SPR Unknown #80 -- FINAL

Dan Musick, M.D.

NMH

d-musick@northwestern.edu

Mary Wyers, MD

Children's Memorial Hospital Chicago

Abstract

3-year-old male with abdominal pain.

Keywords

1686


Publication Date: 2011-01-14

History

3-year-old male with abdominal pain.

Findings


Images 1-7: CT demonstrates numerous low density, homogeneous, relative hypoenhancing intrahepatic lesions. Overall, the liver is enlarged. The background liver parenchyma is unremarkable. There is no intrahepatic biliary ductal dilatation. Multiple low density enhancing lesions are also present throughout the kidneys. A mass within the bowel in the right lower quadrant is seen, the appearance of which is compatible with an ileocolic intussusception. The intraluminal soft tissue mass appears slightly lobulated and thickened suggesting the possibility of a pathologic lead point.




Images 8-9: These axial and coronal CT images were obtained 1 week following presentation and initiation of chemotherapy. Note the rapid response with near-complete resolution of the hepatic and renal masses. The ileocolic intussusception has also resolved without invasive intervention.



Diagnosis

Burkitt Lymphoma

Discussion

Burkitt, or Burkitt’s Lymphoma, is a subtype of Non-Hodgkin Lymphoma involving B cells. It results from a chromosomal translocation of the c-myc gene. Non-Hodgkin Lymphoma is categorized based on histologic subtype: undifferentiated, lymphoblastic, and large cell. The undifferentiated subtype is the most common and usually manifests as intraabdominal disease. This includes the Burkitt variety. Lymphoblastic tumors manifest as an anterior mediastinal mass. Large cell tumors spare the anterior mediastinum and demonstrate heterogeneous imaging features.

Burkitt Lymphoma is divided into 3 clinical categories: endemic variant, sporadic variant, and immunodeficiency-associated. The endemic variant occurs in equatorial Africa where there is an association with Malaria, which is believed to increase susceptibility to Epstein Barr Virus. This variant manifests in the jaw and neck. The sporadic variant is most common outside of Africa, and there is no association with EBV. The ileo-cecal region is commonly affected in this form. The immunodeficiency-associated variant occurs in HIV or post-transplant patients on immunosuppressive therapy. Burkitt lymphoma is associated with 90% of AIDS cases.

NHL, including Burkitt, accounts for 30-50% of childhood lymphomas and 7% of all malignancies in children younger than 15 years. Burkitt Lypmhoma is a high grade, fast growing tumor. Thus, it is extremely sensitive to chemotherapy and can result in ‘tumor lysis syndrome’ during treatment resulting in numerous electrolyte imbalances. Admission and adequate hydration is important during treatment. Prognosis for Burkitt Lypmhoma is overall favorable given its rapid response to chemotherapy.

References

  1. Ghai, S, M.D., et. al. Primary gastrointestinal lymphoma: spectrum of imaging findings with pathologic correlation. Radiographics 2007; 27:1371-88.
  2. Hamrick-Turner, JE, M.D. et. al. Imaging of childhood non-Hodgkin lymphoma: assessment by histologic subtype. Radiographics 1994; 14:11-28.

9 images