Lymphadenopathy in Children: Things that go LUMP in the night (Newman)

Question 1

Define lymphadenopathy

Answer 1

Lymph nodes that are abnormal in size, number, and consistency.

Question 2

Describe important components of the history in the evaluation of a pediatric patient with lymphadenopathy.

Answer 2

1. age of patient
2. location of node(s)
   
   1. supraclavicular are worrisome
3. onset and duration of enlargement of node(s)
4. localized symptoms:
   
   1. cough, headache, sore throat, skin sores or redness
5. generalized symptoms:
   
   1. fever, wt loss (>10% in 6 months), appetite loss, fatigue, arthralgias, pain, night sweats, pruritis
6. other:
   
   1. medications, travel, dental problems, bites, immunizations, high risk behavior, ever had the nodes before?
   2. exposures: uncooked meats, animals, unpasteurized milk/soft cheeses, symptomatic individuals

Question 3

Describe important components of the physical exam in the evaluation of a pediatric patient with lymphadenopathy.

Answer 3

1. vital signs: fever, weight loss
2. size of nodes >2cm is considered enlarged
3. location of nodes
   
   1. SUPRACLAVICULAR LYMPHADENOPATHY IS NOT NORMAL
4. quality of nodes
5. local or generalized
6. tenderness, warmth, erythema
7. lesions distal to the lymph node in question (look for injury, scratch, bite, etc)
8. spleen/liver size

Question 4

Name some viral causes of lymphadenopathy in children.

Answer 4

Epstein-Barr Virus

HIV

Question 5

Name some bacterial causes of lymphadenopathy in children.

Answer 5

• Cat scratch disease (Bartonella henselae)
  
  • Perinaud’s syndrome (conjunctivitis, preauricular adenitis, conjunctival granuloma)
• Tularemia
• Staphylococcus/streptococcus

Question 6

Name a cause for lymphadenopathy in children that is not viral or bacterial.

Answer 6

Lymphoma

Question 7

Identify worrisome findings in a child with lymphadenopathy.

Answer 7

Supraclavicular lymph node

Lymph node >2cm in size

Firm and rubbery

Fixed and matted (not mobile)

2 or more nodal groups involved

Question 8

Identify indications for a lymph node biopsy.

Answer 8

1. If watchful waiting is the plan, but the nodes never get better.
2. If the plan included antibiotics but 4-6 weeks after treatment the nodes are no smaller (or they are bigger).
3. Right away if an enlarged supraclavicular node is palpated along with findings that are consistent with malignancy (night sweats, weight loss >10%, abnormal CXR or CT).

Question 9

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

a. Heterotopic tumor-like lesions

Answer 9

• heterotopic
  
  • microscopically normal cells or tissues that are present in abnormal locations (pancreatic tissue in the wall fo the stomach, adrenal cells in the lungs, etc)
    
    • usually insignificant
    • rarely can become malignant

Question 10

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

b. Hamartomas

Answer 10

• Hamartomas
  
  • excessive, focal overgrowth of cells and tissues native to the organ in which it occurs (hemangiomas, rhabdomyomas of the heart, etc)
    
    • benign histology
    • can cause clinical problems, location, size

Question 11

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

c. Hemangiomas

Answer 11

• Hemangiomas
  
  • most common tumors of infancy
  • cavernous or capillary
  • most commonly in the skin in children
    
    • if large and flat: port wine stain
    • usually regresses with age

Question 12

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

d. Lymphatic tumors

Answer 12

• Lymphangiomas
  
  • hamartomatous (disorganized, benign) or neoplastic
  • cystic and cavernous spaces
• Lymphangiectasis
  
  • abnormal dilations of pre-existing lymph channels

Question 13

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

e. Fibrous tumors

Answer 13

• Fibromatosis
  
  • sparsely cellular proliferations of spindle shaped cells
• Fibrosarcoma
  
  • Richly cellular lesions

Question 14

Name and briefly discuss the benign tumor and tumor-like lesions of infancy and childhood.

f. Teratomas

Answer 14

• Teratomas
  
  • sacrococcygeal are the most common
  • 3 Types:
    
    • benign, well-differentiated cystic lesions (mature teratomas)
    • Lesions of indeterminate potential (immature teratomas)
    • Unequivocally malignant teratomas (usually mixed with another germ cell tumor component)

Question 15

List the sites of origination of most malignant cancers

Answer 15

• Hematopoietic system
• Nervous tissue
• Soft Tissues
• Bone
• Kidney
• Leukemia alone accounts for more deaths in children younger than age 15 than all of the other malignant tumors combined.
• Adult (for comparison): skin, lung, breast, prostate, colon

Question 16

Discuss childhood neuroblastoma and the associated cutaneous finding seen in disseminated disease.

Answer 16

• Neuroblastoma
  
  • most important “neuroblastic” tumor
    
    • Tumors of the sympathetic ganglia and adrenal medulla
    • derived from primordial neural crest cells
  • Most common extracranial solid tumor of childhood
    
    • ​about 90% produce catecholamines
      
      • lab values of urine VMA and HVA
  • in children <2years old
    
    • fever, large abdominal mass, weight loss
    • in infants: multiple cutaneous metastasis causing blue color AKA “blueberry muffin baby”
  • Older children
    
    • may not be diagnosed until metastasis produced symptoms (bone pain, respiratory symptoms, GI ocmplaints, periorbital region)

Question 17

Discuss Wilms tumor and list the three syndromes/conditions associated with increased risk of the development of this tumor.

Answer 17

• the risk of Wilms tumor is increased with at least 3 recognizable groups of congenital malformations associated with distinct chromosomal loci
  
  • WAGR syndrome
    
    • Wilms tumor, Aniridia, Genital anomalies, Mental retardation
    • Lifetime risk of developing Wilms tumor is about 33%
    • Constitutional deletions of 11p13
  • Denys-Drash syndrome
    
    • 90% chance of developing Wilms tumor
    • Gonadal dysgenesis
    • early onset nephropathy
    • genetic abnormality is dominant missense mutation ot the zinc finger region of the WT1 protein (chromosome 11p13)
  • Beckwith-Wiedemann syndrome
    
    • enlargemnt of body organs
    • ompahlocele
    • hemihypertrophy
    • macroglossia
    • abnormal large cells in the adrenal cortex
    • chromosomal abnormality implicated has been localized to band 11p15.5 (WT2)

Question 18

What are the signs and symptoms of Wilms tumor?

Answer 18

• Wilms tumor
  
  • most common primary renal tumor of childhood
  • peak incidence
    
    • between 2-5 years of age
    • 95% occur before 10 years
  • 5-10% involve both kidneys
    
    • simultaneously (synchronous)
    • one after the other (metachronous)
• Wilms tumor usually presents with one of the following:
  
  • a large abdominal mass
  • hematuria
  • pain in the abdomen after some traumatic incident
  • intestinal obstruction
  • hypertension
  • pulmonary metastasis