CASE PRESENTATION
21 SEPTEMBER 2012
Flip Otto
Dept. of Clinical Imaging Sciences
UFS
Clinical information
15 year old girl from Kimberly
Problem list:

Burkitts lymphoma/leukemia (bonemarrow biopsy confirmed
Burkitt type blasts on immunophenotyping)
 Thrombocytopaenia, anaemia
 Subdural collection with mass effect on presentation
 HIV positive, on ARV’s since June 2012, latest CD4 count
501
 Infective endocarditis of mitral valve
 DVT
 On empirical TB treatment
 Presented with sudden loss of vision on 19 August 2012

Clinical information (cont)

Management
 Subdural
collection drained on 14 June 2012
 Intrathecal chemotherapy started (flow cytometry
negative)
 First cycle of chemo started on 26 June, complicated by
neutropenic sepsis
 ICU admission on 13 July for respiratory failure
 Transferred to ward on 10 August
 Patient discharged to Kimberley for further palliative
management
Imaging findings

Initial uncontrasted CT brain on 13 June 2012:
 Left
subdural collection (20HU) 9mm deep
 Left frontal dural-based density 3mm deep
 9mm midline shift to the right

Follow up uncontrasted CT on 28 June 2012:
 Left
parietal burrhole
 Left subdural collection decreased in size with
decreased mass effect
Imaging findings cont.

CT brain pre and post-contrast on 19 August 2012:
 Hyperdense
subdurally based lesions with contrast
enhancement, left frontoparietal and left occipital
regions
 Leptomeningeal/cortical hyperdense lesions, with
contrast enhancement, in the right occipital and left
frontal lobes, with underlying subcortical white matter
hypodensity
Imaging findings cont.

MRI of the brain on 22 August 2012:
 Subdurally
based lesions left frontoparietal and
occipital appear iso-intense to grey-matter on T1, T2,
T2* and T2 FLAIR, with intense contrast enhancement
 Uniform, intense dural enhancement in the left
hemisphere
 Leptomeningeal lesions left frontal and right occipital
are hyperintense on T1, with contrast enhancement
 Underlying subcortical white matter show high signal on
T2 and T2 FLAIR
Diagnosis



Dural metastases of Burkitts lymphoma/leukemia
Differential diagnosis of right occipital and left
frontal leptomeningeal enhancement and underlying
white matter lesions includes subacute ischaemic
infarctions with luxury perfusion, laminar necrosis
and/or haemorrhagic transformation and
leptomeningeal metatsases.
Leukemic cells subsequently confirmed in CSF
Differential diagnosis of Dural
Metastases
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Usually hematogenously disseminated en plaque lesions from
extracranial primary tumours
Lung, breast and prostate cancer, as well as melanoma, known to
cause dural metastases
Breast carcinoma most commonly associated with purely dural
metastases
Dural lymphoma may be the primary focus of neoplasm
Dural plasmacytoma nearly identical to dural lymphoma
In children dural metastases are commonly associated with leukemia
and neuroblastomas
Inflammatory lesions that may simulate dural metastases include
granulomatous infections, sarcoidosis, Erdheim-Chester disease and
Langerhans cell histiocytosis
Discussion: Burkitt’s lymphoma

Burkitt’s lymphoma/Burkitt cell leukemia:
Clinically most aggressive lymphoid leukemia, of B-cell
origin.
 Association with EBV in a variable proportion of cases
 <1% of NHL but 30% of childhood NHL in USA
 Leukemia presents with widespread involvement of the bone
marrow and peripheral blood.
 Lymphoma used for proliferations arising as discrete masses
 Distinction between lymphoma and leukemia often blurred

Pathology

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Cells homogeneous in size and shape with a very
high proliferative fraction
Pathologists sometimes have difficulty distinguishing
between Burkitt’s lymphoma and diffuse large B cell
lymphoma
Distinction can sometimes be made based on the
extremely high proliferative fraction in Burkitt’s
lymphoma
Most rapidly progressive human tumour, with a
propensity to metastasize to the CNS
Burkitt’s lymphoma:
“Starry sky” appearance at low power light microscopy
High power microscopy showing multiple
small nucleoli and high mitotic index
Burkitt’s lymphoma: Clinical
presentation

Three distinct clinical forms:

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
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Endemic (African) type
Sporadic (nonendemic)
Immunodeficiency-associated (in HIV infection)
Extranodal disease common and all three variants are at risk for
CNS disease
Endemic form involves jaws and orbits in 50% of cases (floating
tooth sign on plain radiography)
Sporadic form has predilection for ileocecal region
Ovaries, kidneys and breast may be involved in both
Retroperitoneal and paraspinal disease causing paraplegia is a
presenting feature in up to15% of cases
Leptomeningeal disease can be seen at presentation and is a site of
relapse
Diagnostic and staging work-up
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Once diagnosis of BL suspected, diagnosis and
staging evaluation should be prompt
Since it is the most rapidly progressing human
tumour, delay in starting therapy can be detrimental
to the prognosis
Initial examination should always include CSF
analysis to rule out metastases, in addition to
standard staging investigations for NHL
Treatment and Prognosis

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Treatment should commence within 48hrs of
diagnosis
Intensive chemotherapy regimens including high
doses cyclophosphamide are used
Prophylactic therapy to the CNS is mandatory
One of the first cancers cured by chemotherapy
Cure in high percentage of young patients treated
effectively, but salvage therapy following relapse
generally ineffective, with poor prognosis
References
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Armitage JO, Longo DL: Malignancies of Lymphoid cells, in
Harrison’s Principles of Internal Medicine, 16th ed, DL Kasper
et al (eds.). New York, McGraw-Hill, 2005, Chap 97
Aster JC: Disease of the White Blood Cells, Lymph Nodes,
Spleen and Thymus, in Robbins and Cotran Pathological Basis
of Disease, 7th ed, V Kumar et al (eds.). Philadelphia,
Elsevier Saunders, 2005, Chap 14
Vinnicombe SJ, Reznek RH: Reticuloedothelial Disorders:
Lymphoma, in Grainger & Allison’s Diagnostic Radiology, 5th
ed, A Adam et al (eds.). Churchill-Livingstone, 2008, Chap
72
Yousem DM, Grossman RI. Neuroradiology: The Requisites, 3rd
ed. Philadelphia, Mosby, 2010, p.65-67