The node knows: Solving the mystery of lymphadenopathy

Lymphadenopathy is common in pediatrics and a frequent parental concern. There is a broad differential diagnosis, which ranges from self-resolving benign conditions to aggressive malignant diseases. Most commonly, lymphadenopathy is the result of an infection, but the possibility of malignancy can lead to apprehension in both parents and physicians. Knowing when it is appropriate to observe patients with lymphadenopathy and when a more extensive workup (including referral and/or biopsy) is indicated can be confusing. Most of the time, a thorough history and physical exam will lead to the proper diagnosis. Asking a few key questions can aid in unraveling the diagnostic dilemma.

Is the mass really a lymph node?

Nonlymphoid masses that occur in the neck can mimic lymphadenopathy. Examples are congenital lesions, such as cystic hygromas, branchial cleft cysts, thyroglossal duct cysts and cervical ribs. Other neck masses include goiters, neurofibromas and muscle tumors. These entities occur less often in other areas of the body.

Is the node truly enlarged?

In children, lymph nodes frequently are palpable, especially in the cervical, axillary and inguinal regions. Nodes are not considered abnormally large unless they are greater than1 cm in diameter for cervical and axillary nodes or greater than 1.5 cm for inguinal nodes. Nodes in other regions usually are not palpable. Nodes greater than 2 cm in diameter are much more likely than smaller nodes to be due to a malignancy.

How old is the child?

Forty-four percent of children younger than 5 years of age at well-child visits and 64 percent at sick visits have palpable nodes. Older children and adolescents have palpable nodes less frequently. The causes of lymphadenopathy are very different at different ages. Younger children are more likely than adolescents to have lymphadenopathy due to otitis, upper respiratory tract infections and conjunctivitis. Hodgkin lymphoma and sexually transmitted diseases are rare in children younger than 10 years old but are important causes in adolescents.

Where is the node?

Knowledge of the lymphatic drainage system (See Table 1.) can help pinpoint the cause of the enlargement by directing the evaluation to the source of infection or malignancy. For example, streptococcal pharyngitis often is associated with an enlarged cervical node. Supraclavicular lymphadenopathy is associated with mediastinal or abdominal pathology, often is due to a malignant process and almost always should be biopsied.   

What does the node feel like?

Malignant nodes most often are firm and rubbery, and rarely are tender. They may be matted or fixed to each other, the skin or underlying structures. Conditions such as tuberculosis and sarcoidosis that cause inflammation in tissues adjacent to the nodes also can cause nodes to become matted or fixed. If the node is tender, indurated or fluctuant, or if the surrounding skin is erythematous or warm, infection is the most likely cause.

Is the lymphadenopathy localized or generalized?

Generalized lymphadenopathy, which is the enlargement of two or more noncontiguous nodal groups, is caused by systemic disease. Other systemic signs and symptoms, such as rash, fever, weight loss, night sweats and hepatosplenomegaly, often are present. Localized lymphadenopathy most often is caused by infections of the node itself or its drainage area.

The answers to these questions, as well as other information gathered from the history and physical exam (such as exposures, medications, presence of other symptoms), should limit the differential diagnosis (See Table 2.) and guide further evaluation and treatment. If an abscess is present, it should be drained and cultured. If the cause is a viral infection, no intervention is needed. If bacterial lymphadenitis is suspected, antibiotic therapy that covers streptococci and staphylococci should be initiated. If the lymphadenopathy does not decrease in size in 10 to 14 days, further evaluation is indicated. Depending on the clinical situation, this may include a complete blood count with differential, chest X-ray, LDH, tests for specific infections, tuberculin skin test and tests for autoimmune diseases. Consultation with an oncologist or infectious disease specialist also should be considered. Corticosteroids should not be given without a definitive diagnosis, as they may mask a diagnosis of leukemia or lymphoma and worsen the outcome in these diseases. 

If there is reason to suspect malignancy, a surgical biopsy is indicated. Findings suggesting possible malignancy include: weight loss (greater than 10 percent of body weight); night sweats or fevers; hard, fixed or matted nodes; supraclavicular lymphadenopathy; lymph node size greater than 2 cm; mediastinal mass; and elevated LDH. Biopsy also is indicated if the lymph nodes do not decrease in size in four to six weeks, increase in size after two weeks or do not return to baseline size in eight to 12 weeks. The development of new signs or symptoms should prompt a re-evaluation. Following the above steps will lead to a definitive diagnosis of the cause of lymphadenopathy.

References

Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 17th ed. WB Saunders Co; 2004: 1677-1678.

Friedmann AM. Evaluation and management of lymphadenopathy in children. Pediatr Rev.  2008; 29; 53-60.

Long SS, Pickering LK, Prober CG. Principles and Practice of Pediatric Infectious Diseases. 2nd ed. Churchill Livingstone; 2003: 123-4; 172-3.

Slap GB, Brooks JS, Schwartz JS. When to perform biopsies of enlarged peripheral lymph nodes in young patients. JAMA. 1984; 252(10):1321-6.

Twist CJ, Link MP. Assessment of lymphadenopathy in children. Pediatr Clin N Am. 2002; 49; 1009-25.

Richard Tower, MD, is a pediatric hematologist/oncologist at Children's Hospital of Wisconsin. He also is an assistant professor of Pediatrics (Hematology/Oncology) at the Medical College of Wiconsin and a member of Children's Specialty Group.

For more information Cancer and Blood Disorders Center (414) 266-2420

To make an appointment Central Scheduling (414) 607-5280 local or (877) 607-5280 toll-free

To earn CME credit click here to open the quiz online or to download as a .pdf click here.

You may need Adobe Acrobat Reader to view an item on this page. Click here to download the reader for free.