Head and neck masses are classified, based on etiology, as being either inflammatory or infectious, congenital, caused by vasoformative

lesions, or neoplasms. The majority of lesions in children are either inflammatory or congenital in origin, but the possibility of malignancy must always be considered. The wide variety of pathologic processes can be distinguished with an organized diagnostic approach. The history and physical examination provide initial guidance on evaluation. The age, sex, and race help narrow diagnostic possibilities. Malignant lesions are rare at birth. In infancy and early childhood, inflammatory and congenital lesions are most common. Inflammatory lesions normally have a relatively brief clinical duration and may recur at intervals, whereas congenital lesions are usually diagnosed early in life. A painless rapidly enlarging lesion is more likely to be neoplastic. A preceding upper respiratory tract infection, rash, foreign travel, tuberculosis exposure, dental procedures, previous excision of scalp or skin lesions, family history of similar lesions, or animal contacts may suggest an infectious etiology. A recent cat scratch or raw meat ingestion raises the possibility of toxoplasmosis or cat scratch as causative agents. Midline lesions are usually benign. Posterior cervical adenopathy suggests a possible scalp infection. Cystic lesions are more likely to be benign; solid lesions are more commonly malignant. Specific characteristics of a mass, including size, surrounding edema, erythema, tenderness, and fluctuance should be considered. Enlarged lymph nodes up to 2 cm in diameter are not uncommon, especially following an upper respiratory infection. The presence of multiple small nodes bilaterally with such a history is generally of little concern. However, should there be a solitary lymph node or group of lymph nodes that is extremely hard, matted together, fixed to the skin, or demonstrating progressive enlargement without a history of associated respiratory infection, the concern regarding neoplasm becomes greater. Besides the cervical lymph nodes, one also must evaluate for the presence of generalized lymphadenopathy, hepatosplenomegaly, and fever because cervical lymphadenopathy may be a reflection of a systemic condition. The ear, pharynx, tonsils, dental structures, scalp, and skin must be evaluated thoroughly because these are possible primary sites for infection. Supraclavicular adenopathy should alert the clinician to the possibility of pulmonary or abdominal disease. The most common head and neck mass in children is infectious lymphadenitis. Inflammatory diseases generally arise and usually are associated with overlying erythema, edema, tenderness, and fever. The possibility of a malignancy should be considered at an earlier stage if there is persistent fever, weight loss, fixation of the mass to the skin or subcutaneous tissues, or supraclavicular adenopathy. In these cases, immediate biopsy should be considered. In cases in which infectious etiologies are considered to be more likely, observation of the response to antimicrobial therapy is reasonable. Laboratory and radiographic studies may be obtained immediately if there is a mass that suggests a possible malignancy. These could include a complete blood count (CBC) with a differential to evaluate for inflammation or a blood dyscrasia and possibly serum chemical profiles and urinalysis. If infectious etiologies are considered to be more likely, a Monospot and/or serologic tests for Epstein-Barr virus, cytomegalovirus, toxoplasmosis, tularemia, histoplasmosis, syphilis, Bartonella hensalae (cat scratch disease), and HIV may be considered. If a midline congenital lesion seems likely, thyroid function test and ultrasound may precede biopsy or fine needle aspiration. If malignancy is suspected, it is important to evaluate whether the mass may represent a metastatic lesion before proceeding with diagnostic biopsies. A chest radiograph or CT scan may be indicated, especially if lymphoma is being considered where mediastinal involvement is common. Fine needle aspiration is a widely used diagnostic technique for the evaluation of head and neck mass lesions in adults, but its reliability in pediatric patients is dependent on the experience and expertise of the cytopathologist performing the investigation. It is of particular value for differentiating cystic from solid masses. Fine needle aspiration should be considered for diagnosis if there is a mass in a child less than 6 weeks of age, if the history suggests an unusual

pathogen, or if the patient is immunocompromised or has fever and toxicity requiring hospitalization. If a neoplasm is suspected, an open biopsy should be considered unless a definitive diagnosis is obtained by aspiration. INFECTIOUS MASSES The most common causes of bacterial lymphadenitis are Staphylococcus aureus or group A hemolytic streptococci. Antimicrobial therapy should be initiated with a broadspectrum antibiotic effective against these agents if a head and neck mass is thought to be possibly caused by an infectious etiology. In very young children, particularly those with associated periorbital inflammation, Hemophilus influenzae infection should also be considered. If a dental source of infection is suspected, additional anaerobic antibacterial coverage should be included in the therapeutic trial. If the mass responds to therapy (decreased erythema, tenderness, and size), therapy should be continued for several weeks. If there is no change in the size of the mass over 6 weeks, progressive enlargement of the mass over a 2-week interval, or a failure of the mass to regress over 3 months, or if the mass is fluctulent, aspiration and/or biopsy should be considered. One must recognize that shotty adenopathy may be normal in children and therefore does not require biopsy. Other infections cause lymphadenitis. Viral lymphadenitis is generally self-limited and short in duration. Mononucleosis is generally associated with a triad of symptoms including fever, pharyngitis, and cervical lymphadenopathy. Diagnosis can be confirmed with a Monospot test for serum heterophile antibodies in 70% of cases at the end of the first week of infection and in 95% by the end of the third week of clinical symptoms, except in children less than age 4 years, where Epstein-Barr viral antibodies are more reliable tests. Toxoplasmosis is associated with cervical lymph node involvement in 80% of cases, with serology being diagnostic. Kawasaki disease may present with findings of conjunctivitis, a strawberry red tongue, lymphadenitis, and fever. Histoplasmosis may also cause lymphadenitis. Diagnosis is made by serology and/or on biopsy. Tuberculosis infection often presents with adenopathy in the posterior cervical triangle. A PPD test and controls (to assure the patient is not anergic) should be obtained in suspect cases. Chest radiographs may also aid in confirming the diagnosis. Patients with masses caused by atypical tuberculosis caused by the organisms M. scrofulaceum, M. avium intracellulare, and M. kansasii usually have no constitutional symptoms and present with a mass with a rubbery or dull red appearance, minimal tenderness, and possibly a draining sinus. PPD is either negative or weakly positive. Actinomycosis is caused by commensal organisms of the oral cavity, usually Actinomyces israelii. Often the mass develops following dental extractions or infections. Diagnosis requires biopsy. Cat scratch disease is usually associated with conjunctivitis, fever, malaise, and persistent subacute lymphadenitis. The symptoms generally occur 7 to 10 days after being scratched by the animal. Serum titers for Bartonella hensalae are diagnostic. If cat scratch disease is suspected and the patient is very ill, a course of intravenous gentamicin should be considered. For detailed discussions of each infection see Chap. 13. NONINFECTIOUS INFLAMMATORY MASSES Sarcoidosis may present with masses in the lymph nodes, lungs, skin, eyes, and bones. Biopsy shows typical noncaseating granulomas. Inflammation of nonlymphoid tissue occurs in viral thyroiditis and sialadenitis. Inflammation of the salivary glands may have various etiologies. In the newborn, especially the premature infant, sialadenitis is not uncommon and presents with swelling over one of the salivary glands. Dehydration is thought to be a contributing

factor. Treatment consists of hydration and antimicrobial therapy. Viral sialadenitis secondary to mumps is almost exclusively a disease of childhood. Acute bacterial parotitis may also occur in children. This disorder may be recurrent or persistent through adolescence. If a mass undergoes enlargement associated with eating, inflammatory disease of the salivary glands should be suspected. Though there may be some generalized swelling at all times with an infected salivary gland, an increase in size is often noted specifically at the time of feeding. In cases of salivary gland disease, traditional sialography is of limited value, but a CT sialogram may be very helpful in differentiating a mass lesion from an inflammatory disorder. Malnutrition, bulimia, obesity, and pica all may be associated with enlargement of the salivary glands. Administration of a general anesthetic may result in salivary gland enlargement from compression of the submandibular glands during positive-pressure ventilation through a mask or by a mouth gag. Similarly, wind instrument players may find salivary gland enlargement caused by pneumoparotitis, a condition in which air is forced into the salivary ductal system with resultant gland enlargement. CONGENITAL ABNORMALITIES Congenital abnormalities including branchial anomalies, thyroglossal duct cysts, dermoid cysts, teratomas, and laryngoceles may all present at birth or later in life as masses in the head and neck. These are discussed above (Sec. 15.3) and in Chap. 16. If prenatal ultrasonography identifies an anterior neck mass, probable lesions include teratomas, teratoid cysts, and lymphangiomas. Before delivery, plans should be made to have a high-risk team present at the birth, including a pediatric surgeon, anesthesiologist, otolaryngologist, and neonatologist. If the airway is obstructed, intubation and bronchoscopy should be performed immediately. If this is unsuccessful, tracheotomy or even extracorporeal membrane oxygenation (ECMO) may be required until further diagnostic imaging studies can be obtained to define the lesion and plan further therapy. VASOFORMATIVE LESIONS Hemangiomas, lymphangiomas, and hemangiolymphangiomas are found almost exclusively in children and are usually identified before the age of 2 years. Slow, progressive enlargement is common, but a rapid increase in size may be noted during periods of upper respiratory infection. In addition, hemangiomas often will demonstrate enlargement with exertion or dependency. Lymphangiomas or cystic hygromas generally are soft, compressible, and relatively diffuse, but hemangiomas may be more discrete and firm, especially when located within a muscle or the deep soft tissues. Lymphangiomas are often located in the posterior cervical triangle and may contain multilocular cysts. About one-third of patients with lymphangiomas will originally present with signs of infection. A lymphangioma may be more discrete and firm if it has been present for many years and has undergone morphologic alterations as a result of multiple infections with secondary scarring. Cutaneous vascular changes may be indicative of hemangiomas, which are diffuse and even infiltrative. CT and MRI can allow identification of the type of lesion and define its extent. Hemangiomas usually regress over a period of years, though surgical excision is appropriate when there is a diagnostic question, hemorrhage, repeated infections, airway compromise, high-output cardiac failure, or, rarely, significant cosmetic abnormality. Intravenous or oral steroids may be an appropriate initial alternative. Lymphangiomas rarely undergo spontaneous regression and should be managed

either surgically or with sclerotherapy using an alcohol solution. Recently, several centers have used picibanil (OK-432) as a sclerosing agent. This medication is derived from Streptococcus pyogenes and induces the regression of macrocystic lymphangiomas. This therapy is less likely to be effective in the presence of a significant microcystic component to the lesion, massive craniofacial involvement, or following previous surgical resection. TUMORS Malignant and benign neoplasms are uncommon in the neonatal period and may not be accompanied by additional regional or systemic findings. Malignant neoplasms may be differentiated on occasion from benign lesions by their rapid growth, more indurated feel, and frequent attachment to skin or surrounding tissues. Teratomas are located in or adjacent to the thyroid gland and are usually seen in newborns and young infants. Fibromatosis colli is a benign mass located in the anterior portion of the neck that presents 2 weeks or more after birth. It usually occurs following a history of birth trauma, and there is a gradual spontaneous resolution over 4 to 6 months. An ultrasound examination aids in diagnosis. Surgical excision is not indicated. Pilomatricoma, or calcifying epithelioma of Malherbe, is a benign lesion located in the intradermal or subcutaneous soft tissue that represents a hamartoma of the hair follicle. They are usually firm in consistency and attached to the skin. Surgical therapy is diagnostic and therapeutic. Neuroblastoma and Langerhans cell histiocytosis are more common in the first several years of life. Rhabdomyosarcoma occurs most commonly between 4 and 6 years and 14 to 16 years of age. Hodgkin disease is most common in adolescents and adults, whereas non-Hodgkin lymphoma would be more common in children under age 12 years. Posttransplant lymphoproliferative disease should be suspected in immunocompromised patients following transplantation and is characterized by a discrete solid tumor or diffuse enlargement of lymphoid tissues. REFERENCES CLARY RA , LUSK RP: Neck masses. In: BLUESTONE CD , STOOL SE , KENNA MA , eds: Pediatric Otolaryngology, vol 2. Philadelphia, WB Saunders, 1996:1488-1496 CUNNINGHAM MJ: Congenital malformations of the head and neck. In: COTTON RT , MYER CM III , eds: Practical Pediatric Otolaryngology. Philadelphia, Lippincott-Raven, 1999:663-680 MYER CM III , COTTON RT: Lump in the neck. In: MYER CM III , COTTON RT , eds: A Practical Approach to Pediatric Otolaryngology. Chicago, Year Book Medical Publishers, 1988:212-219 NUSS RC , CUNNINGHAM MJ: Pediatric head and neck masses, cysts, sinuses, and tumors. Curr Opin Otolaryngol Head Neck Surg 1:153-160, 1993 ROSENFELD RM: Cervical adenopathy. In: BLUESTONE CD , STOOL SE , KENNA MA , eds: Pediatric Otolaryngology, vol 2. Philadelphia, WB Saunders, 1996:1512-1524 TORSIGLIERI AJ JR , TOM LW , ROSS AJD , WETMORE RF , HANDLER SD , POTSIC WP: Pediatric neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol 16:199-210, 1988

TUNKEL DE , BAROODY FM , SHERMAN ME: Fine-needle aspiration biopsy of cervicofacial masses in children. Arch Otolaryngol Head Neck Surg 121:533-536, 1995 0 Copyright 2003 by The McGraw-Hill Companies, Inc. All rights reserved. (+/-) Show / Hide Bibliography Bibliographic Citations Bibliographic Citations Export a citation for this title: Or highlight and copy (Ctrl-C) the plain text citation below ............................................................ Abraham M. Rudolph, MD et. al., Colin D. Rudolph, MD, PHD, eds. 2003. Rudolph's Pediatrics. New York. McGraw-Hill Medical Publishing Division. ISBN 0-8385-8285-0. STAT!Ref Online Electronic Medical Library. http://online.statref.com/document.aspx?fxid=13&docid=883. 8/20/2010 11:18:11 PM CDT (UTC -05:00). * Copyright: o Copyright 2003 by The McGraw-Hill Companies, Inc. All rights reserved. * Database Title: o STAT!Ref Online Electronic Medical Library * Editor: o Abraham M. Rudolph, MD et. al. o Colin D. Rudolph, MD, PHD * ISBN: o 0-8385-8285-0 * Publication City: o New York * Publication Year: o 2003 * Publisher: o McGraw-Hill Medical Publishing Division * Title: o Rudolph's Pediatrics - 21st Ed. (2003) * Date Posted: o 2/3/2009 3:41:19 PM CDT (UTC -05:00) * Electronic Address: o http://online.statref.com/document.aspx?fxid=13&docid=883 * Date Accessed: o 8/20/2010 11:18:11 PM CDT (UTC -05:00) * Location In Title: o RUDOLPH'S PEDIATRICS - 21st Ed. (2003) 15. THE EAR, NOSE, OROPHARYNX, AND LARYNX - Robin T. Cotton, Associate Editor 15.5 EVALUATION OF HEAD AND NECK MASSES - James H. Liu, Charles M. Myer III Send Feedback Customer Service 800.901.5494 Title Updates User Responsibilities Training Center What's New

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