|Year : 2013 | Volume
| Issue : 4 | Page : 751-753
Non-thrombotic superior sagittal sinus occlusion with intracranial hypertension following metastatic Burkitt's lymphoma
Ankur Wadhera1, Prasant Peter1, M Joseph John2, Rajesh Chakravarti1
1 Department of Radiodiagnosis, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Hemato-Oncology, Christian Medical College and Hospital, Ludhiana, Punjab, India
|Date of Web Publication||11-Feb-2014|
Department of Radiodiagnosis, Christian Medical College & Hospital, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None
Intracranial metastasis is a known complication of Burkitt's lymphoma, however, superior sagittal sinus invasion by dural metastasis from Burkitt's lymphoma is rare. We report a young adult, known case of Burkitt's lymphoma, who presented with features of raised intracranial pressure secondary to dural sinus invasion from metastasis. Prompt radiotherapy to these lesions can bring about recanalization of the sinus with elevation of symptoms.
Keywords: Burkitt′s lymphoma, dural metastasis, magnetic resonance imaging, superior sagittal sinus occlusion
|How to cite this article:|
Wadhera A, Peter P, John M J, Chakravarti R. Non-thrombotic superior sagittal sinus occlusion with intracranial hypertension following metastatic Burkitt's lymphoma. J Can Res Ther 2013;9:751-3
|How to cite this URL:|
Wadhera A, Peter P, John M J, Chakravarti R. Non-thrombotic superior sagittal sinus occlusion with intracranial hypertension following metastatic Burkitt's lymphoma. J Can Res Ther [serial online] 2013 [cited 2019 Dec 11];9:751-3. Available from: http://www.cancerjournal.net/text.asp?2013/9/4/751/126488
| > Introduction|| |
Burkitt's lymphoma/leukemia constitutes only 2-3% of adult non-Hodgkin's lymphoma (NHL), however, is responsible for up to 30% of childhood NHL.  Intracranial metastasis from lymphomas may be in the form of dural lesions, however, invasion of the dural venous sinuses by such lesions is rare. We report a case of Burkitt's lymphoma who presented with both intra parenchymal as well as dural-based metastatic lesions with invasion of the superior sagittal sinus causing symptoms of raised intra cranial pressure.
| > Case Report|| |
An 18-year-old boy presented to the casualty with complaints of severe headache and blurring of vision since 1 day. The headache was holocranial in distribution and continuous with no relief with analgesics. There was no history of associated fever, vomiting, or seizures. On ophthalmological examination, there was only light perception in both eyes with bilateral papilledema noted on opthalmoscopy. The central nervous system examination was otherwise normal. The patient was diagnosed to have Burkitt's lymphoma 5 months ago when he was operated for intussusception of the intestine. Histopathological studies confirmed Burkitt's lymphoma. Bone-marrow examination showed presence of more than 25% leukemic infiltrates suggestive of lymphoma/leukemia and t (8;14) was positive in the bone marrow. Cerebrospinal fluid examination at diagnosis was normal.
He was initiated on RCOMP.  (Drugs used: Rituximab, Cyclophosphamide, Vincristine, Methotrexate, Prednisolone) chemotherapy. While on treatment after two cycles of treatment, he had persisting back ache and MRI of the spine showed marrow-infiltrative lesion in L3 vertebral body with CSF cytology showing lymphomatous/leukemic infiltration of CSF. He was started on Triple intrathecal twice weekly (TIT) (Drugs used: Cytosine arabinoside: 40 mg, Methotrexate: 12.5 mg, Hydrocortisone: 50 mg) chemotherapy for CNS disease which showed clearance of the disease after 4 TITs. After completion of 6 TITs, he was continued on once-monthly prophylactic TIT and had been asymptomatic till the present admission.
In view of the history and presenting complaints, a contrast MRI of the brain was performed which revealed an enhancing, focal intra parenchymal lesion in the right frontal lobe with surrounding perilesional edema, suggestive of metastasis. Two enhancing dural-based lesions were also noted in the parieto-occipital region in parafalcine location, which were invading into the adjacent superior sagittal sinus in the region of terminal portion of superior sagittal sinus and torcular herophili with loss of the dural sinus signal and prominent collateral channels on MR Venography [Figure 1]. Repeat CSF analysis again revealed extensive lymphomatous infiltration of CSF. A diagnosis of intracranial metastasis with sagittal sinus occlusion was made and the patient underwent seven fractions of whole brain radiotherapy after which, his symptoms, including the vision loss, improved significantly. Repeat MRI with Venography confirmed significant reduction in size of the dural lesions with recanalization of the superior sagittal sinus [Figure 2].
|Figure 1: Magnetic resonance imaging at the time of presentation [Axial T2 FLAIR, Sagittal T2W, post-contrast, Axial and Coronal images, and magnetic resonance (MR) Venography] shows perilesional edema with enhancing lesion in the right frontal lobe, suggestive of metastasis (single arrow). Enhancing dural-based lesions are noted (double arrows) with invasion into the superior sagittal sinus (triple arrows). MR Venography shows non-visualized terminal portion of the superior sagittal sinus and torcular herophili (arrowhead) with prominent vein of Trolard and smaller collateral veins|
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|Figure 2: Magnetic resonance imaging after seven cycles of radiotherapy to the dural lesions (Axial T2W, Coronal T2W, and magnetic resonance (MR) Venography) show significant reduction in size of the dural lesions (single arrow) with normal signal void in the superior sagittal sinus (double arrows). MR Venography shows recanalization of the superior sagittal sinus (arrowhead)|
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| > Discussion|| |
Burkitt's lymphoma is a type of B-cell ALL, included in the ALL-L3 category. The median age of children with Burkitt's lymphoma is 8 years (age range : 0-20 years), with children who are 5-9 years old contributing more than one-third of the cases.  Three distinct clinical forms of Burkitt's lymphoma are recognized - endemic, sporadic, and immunodeficiency-associated. Endemic and sporadic Burkitt's lymphomas occur frequently in children in Africa, and the sporadic form occurs in the Western countries. Immunodeficiency-associated Burkitt's lymphoma is seen in patients with HIV infection.
Epstein-Barr virus is believed to play a role in its pathogenesis and is identified in more than 90% of endemic Burkitt's lymphomas, approximately 20% of sporadic and approximately 40% of HIV-associated cases.  Burkitt's lymphoma is the most rapidly growing tumor in children, with a doubling time of approximately 24 h, so prompt recognition and initiation of therapy are essential.  CNS prophylaxis is recommended during the initial treatment of NHL subtypes that carry a high risk of CNS relapse, especially Burkitt's lymphoma. 
Intracranial metastasis in Burkitt's lymphoma may present as intraparenchymal lesions with leptomeningeal metastasis being less common and manifesting either due to direct extension from the surrounding tumor or due to hematogenous spread. The latter is more important in Burkitt's lymphoma. Hematogenous spread to the leptomeninges and CSF may be either through choroid plexus seeding, through the Virchow-Robin spaces into the pia mater and then subarachnoid space, or by interruption of the thin walls of microscopic vessels in the arachnoid to enter the subarachnoid space.
The commonest tumor metastasizing to the meninges is lung cancer.  Other causative tumors include breast, malignant melanoma, lymphomas, and colorectal cancers. Metastasis to meninges by lymphomas can present as subarachnoid nodules, diffuse leptomeningeal carcinomatosis or less commonly, as dural-based masses. 
Lymphomas presenting with CNS manifestations and suspected superior sagittal sinus (SSS) involvement may be due to either sinus thrombosis or rarely, non-thrombotic sinus occlusion by extrinsic tumor mass. Rapid occlusion of SSS is associated with acute rise in intracranial pressure due to insufficient absorption of collateral pathways resulting in venous congestion and decreased CSF absorption, seen more often in thrombosis. A gradual process like non-thrombotic occlusion manifests as cork-screw-like dilatation of the cortical veins, indicative of venous collaterals, as was seen in our patient. 
Non-thrombotic superior sagittal sinus occlusion is an uncommon complication of a local neoplastic disease which usually presents as chronic intracranial hypertension without focal signs.  The syndrome consists of compressive occlusion of dural venous sinuses secondary to the midline tumor masses in the occipital region of the skull. Although typically described in association with extradural mass beneath the inner table of the skull, it is often difficult to differentiate between compressive occlusion of the superior sagittal sinus and tumor invasion of the dura and sinus. The site of involvement of the superior sagittal sinus is most commonly the terminal portion of the superior sagittal sinus and the torcular herophili,  as had been seen in our patient too.
In conclusion, we would like to suggest that dural metastasis with dural venous invasion should be considered in all known cases of lymphoma presenting with features of raised intracranial pressure and imaging should be done for confirmation as timely radiotherapy to the dural lesions can bring about dural sinus recanalization and elevation of the symptoms of intracerebral hypertension.
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[Figure 1], [Figure 2]