a timely diagnosis with lower morbidity compared to open biopsy. Although FNAB of neck lumps is very accurate, some results may not provide a definitive answer. This may occur because there is not enough lesion material for the pathologist to make a diagnosis. In this case, it will report “inadequate sample.” Another reason is when there is enough sample but the cells obtained do not provide a specific diagnosis.

In both situations, if the patient presents signs and symptoms suspicious of malignancy or has a persistent growth in the neck, a repeat FNA should be attempted before resorting to an open biopsy. If FNA results are inadequate or indeterminate and do not assist in the diagnosis, a core needle biopsy may be considered. This biopsy is usually performed under local anesthesia, using a larger gauge needle compared to FNA (14-18 gauge).

Core needle biopsy may also be preferred in suspected cases of lymphoma, as it allows greater appreciation of tissue architecture. However, core needle biopsies increase the possibility of trauma from the use of larger gauge needles, in addition to increasing the risk of tumor seeding. The latter is the reason why it is contraindicated in patients with suspected squamous cell carcinoma.

On the other hand, open biopsy is the definitive way to obtain a diagnosis. It consists of making an incision in the neck and removing the entire tumor or just a part; which is done under at least local anesthesia, and is often done in an operating room. Because it is more invasive than FNA, it should be reserved for those when FNA has failed to provide a diagnosis or the pathologist requires more tissue.

Certain laboratory tests may be useful and ordered based on clinical suspicion of a specific disease.

Common causes of a neck growth in adults can be classified into 6 main categories:

> Congenital

•  Thyroglossal duct cyst: represents the most common congenital anomaly in the head and neck and, although it is more common in children, remains may remain in 7% of adults. This malformation can develop anywhere from the base of the tongue to the thyroid gland. Most commonly, it presents as a midline cyst near the hyoid bone that rises with tongue protrusion or swallowing. These tumors can be observed or surgically removed through the Sistrunk procedure, which involves removing the cyst along with a portion of the hyoid bone.

•  Branchial cleft cyst : it can arise from any of the first, through the fourth pharyngeal cleft. Similar to the thyroglossal duct cyst, they are usually present at birth but become evident or symptomatic in childhood. Rarely, these cysts can persist into adulthood and are often discovered when they become tender, enlarged, or inflamed after an upper respiratory infection. They can also become infected and cause purulent drainage into the skin or pharynx. Treatment consists of surgical excision.

>  Venolymphatic malformations:

•  Cystic hygroma (lymphangioma): This is a benign congenital anomaly of the lymphatic system that occurs most commonly in children. It rarely occurs de novo in adults. It can occur anywhere on the head and neck as a painless, soft, fluctuating growth. The cause is unknown, but it is likely due to acquired processes such as infection, surgical manipulation, or lymphatic obstruction. These tumors can be observed or treated with sclerotherapy or surgery.

•  Hemangioma: vascular malformation that is usually present at birth and proliferates rapidly in the first years of childhood, but eventually regresses. Sometimes patients may have residual telangiectasias, scarring, or atrophy, which presents as a tumor and may need treatment. There are several therapeutic options: laser therapy, sclerotherapy or surgery.

•  Venous malformations: arise from abnormal and ectatic venous channels and are often present in the head and neck. Similar to hemangiomas, they may be present at birth. However, they tend to grow as the patient ages, without spontaneous resolution. Depending on their size, architecture, location, and flow rate, they may be asymptomatic or cause significant morbidity, with pain, discomfort, life-threatening bleeding, or respiratory compromise. Current treatment strategies include surgery, laser therapy or sclerotherapy.

•  Pseudoaneurysm or arteriovenous fistula: may occur as a result of sharp or penetrating trauma to the neck and presents as soft, throbbing pain, thrill, or murmur. These malformations are potentially lethal and require prompt treatment to prevent rupture or neurological dysfunction. The standard treatment in the past was surgical repair and ligation of the carotid artery. However, today, endovascular techniques with stent grafts have evolved as effective options.

>  Infections

•  Viral infection: There are a variety of viral agents that can cause lymphadenopathy. The most common are those that cause upper respiratory infections and include rhinovirus, coronavirus, and influenza virus. The resulting lymphadenopathy usually resolves within 3 to 6 weeks after resolution of symptoms.

•  Bartonella henselae : is the etiological agent of cat scratch disease and classically infects from a bite/scratch from an infected cat. Patients may develop a bulbous or vesicular lesion at the inoculation site, followed by ipsilateral lymphadenopathy in the cervical, inguinal, or axillary region. Treatment usually involves a 5-day course of azithromycin.

•  Tuberculous cervical lymphadenitis: tuberculosis of the lymph nodes is one of the most common extrapulmonary manifestations of the disease. It may be caused by tuberculous or nontuberculous mycobacteria and may be seen in immunocompromised patients or those who have recently traveled to endemic regions. It usually presents as a chronic, painless cervical tumor, without apparent signs of infection. This finding may be accompanied by other constitutional signs of tuberculosis, such as night sweats, chills, and involuntary weight loss.

> Benign neoplasms .

•  Lipomas: are benign subcutaneous masses of mesenchymal origin that can occur in the head and neck region. They are usually smooth, mobile and asymptomatic. They can be observed or surgically removed.

•  Thyroid nodules: they are common and can be found in 65% of the population. Most are benign and are found incidentally on imaging studies. The gold standard for evaluating a thyroid nodule is ultrasound. Ultrasound results are typically reported based on the Thyroid Imaging Reporting & Data System (TI-RADS) Management score. Management of the thyroid nodule depends on its size in combination with the TI-RADS score. From this score, observation or FNA can be indicated. FNAC results are typically reported using the Bethesda Classification.

> Malignant neoplasms

•  Malignant neoplasm of the upper aerodigestive tract: malignant neoplasms in the oral cavity, nasopharynx, oropharynx, rhinosinusal cavity, hypopharynx and larynx can metastasize to the neck. The most common malignancy is squamous cell carcinoma, which is usually caused by alcohol, smoking and, more recently, the human papillomavirus. Its presentation in the head and neck caused by smoking and alcohol is quite different from that caused by papillomavirus. Squamous cell carcinoma caused by tobacco and alcohol presents as a painful tumor in the upper aerodigestive tract, along with other symptoms: dysphagia, odynophagia, voice changes, otalgia. If the cause is papillomavirus, it appears only as a painless tumor. Most of them have no other symptoms and are often confused with a gill cleft cyst. Management of upper aerodigestive tract cancers depends on location and stage.

•  Thyroid cancer: the most common type is papillary. Other thyroid cancers are follicular, medullary and anaplastic. These cancers have a very good prognosis, with the exception of anaplastic carcinoma. Treatment of thyroid cancer typically involves a thyroidectomy and may include adjuvant radioactive iodine therapy based on the pathology.

•  Cancer of the salivary glands: they can originate in the main glands (parotid, submandibular and sublingual) or in minor salivary glands (located throughout the upper digestive system). The management of these cancers involves surgery followed by adjuvant therapy depending on the pathology.

•  Lymphoma: Cervical lymphadenopathy is one of the most common manifestations of lymphoma. It can generally be classified as Hodgkin lymphoma and non-Hodgkin lymphoma. The former involves the cervical lymph nodes, while non-Hodgkin lymphoma can spread to extranodal sites, such as the major salivary glands, paranasal sinuses, and Waldeyer’s ring. Imaging findings do not help differentiate these 2 forms. Management involves chemotherapy and sometimes the addition of radiotherapy.

•  Metastasis from thoracoabdominal malignancy: Sometimes malignancies of the abdomen and chest can metastasize to a supraclavicular lymph node, known as Virchow’s node. Management depends on the malignancy.

> Systemic diseases

•  Sjögr syndrome: is an autoimmune disease that commonly affects older women. It presents with dry eyes and mouth and persistent enlargement of the submandibular or parotid gland. Patients may have elevated levels of antineutrophil antibodies and rheumatoid factor, as well as anti-Ro/SS-A or anti-La/SSB antibodies. However, these antibodies are not specific for the syndrome. Treatment focuses on symptom control, including topical tear replacement for xerophthalmia and oral hygiene to increase saliva flow.

•  Sarcoidosis: This inflammatory syndrome is characterized by the development of granulomas, leading to permanent scarring or thickening of the organ tissue. Signs and symptoms depend on the location of the granulomas, and up to 10-15% of patients may have head and neck manifestations. In many cases, sarcoidosis resolves on its own, but there are several therapies such as steroids, immunosuppressants, and antimalarials that can control symptoms and prevent further destruction.

•  Other autoimmune diseases: Several conditions, including rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and vasculitis, may have manifestations in the head and neck, which may be the only presenting feature. Classifying the specific type of autoimmune disease requires laboratory testing, and management depends on the type of disease.

Management of a neck lump depends on the underlying cause. As the most common cause is infection, it is valid to first undergo antibiotic treatment and then re-evaluate after 2 weeks. If there is no appropriate resolution or recurrence occurs, further evaluation is warranted.

There are several causes of neck masses, and their differential diagnosis can be difficult. Using a systematic approach will result in an accurate diagnosis and guidance for appropriate treatment. A careful history and physical examination can provide important clues regarding the diagnosis and also dictate the need for evaluation with imaging, tissue biopsies, and necessary referrals. The most important cause to rule out is malignancy. In cases where a diagnosis is not reached, patients should be monitored closely. If the tumor does not resolve or recurs, repeat testing and/or referral to a specialist should be considered.