Non-Hodgkin’s lymphoma (NHL)

Incidence:

• 9.1 per million Caucasians. 4.6 per million African-Americans < 15 years of age in US
• endemic in Africa: 50% of childhood cancers in Africa (known as Burkitt’s lymphoma)
• Increases after 3 years of age – incidence increases through life
• Multiple, contiguous tumors, Commonly extranodal (vs Hodgkins, orderly spread)

Pathology:

• T-, B-, or indeterminate cell origin
• EBV: Burkitt’s lymphoma (jaw)
• 3 subtypes: Lymphoblastic, Large cell, Small noncleaved cell lymphoma (SNCCL)
• Sometimes overlaps with ALL

NHL Subtypes (in order of frequency)

1. Small noncleaved cell lymphoma (40-50%)
   • Abdominal tumor in 80% (abdominal pain, distention, obstruction, bleeding, perforation, intussuception)
   • Other sites: CNS, BM, peripheral LN
   • Jaw involvement (<20% U.S. vs. 70% endemic Burkitt lymphoma in Africa)
   • Rapid growth
   • Cell bio:
     • B-cell markers: CALLA, CD20
     • negative TdT
     • c-myc proto-oncogene translocation
2. Lymphoblastic lymphoma (30%)
   • Mediastinal mass/intrathoracic tumor (dyspnea, CP, dysphagia, pleural effusion, SVC syndrome)
   • Cervical or axillary LAD in up to 80%
   • Bone, BM, Testis, Skin, CNS can be involved
   • Cell bio:
     • predominantly T-cell origin
     • positive TdT
     • majority lack chromosomal translocations
3. Large cell lymphoma (20%)
   • Abdomen, mediastinum, skin, bone, soft tissues
   • CNS involvement is rare

Labs: Vary
Diagnosis: CT/MRI and biopsy

Staging

1. single tumor
2. single tumor with regional nodal involvement
3. tumor or LN on both sides of diaphragm
4. BM or CNS disease, regardless of other sites.

Treatment: Chemotherapy (CHOP), monitor closely for tumor lysis syndrome. Radiation therapy not very effective (vs Hodgkins)

Prognosis:

• Related to grade vs. stage; Generally worse than Hodgkins
• Limited disease: >90% cure rate
• Advanced disease:
  • SNCCL 70-80%
  • LCL 50-70%

CHLA Board Review 2005
Schwartz. The 5-minute pediatric consult, 3rd edition.