Lymphoid Lesions Burkitt’s Lymphoma

Lymphoid Lesions

Burkitt’s Lymphoma

Etiology • B lymphocyte malignancy associated with genetic mutations:
C-MYC, P53, and others • Causative association with Epstein-Barr (EB) virus, and malaria
cofactor believed to increase the risk for gene-translocation accidents
• African form closely associated with EB infection; North American form less strongly associated

Clinical Presentation • Rapidly progressive facial asymmetry, chiefly of the mandible • Proptosis in children may occur in association with maxillary
lesions. • Pain and paresthesia associated with jaw lesions • Children predominately affected • Facial presentation noted in 25% of North American (nonen-
demic) cases; nearly 100% in African children (endemic cases) • Abdominal (retroperitoneal) presentation usually noted initially
in nonendemic form and in a wider age range than in endemic form
Radiographic Findings • Ill-defined radiolucency • Loss of lamina dura and developmental crypt(s) around
unerupted teeth • Uniform widening of periodontal membrane space • Teeth may be displaced, causing malocclusion and/or exfoliation.
Diagnosis • A diffuse proliferation of small noncleaved lymphoid cells
(B lymphocyte–derived cells) • Tumor cells have round nuclei and prominent nucleoli. • Scattered macrophages with abundant pale cytoplasm containing pyknotic cellular debris represent the “stars” in the socalled starry sky appearance.
Differential Diagnosis: Clinical • Other jaw malignancies of childhood (Ewing’s sarcoma,
osteosarcoma) • Acute infection
Differential Diagnosis: Microscopic • Other round cell malignancies of childhood (neuroblastoma,
leukemia, embryonal rhabdomyosarcoma)
Treatment • Multiagent chemotherapy • Overall cure rate in children is now up to 90% with intensive,
high-dose fractionated therapy
Prognosis • Fair