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Flashcards in 55 Pediatric Head & Neck Tumors Deck (21):
1

What are the categories of neck masses in children that are important when creating a differential diagnosis?

What are the categories of neck masses in children that are important when creating a differential diagnosis?

  • Congenital neck masses are those that are present at birth and secondary to defects occurring in embryology.
  • Infectious neck masses are those that present due to an infection and typically resolve with treatment of the infection. These are most commonly infected or reactive lymph nodes, but can also occur in other tissues in the head and neck such as the salivary glands.
  • Inflammatory masses that do not have a known infectious cause, such as those associated with Kawasaki’s disease.
  • Neoplastic lesions of the neck including benign and malignant processes. These encompass malignant lymphadenopathy, benign and malignant salivary gland tumors, benign and malignant thyroid tumors, and tumors originating from neurologic, muscular, vascular, lymphatic, cartilaginous, or osseus tissues.
  • Vascular malformations (see Chapter 54).

2

What is the most common neck mass in a child?

What is the most common neck mass in a child?

An enlarged lymph node is the most common reason that a child presents with a neck mass. The most common cause of enlarged lymphadenopathy is infection, either viral or bacterial. Viral causes of lymphadenopathy include adenovirus, rhinovirus, and enterovirus, which can all occur with a viral upper respiratory infection. Epstein-Barr virus (EBV) causes mononucleosis, which consists of cervical lymphadenopathy, exudative tonsillitis, and hepatosplenomegaly.

Bacterial causes of an enlarged lymph node most commonly include infections due to Staphylococcus aureus and Streptococcus pyogenes. Sometimes, the infected lymph node can suppurate and create a neck abscess. Other significant causes of bacterial lymphadenitis are atypical mycobacterium, Bartonella henselae (cat-scratch disease), and tuberculosis.

3

What presenting features suggest an acute infectious cause of a neck mass?

What presenting features suggest an acute infectious cause of a neck mass?

Fever, pain, acute swelling, erythema of the overlying skin, decreased neck range of motion, and odynophagia can indicate that a neck mass is secondary to an infectious cause. Concomitant upper respiratory tract symptoms, exposure to sick contacts, foreign travel, exposure to animals (cats, ticks), and the presence of immunodeficiency are all important historical features that can allow better understanding of the etiology of a neck mass.

4

What other type of neck lesions can present with an acute infection or inflammation?

What other type of neck lesions can present with an acute infection or inflammation?

Congenital lesions including thyroglossal duct cysts, dermoids, branchial cleft cysts, vascular malformations, and preauricular cysts can often present with acute swelling, erythema, pain, and fevers. The preferred treatment of these infectious exacerbations is antibiotic therapy. Incision and drainage should be performed only if necessary because they may complicate the definitive resection of the congenital mass. The resection of a congenital lesion is more easily accomplished after complete resolution of the infection.

5

What congenital neck masses occur in the midline neck?

What congenital neck masses occur in the midline neck?

Thyroglossal duct cyst is the most common congenital neck mass. Thyroglossal duct cysts occur in the midline due to incomplete obliteration of the thyroglossal duct. The median thyroid anlage starts at the foramen cecum of the tongue and migrates caudally in the neck until it reaches its final anatomic position near the cricoid cartilage. The thyroglossal duct should obliterate, but occasionally, this process is incomplete. A cyst can then form at that location with a tract that connects the cyst to the foramen cecum. Ectopic thyroid tissue can occur anywhere from the foramen cecum to the normal position of the thyroid gland.

Dermoid cysts are benign cystic structures that can occur anywhere in the body. They frequently occur in the head and neck and can occur in the midline neck and mimic a thyroglossal duct cyst. Other common locations include the nose, oral cavity, orbit, and nasopharynx. Dermoid cysts arise due to entrapment of epithelial cells along lines of fusion. They usually contain other skin appendages including sebaceous glands, hair, or hair follicles. They can often be adherent to the overlying skin and may even have a small draining sinus.

Teratomas are similar to dermoids, with the exception that they contain cell types of ectodermal, mesodermal, and endodermal origin. They may present as a firm neck mass and can cause respiratory symptoms when they are very large. Treatment requires complete surgical excision.

Laryngoceles occur as midline neck masses when they herniate through the thyrohyoid membrane (external laryngocele). If confined to the larynx, it is an internal laryngocele and will not likely present as a mass. Symptoms include hoarseness, dysphagia, and severe dyspnea, particularly when presenting in a neonate.

6

What is the significance of the Sistrunk procedure used to resect a thyroglossal duct cyst?

What is the significance of the Sistrunk procedure used to resect a thyroglossal duct cyst?

The earliest reports of thyroglossal duct cyst excision were plagued by a rate of recurrence as high as 50%. Resection of the hyoid bone along with cyst improved recurrence rates to 20%. Walter Sistrunk expanded that technique to include resection of the cyst, hyoid bone, and suprahyoid tongue musculature to ensure that the tract(s) connecting the cyst to the foramen cecum was adequately resected. This decreased the recurrence rate to near 5%. Removal of the cuff of lingual musculature is important because the tract may pass anterior or posterior to the hyoid and may be multiple.

7

What congenital neck masses occur in the lateral neck?

What congenital neck masses occur in the lateral neck?

Congenital lateral neck masses are most likely to be of branchial origin. Otherwise, congenital lateral neck masses can include vascular malformations and thymic cysts. Branchial anomalies presenting as a mass are most likely to be a cyst with or without a sinus, which may connect it to the skin or to an internal structure. Branchial anomalies are classified by their branchial origin. They can be first, second, third, or fourth branchial anomalies.

The most common branchial anomaly is a second branchial cleft cyst. Second brachial cleft cysts occur in the upper neck and present as a cystic mass anterior to the sternocleidomastoid muscle. They can present as an acute, painful swelling of the neck that follows upper respiratory tract symptoms. It often could be confused with a suppurative lymph node. The differentiation between these two entities can be difficult, but imaging with contrast-enhanced computed tomography may be helpful. The infected cyst will have a circumferential thin wall compared to a thicker wall with more surrounding inflammation of an abscess.

The second most common branchial anomaly is a first branchial cleft cyst. These occur in the infra-auricular region and may occur anterior, inferior, or posterior to the lobule. These can be associated with the facial nerve and resection will require dissection of the facial nerve.

Least common are branchial anomalies of the third and fourth arch which typically present as left sided infections in the lower neck. Thyroiditis may occur in the setting of a fourth branchial cleft cyst as the tract should extend through the thyroid gland. These cysts will often have a connection to the piriform sinus. Proper resection of a fourth arch anomaly requires hemithyroidectomy. Otherwise, recurrence is more likely.

Thymic cysts can occur in the lateral neck as thymic tissue originates from the third branchial arch and descends into the mediastinum via the thymopharyngeal duct. Thymic cysts usually occur in the left neck and can be unilocular or multilocular. The diagnosis is confirmed on pathology with the presence of Hassall corpuscles (concentric epithelioreticular cells and macrophages in the medulla).

Vascular malformations are discussed in greater detail in Chapter 54. These include a range of pathologies that include venous malformation, lymphatic malformation, venolymphatic malformation, and arterovenous malformation. These classically present in the posterior triangle of the neck.

8

How does an atypical mycobacterial infection present?

How does an atypical mycobacterial infection present?

Atypical mycobacterium infections in the neck often present with a mass, which is often firm, nontender, and located in the submandibular or preauricular regions. Classically, there is a violaceous discoloration to the overlying skin. The skin may be thin with areas of fluctuance. Typically, there is no preceding illness, fevers, weight loss, or night sweats. An intradermal tuberculin test will usually be negative or indeterminate. Computed tomography and magnetic resonance imaging may suggest an infectious cause by revealing fat stranding, thick wall of abscess cavity (if present), inflammation of skin, and multiloculated appearance, but they are not diagnostic.

The clinical course may start as a discrete mass in the submandibular or periauricular regions. A course of antibiotics will have no significant effect. The mass may continue to enlarge and it is not uncommon for violaceous skin changes to occur overlying a fluctuant component of the mass. The fluctuant area may progress to the point that the skin breaks down and a draining wound is created. The natural history is that this infection is self-limited and will heal without treatment. Unfortunately, the natural history is not typically expedient and may take many months to a couple of years for resolution.

9

What are the treatment considerations for atypical mycobacterium?

What are the treatment considerations for atypical mycobacterium?

Treatment options consist of observation, medical management with antibiotics, surgical curettage, and surgical excision or any combination of the above. The optimal treatment would be one that eradicates the disease quickly and does so with minimal short- and long-term risk to the patient. Prompt eradication is expected if complete surgical excision is performed. As such, many studies have supported complete excision as the optimal treatment for quicker time to resolution and better cosmetic outcomes. Unfortunately, the disease process often closely encounters the facial nerve or its branches. Complete excision can be associated with temporary nerve injury in approximately 20% to 30% and permanent nerve injury in 2% to 5% of cases. Other outcomes include higher incidence of poor scarring (21%), wound infection (14%), and recurrence (9%). While the risk of permanent injury is low, the risk is significant considering that the disease resolves, albeit more slowly, without surgery.

If complete surgical excision is deemed too risky, other treatment options consist of surgical incision and curettage of the purulent portion of the mass. This can help confirm the diagnosis and may help treat a draining or “about to drain” wound.

Medical therapy with various antibiotics (clarithromycin, azithromycin, ethambutol, and rifabutin) may be helpful and antimicrobial therapy can be utilized alone or in combination with surgical treatment. The use of medical therapy is often debated, however, as there has been no research proving its efficacy over observation. Observation alone allows the disease to take its expectant course and most patients will have resolved the infection by one year.

10

What is the differential for inflammatory, but noninfectious lymphadenopathy?

What is the differential for inflammatory, but noninfectious lymphadenopathy?

Kawasaki disease, also known as mucocutaneous lymph node syndrome, typically occurs in children less than 5 years old. It is considered a vasculitis and presents with high fever for 5 days and four of the following signs: acute cervical adenopathy, nonexudative conjunctivitis, strawberry tongue, lip fissure, rash, erythema of palms and soles, edema of hands and feet, and desquamation. It may cause coronary artery aneurysm and cardiologic evaluation is necessary. Treatment consists of intravenous immunoglobulins and aspirin.

PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and cervical adenitis) causes recurrent high fever in children typically less than 5 years old without signs of concomitant upper respiratory infections. Between episodes, they are likely asymptomatic. Diagnosis must rule out other causes of recurrent fevers and medical treatment can be helpful (steroids and/or cimetidine). Tonsillectomy is often curative.

Castleman disease is giant lymph node hyperplasia that causes concern due to the large size of lymph nodes. Fortunately, it is benign and excisional biopsy can be both diagnostic and curative.

Rosai-Dorfman disease consists of massive lymphadenopathy due to sinus histiocytosis occurring in patients under 10 years old. Diagnosis is made on biopsy of a lymph node. Treatment can be observational, but surgery, radiation, and chemotherapy may also play roles depending on severity of the disease.

Kikuchi-Fujimoto disease is a histiocytic necrotizing lymphadenitis typically occurring in 20- to 40-year-olds and may be unilateral or bilateral. Biopsy of the lymph node is required for diagnosis and resolution occurs in about 6 months.

11

What are reassuring sonographic findings of lymph nodes?

What are reassuring sonographic findings of lymph nodes?

  • Hypoechoic to muscle
  • Flat, oval shaped, short axis : long axis <0.5, but submandibular and parotid nodes may be rounder
  • Echogenic hilum
  • Hilar vascularity seen in reactive or normal node
  • Surrounding edema
  • Sharp margins

12

What are sonographic features of malignant nodes?

What are sonographic features of malignant nodes?

  • Markedly hypoechoic to muscle (except if papillary thyroid cancer)
  • Round shape
  • No echogenic hilus
  • Coagulation necrosis
  • Eccentric cortical hypertrophy
  • Cystic necrosis
  • Ill-defined borders
  • Peripheral or mixed vascularity
  • Calcifications suggest metastatic thyroid cancer
  • No surrounding inflammation

13

What is the most likely malignant pathology type to be found in the head and neck?

What is the most likely malignant pathology type to be found in the head and neck?

Lymphomas (Hodgkin and non-Hodgkin) are the most common malignancy in the head and neck. Enlargement of lymph nodes is very common in children and when it is not reactive lymphadenopathy, lymphoma is the primary pathology that must be considered. Ultrasound can be reassuring when a normal appearing fatty hilum is identified. Otherwise, a persistently enlarged lymph node without resolution in time or antibiotic course may warrant biopsy to investigate further. Fine needle aspiration can often diagnose if a malignancy is present, but usually flow cytometry of a lymph node that has been completely excised is required to make the specific diagnosis that can help clarify the treatment of choice.

14

What is the most likely type of thyroid malignancy in a child?

What is the most likely type of thyroid malignancy in a child?

Papillary thyroid carcinoma is the most common thyroid malignancy in adults and children. Papillary thyroid carcinoma will present with a painless, firm thyroid nodule. Usually the nodule will be cystic and nonfunctioning. Fine needle aspiration is recommend to achieve the diagnosis. Suspicious lymph nodes in the central and lateral neck compartments should be biopsied at the same time. Nodal metastasis is quite common, but overall survival remains high (2.5% mortality). Treatment is most often total thyroidectomy with central and/or lateral neck dissections for biopsy for proven or highly suspicious lymph nodes, followed by radioactive iodine ablation.

15

What are common infectious or inflammatory lesions of salivary glands?

What are common infectious or inflammatory lesions of salivary glands?

Acute bacterial sialadenitis, viral sialadenitis, and juvenile recurrent parotitis. Acute bacterial sialadenitis occurs as unilateral, painful swelling with purulent drainage coming from the salivary duct. Treatment is with hydration, warm compress, massage, and sialogogues. Viral sialadenitis can occur due to coxsackievirus, cytomegalovirus, parainfluenza, or mumps.

16

What pathologies are causes of benign salivary gland tumors?

What pathologies are causes of benign salivary gland tumors?

In children, the most common benign tumors are pleomorphic adenoma occurring in the parotid, submandibular, and minor salivary glands and hemangioma occurring in the parotid gland. Other benign pathologies exist and include Warthin’s tumors (papillary cystadenoma lymphomatosum), basal cell adenoma, myoepithelioma, and lymphoepithelioma.

Hemangioma of the parotid gland, and submandibular gland less commonly, presents at birth or shortly thereafter. Like hemangiomas occurring elsewhere, there is a rapid proliferative phase that occurs in the first few months of life followed by slow involution over a period of one to many years. Treatment of hemangioma classically is to allow involution over time and to medically treat with steroids, interferon, and more recently propranolol for more symptomatic lesions. Surgical resection is also an option, but must be carefully considered due to an increased risk to the facial nerve.

17

What pathologies are the most common causes of malignant salivary gland tumors?

What pathologies are the most common causes of malignant salivary gland tumors?

Mucoepidermoid, acinic cell, adenocarcinoma, adenoid cystic carcinomas, and carcinoma ex pleomorphic are possible salivary malignancies in childhood. Mucoepidermoid carcinoma is the most common malignant salivary mass in children, typically presenting in adolescence. Like benign tumors, treatment is with complete surgical excision with margins if possible. Neck dissection will be required in some malignancies, particularly if the lesion is of higher grade, lymph nodes are clinically suspicious, or there is evidence that the tumor has extraglandular extension or facial nerve invasion. Adjuvant radiotherapy is used as well if there is significant nodal involvement, extraglandular extension, perineural invasion, or incomplete excision. The risks and benefits of radiotherapy must be carefully considered in children due to the risk of second malignancy and other complications of radiation therapy.

18

What is the most common type of sarcoma occurring in the head and neck of children?

What is the most common type of sarcoma occurring in the head and neck of children?

Rhabdomyosarcoma (RMS) is the most common sarcoma occurring in the head and neck region of children. Approximately one third of RMS occur in the head and neck and within the head and neck, the orbit is the most common site. The presenting symptoms will depend on the site of origin for the tumor. Tumors can be classified as parameningeal, which implies that they arise either along the anterior or lateral skull base, or nonparameningeal, occurring in oro/hypopharynx, parotid, external ear, or lateral neck. Ideally, complete surgical resection with wide margins results in the best long-term prognosis. Unfortunately, in the head and neck wide margins are rarely possible without major functional impairment. Thus, the initial treatment involves obtaining the tissue diagnosis. Although fine needle aspiration may identify tumor as a sarcoma, more detailed histologic typing by a pathologist experienced in sarcomas is required to allow proper treatment decisions. Therefore, core or open biopsies are often required. Treatment of head and neck sarcomas will often consist of chemotherapy (directed by the Intergroup Rhabdomyosarcoma Studies [IRS] protocols for rhabdomyosarcomas or Children’s Oncology Group [COG] protocols for nonrhabdomyosarcomas), surgery, and radiation therapy.

19

What are other types of sarcomas?

What are other types of sarcomas?

  • Fibrosarcoma
  • Onrhabdomyosarcoma
  • Hemangiopericytoma
  • Osteosarcoma
  • Chondrosarcoma
  • Extraskeletal Ewing sarcoma
  • Liposarcoma
  • Leiomyosarcoma

20

What are the types of neuroblastic neck masses?

What are the types of neuroblastic neck masses?

Neuroblastic masses include ganglioneuroma, ganglioneuroblastoma, and neuroblastoma. Neuroblastoma is the most common of these lesions and is the third most common malignancy in children. These three pathologies represent different stages of the same disease process, with neuroblastoma being the least differentiated with the most malignant potential and ganglioneuroma being the most differentiated with likely no malignant potential. Typically, these are painless neck masses and symptoms are related to nerve (cranial nerves, sympathetic chain) or aerodigestive tract compression.

21

What is the importance of creating a differential diagnosis as it pertains to investigating a neck mass?

What is the importance of creating a differential diagnosis as it pertains to investigating a neck mass?

Creating an appropriate differential diagnosis for each patient presenting with a neck mass allows judicious consideration for the appropriate diagnostic tests to order, whether it be laboratory assessment or radiographic imaging. The history should dictate which imaging modality should be utilized and this is particularly important when considering computed tomography scans. Through the cumulative effects of radiation exposure, two to three computed tomography scans of the head can increase the risk of brain cancer threefold and 5 to 10 head CTs may increase risk of leukemia by threefold. As such, special consideration should be taken when considering each imaging study.

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