This is usually a very troublesome situation and the chance of cure is unfortunately low provided she has received adequate frontline therapy. It is also most unusual with isolated relapses in CNS. How was the relapse diagnosed and is it during or after primary treatment? Do you have pathologic confirmation?

As far as I know of no established guidelines or protocols exist. It is extremely important to look for other possible tumor sites and I would do a PET scan.

If it truly is isolated CNS relapse most clinicians agree that it is essential to give some courses of systemic intensive chemotherapy in combination with Rituximab. Many would choose RICE (rituximab+ifosfamide+carboplatin+etoposide). Ofcourse she needs also local therapy and, usually triple it with methotrexate/cytarabine/prednisolone is used. Many would also give additional it therapy with Rituximab. If the effect is poor of it chemo Depocyte may be considered

Finally after evaluating therapy response one must strongly consider cranial irradiation as an additional therapeutic option and some would (especially if other sites are involved) favor consolidation with allogeneic stem cell transplant.

Hope this can be of some help but remember that the evidence for treatment recommendations in this situation is very scant

Good luck

Relapsed/Refractory Burkitt Lymphoma

The best management approach to these patients is not well defined. Most patients in this group respond poorly to salvage therapy, although some patients are reported to have long-term survival.

The salvage regimen typically incorporates chemotherapeutic agents to which the patient has had no previous exposure. The DHAP (dexamethasone at 40 mg PO for 4 d, high-dose cytarabine [Ara-C] at 2000 mg/m2 IV q12h for 2 doses, and cisplatin at 100 mg/m2 IV for 1 dose) regimen is often used as salvage therapy. Among these patients, those whose disease demonstrates some chemosensitivity are then referred for high-dose chemotherapy and autologous stem cell transplantation (SCT)/bone marrow transplantation (or allogeneic SCT in clinical trials). Patients with chemoresistant disease are usually referred for best supportive care (see the Sweetenham et al ).

Burkitt Lymphoma and Burkitt-like Lymphoma Treatment & Management

Author: Ali H Kanbar et al.

http://emedicine.medscape.com/article/1447602-treatment#aw2aab6b6c10aa

Is there a confirmation of t (8:14) from the initial BM in this child? My opinion is treatment should consist of salvage systemic chemo + intrathecal (triple therapy) followed by allogeneic BMT if donor 

Your description suggests possible CSF involvement and this could be a "sanctuary site" recurrence.  Classic treatment for similar Acute Lymphocytic Leukemia patients would add craniospinal irradiation to chemotherapy; studies have documented that high dose Methotrexate and intrathecal treatment may substitute for the radiation therapy in ALL (in a prophylactic situation) but that may not be the case in this patient's situation, with the patient potentially also needing Ara-C intrathecally.  Do you have access to the NCCN Guidelines at www.nccn.org?  You can reference the BURK-2 in NCCN Guidelines Version 4.2014 Burkitt Lymphoma and note that on BURK-A page 1 of 2 in the same that footnote (b) indicates that all regimens for Burkitt Lymphoma include CNS prophylaxis/therapy. Additional information which may be useful is found in http://www.just.edu.jo/DIC/ClinicGuidlines/Non%20Hodgkin%20Lymphoma%20(Burkitt%20Lymphoma).pdf (see link below) where one could consider using a high-risk schedule appropriate for involvement of the CNS. In ALL the dose of 18 Gy cumulative dose to the cranium is used (1.8 Gy X 10), but this situation may unfortunately require a higher than prophylactic dose as the disease may be more radio-resistant than ALL and is present in a grossly-detectable amount, apparently.  Such treatment would require systemic chemotherapy anyway and the radiotherapy may well cause problems with spinal development, increased risk for second malignancies, etc.  Dr. Abrahamson's response appears appropriate above.

http://www.just.edu.jo/DIC/ClinicGuidlines/Non%20Hodgkin%20Lymphoma%20(Burkitt%20Lymphoma).pdf

I would suggest radiation to the leptomeningeal sites (15-20 Gy in 10 fractions) along with intrathecal MTX alternating with ARA-C (intially twice weekly until CSF clears, then once weekly for 4 weeks then evey other week for 4 times). For salvage chemotherapy, it depends on what the patient received initially in the front setting: Did she recieve ALL inspired therapy? If the paln is to consolidate with allogeneic SCT then 1-2 cycles or RICE, R-DHAP or R-GEMOX as a bridge to transplant is reasonable, but transplant should be seriously considered in this case.

I agree that the data is scant. I hope this answer is helpful.