Burkitt-Lymphom

School ager
with abdominal pain. Axial (above), coronal (lower left) and sagittal (lower right) CT with contrast of the abdomen shows multiple dilated loops of small bowel and a soft tissue mass in the right upper quadrant just beneath the gall bladder that shows the target sign on the sagittal image and the pseudokidney sign on the transverse and coronal images. The soft tissue mass was still present on an US performed 1 hour later.The diagnosis was small bowel obstruction due to an ileal-ileal intussusception caused by Burkitt lymphoma.
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School ager
with bilateral abdominal masses. Coronal T1 MRI without contrast of the abdomen (upper left) shows bilateral enlarged kidneys which after the administration of contrast (upper right) is seen to be due to multiple non-enhancing renal masses. Transverse US of the liver (below) shows a round hypoechoic mass in the liver, superior and to the left of the gall bladder.The diagnosis was Burkitt lymphoma with renal and liver involvement.
pediatricimaging.org • CC-by-nc-sa 4.0
School ager
with crampy abdominal pain. Axial CT with contrast of the abdomen (above) shows a round soft tissue mass on the left side of the pelvis that has a target sign appearance. Coronal (below left) and sagittal (below right) CT show multiple large mesenteric lymph nodes and that the soft tissue mass arises in the pelvis and also has a pseudokidney sign appearance. The diagnosis was small bowel-small bowel intussusception due to Burkitt lymphoma.
pediatricimaging.org • CC-by-nc-sa 4.0
Imaging
findings of gastrointestinal tract tumors in children and adolescents. a, b A 11-year-old girl with Burkitt lymphoma. She complained of abdominal pain for the last 2 months. a US image demonstrates pathological diffuse thickening of the intestinal wall (arrow) causing intussusception at the level of the ileal loops and appearance of a pseudo-mass. b Axial post-contrast CT image shows mass lesion (arrow) in the right lower quadrant in the intestinal wall. Also note the pelvic fluid
insightsimaging.springeropen.com • CC-by-4.0
Imaging
findings of gastrointestinal tract tumors in children and adolescents. a, b A 13-year-old boy with Burkitt lymphoma. The patient had abdominal pain and vomiting for the last 15 days. Invagination was detected in US performed in an external center. Axial and coronal reformatted CT images show intussusception at the level of the ileocecal valve (arrows)
insightsimaging.springeropen.com • CC-by-4.0
Imaging
findings of gastrointestinal tract tumors in children and adolescents. a–e A 4-year-old boy with Burkitt lymphoma. a, b Axial T2 weighted images show hypointense mass lesions that are difficult to distinguish from intestinal loops (arrows). c, d Diffusion-weighted images (b = 800 s/mm2) and (e, f) apparent diffusion coefficient (ADC) maps show diffusion restriction in the lesions (arrows)
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Burkitt lymphoma is an aggressive B-cell lymphoma predominantly affecting children.

Epidemiology

Burkitt lymphoma is the most common (40%) type of non-Hodgkin lymphoma in childhood. Median age is eight years with a male predominance (M:F = 4:1) . It is less common in adults, accounting for 1-2% of lymphomas .

It is considered endemic in parts of Africa where rates are up to 50 times higher than in the USA. Cases occur sporadically outside of the tropics.

Risk factors
  • HIV/AIDS
  • post-transplant immunosuppression

Clinical presentation

Burkitt lymphoma affects many organs, and this affects presentation. Extranodal involvement is common (~30%) at presentation, most often presenting as an abdominal or pelvic mass. Most patients present with widespread disease.

Pathology

Three forms of Burkitt lymphoma have been described :

  • endemic Burkitt lymphoma: linked to Epstein-Barr virus (EBV) and Plasmodium falciparum malaria infections
  • sporadic Burkitt lymphoma: etiology unknown
  • immunodeficiency-associated Burkitt lymphoma: occurs in patients with HIV, post-transplant or congenital immunosuppression

Burkitt lymphoma is an aggressive tumor with a doubling time of 24 hours. It can present in a wide variety of locations:

  • head and neck, e.g. facial bones, Waldeyer ring
  • pleural space (~70%)
  • gastrointestinal tract, especially the ileocecal region
  • mesentery, peritoneum, retroperitoneum
  • kidneys
  • gonads (~75%)

Nodal involvement is more common in adults than in children .

Radiographic features

Radiographic features vary widely depending on organ involvement:

  • abdominal manifestations of Burkitt lymphoma
  • musculoskeletal manifestations of Burkitt lymphoma
  • head and neck manifestations of Burkitt lymphoma

Treatment and prognosis

Burkitt lymphoma can be treated with chemotherapy. In children, the prognosis is good with survival rates >90%. In adults, the prognosis is poorer, with a 5-year survival rate of ~50% and is even worse with bone marrow or CNS involvement (>30% 5-year survival rate) .

History and etymology

First described by Denis Parsons Burkitt (1911-1993), an Irish surgeon, in 1958 in Uganda, Africa .

Differential diagnosis

  • other forms of high-grade B-cell lymphoma
  • Crohn disease may mimic Burkitt lymphoma on ultrasound, barium studies, and CT
Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Burkitt-Lymphom:
Lymphom des
Gastrointestinaltraktes
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primäres
Lymphom des Knochens
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