Unusual Non-Hodgkin's Lymphoma
THE CENTRAL NERVOUS SYSTEM (CNS) LYMPHOMA
CNS - means brain and spinal cord. The risk of CNS involvement is sometimes so high in some lymphomas that treatment to the CNS is given upfront with the general chemotherapy.
Low grade lymphomas are not at significant risk of developing CNS disease. The risk with intermediate grade is (6.5%) and high grade lymphomas is (16.7%).
The following groups have a higher risk of CNS involvement, usually at relapse. (Liang R; Chiu E; Loke SL Hematol Oncol ; 8(3):141-5 1990)
Orbital disease 43%
Peripheral blood 33%
Nasal/paranasal sinuses 23%
Bone marrow 20%
CSF examination should be part of staging of all patients with intermediate and high grade lymphomas with these risk sites, particulary if young and associated with bulky retroperitoneal disease.
Full Acute Lymphocyctic Leukaemi like prophylactic treatment of the CNS should be given to patients with Burkitts or lymphoblastic lymphoma.
Prophylaxis should be considered in patients with paranasal or base of skull, extra dural and testicular lymphoma .This should consist of intrathecal methotrexate 12.5 mg once or twice weekly up to x 9 and moderate dose intravenous methotrexate given in conjunction with the treatment programme for the sytemic disease, followed by cranial radiotherapy (24 Gy in 12 daily fractions) in patients with paranasal sinus or testicular disease.
Methotrexate 12.5mg it
Take CSF sample for cytology prior to chemotherapy. Treatment is 2 x weekly until CSF remission, then once a week x 4 and subsequently every 2 weeks until RT if indicated to a total of 12 - 16.
If the patient is in systemic remission at attainment of CSF remission, then craniospinal RT should be considered as consolidation therapy to a dose of up to 35 Gy/20F, modified if necessary in the light of previous RT. If spinal RT not possible, then cranial RT should still be considered. Localised lymphomatous masses should be considered for RT even in the palliative setting although the prognosis is poor. In palliation the recommended dose is 20 Gy in 5#.
PRIMARY EXTRA NODAL LYMPHOMAS
A. Age <65 - combined modality therapy with  CHOD/BVAM chemo then  RxT.
B. Severely disabled and/or > 65 - RT alone.
A1. Chemotherapy; CHOD/BVAM (JCO;Vol.14,No.3, pp945-954)
A2. Radiotherapy - Total dose 54Gy in 30F to primary site:-
Brain: Phase I - - 9Gy in 5F to tumour site
Phase II - 45Gy in 25F to whole brain. C2/3 interspace (Include optic nerve extension into orbits if csf +ve)
Spinal cord: (if CSF +ve) - 35Gy min in 21F over 5 weeks (To S2/S3 interspace, with a gap change at the 7th and 14th fraction)
[B] Radiotherapy alone
Phase I - 45 Gy in 25F to whole brain (supine cast)
Phase II - 10 Gy in 10F to tumour site.
RECURRENT CEREBRAL LYMPHOMA
High dose methotrexate - see chemotherapy protocols
A full examination under anaesthetic is required. For paranasal or basilar skull sinus disease CNS prophylaxis should be considered. There is an association between B-cell lymphomas of the Weldeyers ring andgastrointestinal lymphoma (Ree et al 1980. Cancer, 46,1528). Barium meal examination should be considered in the initial staging, RxT should include the whole Weldeyers ring, but otherwise treat as NHL above. Very elderly with stage I or IIE Weldeyers ring disease would probably best be treated with RxT alone.
Low grade B-cell gastric lymphomas is characterised by an indolent natural history and a tendency to remain localised for a long period of time. Pathologically lesions may be multifocal in the gastric mucousa with sites remote from the main tumour mass. These have been characterised as part of the histological appearances found in mucosal associated lymphoid tumours MALT of the stomach (Isaacson 1987, Histopathology11:445)
Diagnosis is best made by endoscopic biopsy rather than gastrectomy.
Follow resection Follow resection. If complete resection has occured with only the proximal gastric nodes involved the prognosis is excellent (Sheppard JCO 1988, 6:253), but there is a tendency for the re-appearance of the MALT lesions (Wotherspoon 1993Lancet, 3442:575.) All patient should have treatment for Helicobacter pylori. Then either observed or given Chlorambucil. Alternatively patients may be entered into the LYO3 trial (see appendix).
Following incomplete resection or endoscopic biopsy. All patient should have treatment for Helicobacter pylori. Then given Chlorambucil. Alternatively patients may be entered into the LYO3 trial (see appendix).
Anti-H.Pylori treatment - omeprazole 20mg daily, Clarithromycin 250 mg bd, tinidazole 500 mg bd for 2 weeks. In the 10% of people were this is not successfull - colloidal bismouth 120mg qds, metronidazole 400 mg tds, tetracycline 500mg qds & omeprazole 20mg od.
HIGH AND INTERMEDIATE GRADE
Patient who are able to undergo complete surgical resection of their tumour have a better survival than those with partial or no resection (Morton 1993, Cancer 67:776).
Following complete resection +/- adjuvant CHOP or LYO4 study (see appendix)
Following incomplete resection CHOP x3-6 or LYO4 study (see appendix).
For patients with early stage AIDS, ie few or no significant other HIV-related complications, treatement should be as for HIV negative lymphoma. If patient has more severe HIV-related problems or a poor performance status, the prognosis is poor and treatment should be palliative.
Particular problems in HIV-related lymphoma:
1. Bone marrow reserve is limited due to HIV-related marrow impairment and concomitant medication, particularly AZT.
2. Patients are particularly radiosensitive and this may be a severe problems if mucous membranes are treated and may justify palliative chemotherapy where RT would be the treatment of choice in HIV-ve patients.
3. The prognosis of HIV-related cerebral lymphoma is particularly poor.
Further general information Your doctors and specialist nurses are in an ideal position to give you relevant information on your disease and treatment as they know your individual circumstances. Cancerbackup has a help line (0808 800 1234) and a prize winning video available in English, Italian, Urdu, Bengali, Gujarati & Hindi explaining Radiotherapy & Chemotherapy. Cancernet.co.uk has over 500 pages describing cancer, its management, practical tips and tool which patients, their carers and their doctors have found helpful during the cancer journey.