TITLE: Pediatric Head and Neck Tumors
SOURCE: Grand Rounds Presentation, Dept. of Otolaryngology, UTMB
PRESENTATION DATE: January 6, 1999
RESIDENT PHYSICIAN: Kyle Kennedy, M.D.
FACULTY PHYSICIAN: Norman Friedman, M.D.
SERIES EDITOR: Francis B. Quinn, Jr., M.D.

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"This material was prepared by resident physicians in partial fulfillment of educational requirements established for the Postgraduate Training Program of the UTMB Department of Otolaryngology/Head and Neck Surgery and was not intended for clinical use in its present form. It was prepared for the purpose of stimulating group discussion in a conference setting. No warranties, either express or implied, are made with respect to its accuracy, completeness, or timeliness. The material does not necessarily reflect the current or past opinions of members of the UTMB faculty and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion."


Introduction

It is not uncommon for children to present to the otolaryngologist-head and neck surgeon for evaluation of a mass in the head and neck region. The majority of these lesions are inflammatory in nature, but other etiologies include congenital, benign non-inflammatory, benign neoplastic, and malignant neoplastic lesions. This discussion will focus on masses of a proliferative or frankly neoplastic nature. In the pediatric age group, a great many of these pathologic processes are represented by congenital and malignant neoplastic lesions. Some of the tumors, such as hemangioma, are quite common, while others, like Langerhans cell histiocytosis, are rare. It is important for the clinician to have a working knowledge of these pathologic processes in order to determine the clinical significance of a given lesion upon presentation. This knowledge will guide the physician in further evaluation and management of the patient.

The presentation will begin with an overview of the epidemiology of various pediatric head and neck tumors followed by some general observations on the age distribution of these lesions. A patient’s age at presentation with a given head and neck lesion may provide an important clue as to its ultimate etiology. A heightened index of suspicion for a given type of tumor in a particular subset of the pediatric age group may facilitate further evaluation and management. The majority of the presentation will entail a general discussion of features of the various tumors. Again, emphasis will be placed on congenital and malignant neoplastic lesions.

The presentation is intended to highlight features of the more commonly encountered tumors and is not meant to be exhaustive, nor is it meant to encompass all subtleties of management of the various lesions.

 

Epidemiology

Hemangioma is the most common tumor of infancy and childhood and usually becomes evident within the first 6 weeks of life. The lesion is more common in girls than boys by a ratio of 3:1 and is more common in caucasians than blacks. The incidence is approximately 10-12% in both premature infants weighing 1500-2500 grams and full-term caucasian infants by 1 year of age. However, in premature infants weighing less than 1000 grams, the incidence rises to approximately 23%.

Vascular malformations are present at birth. Generally speaking, venous and lymphatic malformations usually become manifest in infancy, while arteriovenous malformations usually become evident later in childhood.

Malignant neoplasms are rare in the pediatric age group, but cancer is second only to trauma as a cause of death in patients aged 1 to 14 years. Malignant lesions are rarely present at birth. Lesions present at birth are most likely congenital or a benign neoplasm. 40% of malignancies are diagnosed in children below the age of 5 years, and primary head and neck lesions account for 5% of these cancers. 70% of head and neck malignancies are represented by lymphoreticular malignancies and soft tissue sarcomas. Overall, neuroblastoma is the most common solid malignant tumor in infants less than 1 year of age. Lymphomas are the most common primary malignancies of the head and neck. In general, non-Hodgkin’s lymphoma occurs more frequently than Hodgkin’s lymphoma, but they occur with approximately equal frequency in the head and neck.

Hodgkin’s lymphoma exhibits a bimodal peak incidence with one peak in the 15-34 year old age group and the other in later adulthood. The male to female ratio is 2:1, and Epstein-Barr virus (EBV) is associated with approximately 40% of cases. There is a higher incidence in developed countries.

The incidence of non-Hodgkin’s lymphoma (NHL) increases throughout life, and the male-to-female ratio is 2 to 3:1. The disease is twice as common in caucasians as it is in blacks. For unknown reasons, there has been an approximately 30% increase in NHL in the United States from 1973 to 1991. The Burkitt’s lymphoma subtype of NHL accounts for approximately 50% of childhood malignancies in equatorial Africa. EBV appears to play a role in the pathogenesis of this disease, and patients with congenital or acquired immunodeficiencies are at an increased risk for development of NHL.

Rhabdomyosarcoma is the most common soft tissue sarcoma of the head and neck region in children. The incidence is approximately 4.5 per 1 million with the peak incidence between ages 2-5 years. Caucasians are more commonly affected than blacks in a ratio of approximately 3:1, and a slight male predominance noted in a male-to-female ratio of 1.4:1. This lesion may be seen in children with a history of retinoblastoma treated with radiation therapy.

Thyroid carcinoma is uncommon and usually presents in children older than 10 years of age. A female predominance is evident. The major risk factor for development of differentiated thyroid carcinoma is exposure to radiation. Differentiated thyroid carcinoma is also associated with Gardner’s syndrome (colonic polyposis) and Cowden syndrome (goiter and skin hamartomas). Medullary thyroid carcinoma is rare. Approximately 25-30% of cases are familial and are inherited as an autosomal dominant trait. Cases may be familial alone or associated with Multiple Endocrine Neoplasia syndromes 2A (medullary thyroid carcinoma, hyperparathyoidism, and pheochromocytoma) or 2B (medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and marfanoid-like features).

Neuroblastoma, as mentioned previously, is the most common solid malignant tumor overall in infants less than 1 year of age. Approximately 90% or more of these lesions are seen in children less than 10 years of age. Most present from birth to 5 years of age with the peak incidence at age 2.

Langerhans cell histiocytosis is rare. In the pediatric age group, it is most commonly diagnosed in children under the age of 2. This is usually the acute disseminated type, formerly known as Letterer-Siwe disease. The chronic disseminated type, formerly known as Hand-Schuller-Christian disease, is seen more often in a somewhat older age group. The chronic unifocal type, formerly known as eosinophilic granuloma, is most often seen in adolescents. There is a male predominance.

 

Age Distribution

A patient’s age at the time of presentation with a head and neck mass is an important consideration in determining the etiology of a particular lesion. The likelihood of a given type of tumor being present may heighten the index of suspicion and help guide the evaluation. The following is a brief, generalized summary of the typical age at presentation for the more commonly encountered malignant neoplasms:

Hodgkin’s lymphoma

adolescents and adults

Non-Hodgkin’s lymphoma

children less than 12 years (preadolescence)

Rhabdomyosarcoma

peaks at ages 2-5 and 15-19 (primarily preschool years)

Thyroid carcinoma

adolescence

Neuroblastoma

first few years of life (neonates very young children)

Langerhans cell histiocytosis

most commonly diagnosed children under 2

Salivary gland carcinoma

adolescence

 

Therefore, rhabdomyosarcoma, neuroblastoma, and Langerhans cell histiocytosis are important considerations in young children. NHL is usually seen in older, preadolescent patients, and Hodgkin’s lymphoma, thyroid carcinoma, and salivary gland carcinoma are most often encountered in adolescents.

 

Congenital Lesions

A wide variety of congenital lesions may present as masses or tumors in the head and neck region. These include vascular anomalies, branchial cleft cysts (BCC), thyroglossal duct cysts (TDC), dermoid cysts, and teratomas. Vascular anomalies are the most frequently encountered and will receive the majority of our attention here. As purely developmental anomalies, BCC and TDC will not be discussed further, and dermoid cysts, teratomas. and congenital muscle torticollis will only be mentioned briefly.

 

Classification

In the past, confusing terminology has made categorization and management of the diverse array of cervicofacial vascular anomalies somewhat difficult. The same term was often used to describe disparate lesions of quite different cellular characteristics and natural history.

In 1982, Mulliken and Glowacki, developed a simplified biologic classification scheme which divides cervicofacial vascular anomalies into 2 broad categories: hemangiomas and vascular malformations. Vascular malformations include slow-flow and fast-flow lesions. The classification is said to be biologic because it attempts to correlate the cellular characteristics of a given lesion with its clinical behavior.

A hemangioma is usually not evident at birth. This lesion lesion undergoes a rapid proliferative phase in early infancy and is characterized by endothelial cellular proliferation. An involutive phase then follows with regression of the lesion. A vascular malformation represents a structural anomaly of vascular morphogenesis. The lesion is present at birth. A normal rate of endothelial cell turnover is evident, and the lesion grows commensurately with the child.

 

Distinguishing Features of Hemangioma and Vascular Malformations

Hemangiomas and vascular malformations differ in a number of areas which allow the clinician to distinguish one from the other in the majority of cases based on a thorough history and physical examination.

As mentioned previously, hemangiomas are usually not clinically evident at birth. They usually present within the first 6 weeks of life and undergo a rapid proliferation in the postnatal period for approximately 8-12 months. This is followed by a slow, progressive involution over approximately 5-8 years. Vascular malformations, however, are present at birth and grow commensurately with the child. They may also expand as a result of secondary causes such as hormonal changes in puberty or pregnancy, infection, trauma, or manipulation from embolization or surgery. It is important to distinguish these changes from the active proliferative phase which occurs in hemangiomas.

Histologically, hemangioma is noted to have endothelial proliferation during the proliferative phase. This is accompanied by multilamination of the endothelial basement membrane and the presence of an increased local number of mast cells. Endothelial proliferation diminishes during the involutive phase, and the proliferative tissues are replaced by fibrofatty tissue. Vascular malformations demonstrate a progressive dilatation of vessels of calibers which have an abnormal mural structure. These vessel are lined by a normal endothelial with a unilaminar basement membrane and the presence of normal numbers of mast cells.

Neonates with large or extensive hemangiomas may experience a life-threatening platelet-trapping or thrombocytopenic coagulopathy known as Kasabach-Merritt syndrome. The syndrome usually occurs in the first few weeks of life, and edema is the typically the earliest sign. Hemorrhage into areas such as the upper aerodigestive tract, gastrointestinal tract, pleural space, or central nervous system may result. It occurs in approximately 1% of cases and is associated with a 30-40% mortality despite treatment. Most patients with arterial and lymphatic vascular malformations demonstrate normal coagulation studies, but large or extensive venous malformations may sometimes result in result in local or disseminated intravascular coagulopathy.

Radiographically, hemangioma consists of a discrete, well-organized lobular mass while vascular malformations consist of vessels of different channel sizes without intervening parenchyma. On ultrasonography or MRI, the flow within proliferative-phase hemangiomas and arteriovenous malformations sometimes makes it challenging to distinguish these lesions. Vascular malformations will have slow or fast-flow characteristics on imaging depending on the type of lesion. Ultrasonography (including Doppler flow studies), MRI, CT, and angiography, usually selected in this order, are the modalities employed to image these lesions.

Hemangiomas do not typically cause any hypertrophy of the bony or cartilaginous craniofacial skeleton, whereas slow-flow vascular malformations may result in distortion of these structures. Fast-flow lesions may result in osseous erosion.

The majority of head and neck vascular anomalies can be diagnosed with a thorough history and physical examination. Having drawn the above distinctions, perhaps the 3 most important historical points to elicit then become:

    1. Was the lesion present at birth?
    2. Has the lesion grown?
    3. Has the lesion gotten smaller?

 

Hemangiomas

Hemangioma is the most common tumor of infancy and childhood and most commonly present in the head and neck region. There is a single tumor in 80% of cases and multiple tumors in the other 20%. Children with visceral involvement usually have multiple cutaneous lesions as well, and patients with cervicofacial involvement may also have lesions involving the airway. Over 50% of children with subglottic hemangioma also have at least one cutaneous lesion in the head and neck region.

Cutaneous lesions differ in appearance by virtue of the depth of involvement. Histologically, it is apparent that these morphologically disparate lesions have all the same features, and confusing terms such as capillary and cavernous no longer have any meaningful clinical usefulness. Most lesions are well-circumscribed, firm, and rubbery. A superficial hemangioma primarily involves the superficial dermis with elevation of the overlying skin a bright red color. Tumors primarily involving the deeper layer of the dermis and subcutaneous tissues are termed deep hemangiomas. These lesions have a bluish color, the overlying skin may be relatively flat and normal in appearance. Lesions with combined superficial and deep involvement exist as well.

Complications of hemangioma may be minor or life-threatening. Obstruction of the visual system may result in amblyopia and failure to develop binocular vision. A seemingly inconsequential periorbital lesion may lead to difficulties depending on its location. Any child with periorbital hemangioma should receive an expedient ophthalmologic examination. Obstruction of the airway may occur with lesions of the subglottis or other locations within the larynx or trachea. Subglottic lesions are usually eccentrically placed in the posterior or posterolateral portion of the subglottic space. Infrequently, the lesions are circumferential. Patients may present with stridor and frank respiratory distress or after a prolonged croup-like illness, and urgent intervention may be necessary. Tumors involving the epidermis may ulcerate and bleed, but minimal intervention is usually all that is required. Patients with numerous or extensive cutaneous hemangiomas and those with visceral involvement are at risk for development of high-output congestive heart failure. Kasabach-Merritt syndrome (platelet-trapping coagulopathy) has been mentioned previously.

Treatment is not indicated for the small, inconspicuous, uncomplicated hemangioma, and the majority of lesions are in this category. Observation for gradual involution and resolution is all that is required in this instance. Approximately 50% of these will resolve by the age of 5 years, 70% by age 7 years, and the remainder by age 10-12 years.

Systemic corticosteroids are the mainstay of medical therapy for complicated lesions. Intralesional steroid administration is an alternative for lesions in aesthetically-sensitive areas such as the eyelid and nasal tip. Interferon may be indicated for those complicated lesions that do not respond to steroids, and particularly those which are life-threatening, such as Kasabach-Merritt syndrome. However, early withdrawal may lead to recurrence. Platelets and blood products are best avoided in this syndrome, as they may worsen the condition. and heparin may cause an increase in growth of the offending hemangioma. Approximately 60% of subglottic hemangiomas respond to steroid therapy, but for those that do not, carbon dioxide laser excision may represent a treatment alternative. However, its use is associated with a 20% incidence of acquired subglottic stenosis. Eccentric lesions may be removed in a single procedure, but circumferential tumors are best managed with staged procedures. In those cutaneous lesions resulting in complication or deformity, laser photocoagulation (superficial hemangioma) or excision (deep hemangioma) may be necessary. Combined lesions may require a combination of the two. Application of the laser may be particularly beneficial early in the proliferative phase. A conservative approach with subtotal or staged excision is usually the most prudent. These excisions often involve removal of excess or redundant residual fibrofatty tissue to alleviate cosmetic deformity.

 

Vascular Malformations

As mentioned previously, vascular malformations are evident at birth and grow commensurately with the child. The lesions may undergo a rapid change in size secondary to other influences such as infection, trauma, hormonal changes, or surgery. Capillary, venular, venous, lymphatic, and combined forms comprise the slow-flow vascular malformations.

Capillary malformations consist of abnormally dilated capillary and venule-sized channels. A representative lesion is the common birthmark or nevus flammeus (port-wine stain). Some authors consider this to be a capillary malformation, while others consider it best termed a venular malformation, as it is composed of postcapillary venules. With time, the lesions usually progress to a dark purple color and may even become thickened, raised, and nodular. Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a port -wine stain in the cutaneous distribution of the trigeminal nerve with angiomatous masses in the underlying leptomeninges. These usually extensive lesions involve the V1 cutaneous distribution and may also extend into the V2 and V3 distributions. This syndrome is not seen in patients with lesions involving the V2/V3 areas alone. Mental retardation and other anomalies may be associated with this syndrome as well. Lesions involving the upper eyelid result in an increased incidence of glaucoma, and prompt ophthalmological evaluation is warranted. Complicated lesions may require laser therapy or surgical excision.

Venous malformations are composed of abnormally dilated venous channels and may take the form of discrete venous masses or extensive, ill-defined lesions. They usually occur in pure form but may also be combined with capillary and lymphatic elements. Dystrophic calcification with phlebolith formation is not uncommon. The lesions are common in the head and neck and often involve the lips or cheeks. They are usually compressible and may expand with jugular venous compression or Valsalva.

Intramuscular and craniofacial intraosseous lesions may be encountered, and the masseter muscle and mandible, respectively, are the most common sites. Intraosseous venous malformations characteristically demonstrate a so-called "soap bubble" appearance on plain films.

Lymphatic malformations are uncommon lesions consisting of dilated lymphatic channels lined with normal endothelial cells. The channel walls contain fibromuscular elements and are of variable thickness. Lymphoid tissue may be present, and there may also be evidence of hemorrhage into the lesion. The lesions vary in morphology from discrete, lobular masses of multiloculated, cystic spaces to extensive, ill-defined lesions infiltrating tissue planes. Lymphangioma and cystic hygroma are older terms which are no longer useful. The misconception still exists that these lesions are capable of recurrence after excision, and this implies a neoplastic growth potential not present in these lesions. However, they can be seen to proliferate into the scar tissue of a previous surgical excision site. Presentation may be quite variable and is based on location and extent of the lesion. Cosmetic deformity and hypertrophy of the craniofacial skeleton may be evident with extensive lesions.

Surgical excision is the treatment of choice for lymphatic malformations, but carbon dioxide laser excision may be appropriate for some lesions, especially those involving mucosal surfaces. For management purposes, lymphatic malformations are arbitrarily divided into 2 categories. Type I lesions are located below the mylohyoid and involve the anterior and posterior triangles. They are usually more discrete and more easily excised. The goal is complete excision at the time of the initial procedure. Type II lesions are located above the mylohyoid and usually involve the oral cavity, lip, and/or tongue. These lesions are usually ill-define and may be quite extensive. Extensive infiltration of tissue planes is the rule, and even subtotal excision may be quite difficult. It is imperative to carefully preserve all adjacent vital structures during any surgical intervention for these otherwise benign lesions. Infection with sepsis, hemorrhage, or airway compression may require special management considerations. Size may fluctuate considerably with upper respiratory infection, and excision may produce expansion of the lesion into other areas which were previously uninvolved.

Fast-flow vascular malformations are considerably less common than slow-flow lesions and are comprised of arteriovenous malformation (AVM) and fistula (AVF). AVM is the more common lesion. Histologically, the lesions are composed of irregular arterial channels with abnormal mural architecture. The venous component of the lesions may exhibit mural hypertrophy or sclerosis. Most of these lesions become clinically evident in later childhood or adolescence. Visibly, there may be an erythematous blush of the skin overlying the lesion with a palpable thrill and/or audible bruit. The patient may complain of pain, ulceration, bleeding, or pulsatile tinnitus. Osseous destruction of the craniofacial skeleton adjacent to the lesion may development, and high-output congestive heart failure is possible with larger anomalies. MRI and angiography are necessary for thorough evaluation. Symptomatic or complicated lesions may necessitate an attempt at surgical excision or selective embolization.

In summary, many, if not most, hemangiomas and vascular malformations of the head and neck require nothing more in terms of management than watchful waiting. Symptomatic or complicated lesions may require medical or surgical therapy, the type and timing of which are dictated by the specific lesion involved. Careful clinical evaluation is usually all that is necessary to arrive at a diagnosis and plan a course of management for the majority of these vascular anomalies.

 

Other Congenital Lesions

Dermoid cysts and teratomas may present as midline neck masses and deserve a brief mention. A dermoid cyst is composed of tissues derived from ectodermal and mesodermal remnants and is usually located in the submental region. It may be confused with thyroglossal duct cyst or ranula, and treatment is by surgical excision.

Teratomas are composed of mature elements from the 3 germ layers as well as immature embryonic tissues and are thought to be derived from misplaced pluripotential primordial germ cells. These lesions, which may be quite large, are semicystic and encapsulated. Cervical lesions are usually present at birth. There is a 20% incidence of malignancy. Ultrasound demonstrates heterogeneous echogenicity. Larger masses may present in the neonatal period with respiratory distress secondary to tracheal compression necessitating urgent or emergent intervention.

Congenital muscle torticollis results from unilateral contracture of the sternocleidomastoid muscle and is thought to arise from fibrosis secondary to venous occlusion. The lesion is usually evident within the first 10 days of life. The head tilts toward the affected side with the chin rotated toward the opposite side. Treatment is by passive stretching exercises.

 

Benign Neoplasms

Benign neoplasms of the head and neck in the pediatric age group are relatively uncommon and are represented by a wide variety of tumors. Thyroid, salivary gland, neural, fatty, and osseous neoplasms may be encountered. Management may require surgical excision.

 

Malignant Neoplasms

Lymphoma: Hodgkin’s Disease and Non-Hodkin’s Lymphoma

Hodgkin’s disease is a lymphoreticular malignancy. The majority of patients present with cervical adenopathy consisting of non-tender, firm, rubbery nodes. Spread typically occurs in an orderly, contiguous fashion. Asymmetric supraclavicular cervical adenopathy is common. 30-40% of patients may have constitutional symptoms of fever, night sweats, and weight loss. Mediastinal involvement may result in compression of the superior vena cava or tracheobronchial tree. Axillary, inguinal, and Waldeyer’s ring involvement is uncommon. Diagnosis requires the histologic identification of Reed-Sternberg cells. The Rye Classification is used to characterize the disease and has prognostic value. Variants with a lower ratio of neoplastic to benign inflammatory cells have a better prognosis, and a higher ratio denotes the opposite. Lymphocyte-predominant, mixed-cellularity, nodular-sclerosing, and lymphocyte-depleted forms are recognized. Excisional lymph node biopsy is usually necessary to arrive at a diagnosis. Various imaging and laboratory studies, as well as staging laparotomy, may be required to accurately stage the disease. The Ann Arbor System is employed to stage the disease and plan therapy. Stage I indicates involvement of a single lymph node group or extralymphatic site. Stage II denotes involvement of 2 or more sites on the same side of the diaphragm. Stage III consists of involvement of sites on both sides of the diaphragm. Stage IV represents disseminated disease. Most patients have Stage II or III disease at the time of diagnosis. External beam radiation therapy is used to treat early stage disease, whereas combination chemotherapy is employed to treat advanced disease. 10-year survival is approximately 90% for early stage disease, and long-term disease-free survival is at least 50% for advanced disease.

Non-Hodgkin’s lymphoma in children usually present with rapidly proliferating extranodal disease in the head and neck and mediastinum and may be seen to spread widely in a rapid fashion. Constitutional symptoms may be present. Approximately two-thirds of the patients have locally advanced or disseminated disease at initial presentation. Pancytopenia usually indicates bone marrow involvement, and mental status changes may signify involvement of the CNS. NHL is classified as low, intermediate, or high-grade. Burkitt’s lymphoma is a subtype of NHL with a rapid proliferative potential. The approach to diagnosis and staging of NHL is similar to that of Hodgkin’s disease. Systemic therapy is the primary method of treatment due to the frequent presence of early hematogenous dissemination and advanced disease. Radiation therapy is usually reserved for emergency management of complications involving the airway, CNS, or vascular system. There is an excellent prognosis for patients with early-stage disease. Treatment protocols and prognosis vary more for patients with respect to histologic subtype in patients with advanced-stage disease. Long-term disease free survival with advanced disease may approach 50-85%, but approximately 30% of patients relapse or do not achieve a first remission.

 

Soft Tissue Sarcomas

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The head and neck is the most common site of occurrence and is involved in approximately 35-40% of the cases. RMS is an aggressive mesenchymal tumor. The 3 main histologic types are embryonal, alveolar, and pleomorphic. There is also a botryoid variant of embryonal RMS. The embryonal form is the most common type encountered in the head and neck.

Orbital involvement is most common, and parameningeal sites (nasopharyngeal, sinonasal, aural) are commonly involved. The parameningeal sites carry a worse prognosis. Recognition may be delayed due to the benign nature of many of the initial complaints and findings, such as sinonasal symptoms or recurrent otitis media. Approximately 50% of the tumors presenting in the ear have neurologic complications, especially of cranial nerve VII. Staging depends on surgical excision. Stages I indicates complete surgical excision. Stage II denotes residual microscopic disease. Stage III represents gross residual disease. Metastatic disease is present in Stage IV. Most tumors are Stage III when initially diagnosed.

Multimodality therapy is the mainstay of treatment. Aggressive chemotherapy and radiation therapy are the primary modalities. Radiation therapy may be provided after chemotherapy or in the initial treatment, particularly if CNS or cranial nerve involvement exists. Surgery plays a role in the initial biopsy and diagnosis and for attempted radical resection of residual or recurrent disease following primary chemoradiation. Complete initial resection is desirable only if it may be performed without sacrifice of vital structures or function. The Intergroup Rhabdomyosarcoma Studies have demonstrated improved overall survival with this multimodality approach.

Non-rhabdomyosarcoma sarcomas are rare. The incidence of RMS is at least equal to that of all of these lesions combined. The diagnostic approach for these lesions is essentially the same as it is for RMS. Wide local excision is the treatment of choice followed by chemoradiation for residual/recurrent and metastatic disease.

Neuroblastoma is a malignant tumor of the sympathetic nervous system, and the precursor cells are of neural crest origin. It is the most common malignancy in children under 1 year of age, but few of these patients present with head and neck primary lesions. Most patients have metastatic disease at initial presentation, and cervical metastatic disease is more common than primary cervical disease. Upper aerodigestive tract compressive signs and symptoms, Horner’s syndrome, or heterochromia iridis may be evident. Bony metastases are common. A thorough initial evaluation should include assessment of chest, abdomen, and skeleton. Chemistries should include urinary catecholamines. Complete surgical excision is the treatment of choice with chemoradiation reserved for residual/recurrent and metastatic disease. Overall, complete excision of lesions in children less than 1 year of age yields a 90% survival rate, and primary head and neck lesions have a somewhat better prognosis.

Thyroid carcinoma usually presents as an asymptomatic anterior neck mass. The mass is typically firm and mobile. Fixation of the mass to adjacent tissues, pain, hemoptysis, hoarseness, and vocal cord paralysis are ominous findings. In children, up to 40% of solitary thyroid nodules may harbor carcinoma. Approximately three-quarters of the patients have palpable cervical lymph node metastases at presentation. Most tumors represent differentiated thyroid carcinoma, and papillary carcinoma is the most common type. The presence of various physical stigmata may suggest the presence of a Multiple

Endocrine Neoplasia syndrome, and in this instance, the evaluation should include assessment for urinary catecholamines and hypercalcemia. Fine needle aspiration biopsy may assist in diagnosis, but excisional biopsy may be necessary. Near-total or total thyroidectomy with or without neck dissection are indicated when the presence of malignancy is comfirmed. Appropriate post-surgical follow-up and monitoring is essential. Prognosis for differentiated thyroid carcinoma is good, especially with complete resection and the absence of regional or distant metastatic disease.

Nasopharyngeal carcinoma in children is rare. The tumors are categorized based on the World Health Organization classification as WHO I (keratinizing squamous), WHO II (non-keratinizing squamous), and WHO III (undifferentiated). There is a strong, but not thoroughly proven association with EBV. Most tumors in children are undifferentiated, and children typically present with cervical lymph node metastasis.

Distant metastatic disease at the time of presentation is not uncommon. Nasal obstruction may be present, as well as headache, otitis media, or cranial nerve palsies. Treatment includes radiation therapy along with adjuvant chemotherapy for distant metastatic disease. Overall 5 year survival is approximately 40%.

Langerhans Cell Histiocytosis (LCH) is a term emcompassing a spectrum of disorders which all share the feature of abnormal proliferation and dissemination of Langerhans cells. These mononuclear cells are normally found in the skin and play a role in various immune functions and modulation. Birbeck granules, or cytoplasmic inclusion bodies, are seen in the lesional cells with the aid of electron microscopy and are used as a marker of the disease. LCH may be further subdivided into disease categories which are restricted or extensive based on the degree of organ system involvement and organ function. Historically, LCH was once commonly known as Histiocytosis X and was composed of the following subtypes: eosinophilic granuloma (chronic unifocal form), Hand-Schuller-Christian disease (chronic disseminated form), and Letterer-Siwe disease (acute disseminated form). A number of patients with Hand-Schuller-Christian disease may display the classic triad of multiple calvarial osteolytic lesions (geographic skull), exophthalmos, and diabetes insipidus. In the head and neck, otologic involvement is common, with otitis media being the most common manifestation. Lesions may be encountered within the temporal bone and result in swelling over the mastoid and erosion of the overlying skin. External auditory canal granulation tissue, otorrhea, hearing loss (usually conductive), and facial nerve paralysis may occur. With jaw involvement, gingival pain and swelling, loose teeth, and fracture may be seen. Treatment is by surgical excision (including curettage), radiation therapy, chemotherapy, or a combination of these modalities.

Salivary gland carcinoma in children is rare. The parotid is most commonly involved. Excluding vascular tumors, 50% of salivary gland tumors in children are malignant. These lesions are often seen in patients that have survived previous malignancies and have received radiation therapy to the head and neck region. Mucoepidermoid carcinoma is the most common histopathologic type in children and are usually low-grade. Acinic cell carcinoma is the second most frequent malignancy. Treatment is by excision.

 

Guidelines for Further Evaluation and Biopsy

Most lymphadenopathy or masses in the head and neck are the result of an inflammatory, and it may be difficult to distinguish inflammation from neoplasia on clinical grounds. Constitutional symptoms of fatigue, weight loss, night sweats, and fever are concerning. A persistent, asymptomatic, firm, rubbery, non-tender mass larger than 3 cm with rapid growth and fixation to adjacent tissues in a supraclavicular or posterior triangle location are all suspicious findings.

Distinguish normal cervical masses in children including: styloid process, greater cornu of hyoid and thyroid cartilage, lateral processes of C2 and C6, mastoid tip, and mandible.

Some general guidelines on what constitutes an abnormal head and neck mass are helpful:

Most lesions are inflammatory, and the majority of children receive an initial 2 week trial of oral antibiotic therapy. Biopsy of an otherwise asymptomatic head and neck mass in a child should be considered under the following circumstances:

Biopsy may be open or by fine needle aspiration cytology. Cultures should be obtained with any biopsy procedure. With open biopsy for suspected lymphoma, the specimen should be transported in a fresh condition, and the pathologist should be alerted to the suspected diagnosis. The method chosen will be dictated by the lesion, and additional evaluation, such as imaging and laboratory work may be indicated as well. A chest roentgenogram should be obtained in essentially all patients to evaluate for mediastinal adenopathy which may result in airway compromise upon induction of general anesthesia. Careful follow-up and re-evaluation is mandatory, even with a negative or non-diagnostic biopsy, if the index of suspicion for possible malignancy is high.

 

Conclusions

Most pediatric head and neck masses are inflammatory, congenital, or neoplastic. A careful history and physical examination will help distinguish most of these lesions, especially hemangioma and vascular malformations. Primary head and neck malignancies account for approximately 5% of childhood cancers. Evaluation and management are dependent on histologic type and extent of disease. The clinician should maintain a high index of suspicion when dealing with a potential malignancy, and careful follow-up of children with suspicious head and neck masses is mandatory.


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