Skip to main content

Advertisement

SpringerLink
Log in

A rare case of paediatric primary central nervous system lymphoma treated with high-dose methotrexate and rituximab-based chemoimmunotherapy and whole brain radiotherapy followed by tumour bed boost with three-dimensional conformal radiation technique

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Primary central nervous system lymphomas (PCNSL) are rare in the paediatric population.

Clinical case

A 12-year-old boy presented to our clinic with complaints of multiple episodes of generalised tonic-clonic seizures for 1 year and gradual loss of vision in both eyes for 3 months. Baseline magnetic resonance imaging (MRI) of the brain showed a large (7.2 × 7 cm) enhancing soft tissue lesion in the right frontal lobe causing mass effect and midline shift. With a radiological diagnosis of supratentorial primitive neuroectodermal tumour, he underwent subtotal resection of tumour. The post-operative histopathology revealed diffuse large B cell lymphoma (DLBCL). Systemic lymphoma workup was essentially normal. He received five cycles of chemoimmunotherapy with rituximab, high-dose methotrexate (HDMTX), vincristine and procarbazine and had complete radiological response (CR). This was followed by whole brain radiotherapy (WBRT) to a dose of 36 Gy in 20 fractions and sequential tumour bed boost to a dose of 9 Gy in 5 fractions by three-dimensional conformal technique. Subsequently, he received two cycles of consolidation chemotherapy with high-dose cytarabine. At completion of treatment, 3 and 6 months thereafter, MRI brain showed CR. At last follow-up visit, 13 months from the date of diagnosis, he was disease-free and asymptomatic with the exception of dimness of vision in both eyes due to long-standing bilateral optic atrophy.

Conclusion

This report highlights the fact that paediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy followed by consolidation with conformal WBRT and tumour bed boost. Lack of awareness of this rare entity may lead to diagnostic delay and potential ramifications as exemplified by chronic atrophic papilloedema and visual loss in the illustrative case.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Ricard D, Idbaih A, Ducray F, Lahutte M, Hoang-Xuan K, Delattre J-Y (2012) Primary brain tumours in adults. Lancet 379(9830):1984–1996

    Article  PubMed  Google Scholar 

  2. Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ (2011) Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer 105(9):1414–1418

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  3. DeAngelis LM, Seiferheld W, Schold SC, Fisher B, Schultz CJ, Radiation Therapy Oncology Group Study 93-10 (2002) Combination chemotherapy and radiotherapy for primary central nervous system lymphoma: Radiation Therapy Oncology Group Study 93-10. J Clin Oncol 20(24):4643–4648

    Article  PubMed  Google Scholar 

  4. Abla O, Weitzman S, Blay J-Y, O'Neill BP, Abrey LE, Neuwelt E, Doolittle ND, Baehring J, Pradhan K, Martin SE, Guerrera M, Shah S, Ghesquieres H, Silver M, Betensky RA, Batchelor T (2011) Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG). Clin Cancer Res 17(2):346–352

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  5. Thorer H, Zimmermann M, Makarova O, Oschlies I, Klapper W, Lang P, von Stackelberg A, Fleischhack G, Worch J, Juergens H, Woessmann W, Reiter A, Burkhardt B (2014) Primary central nervous system lymphoma in children and adolescents: low relapse rate after treatment according to Non-Hodgkin-Lymphoma Berlin-Frankfurt-Münster protocols for systemic lymphoma. Haematologica 99(11):e238–e241

    Article  PubMed  PubMed Central  Google Scholar 

  6. Yoon JH, Kang HJ, Kim H, Lee JW, Park JD, Park KD, Shin HY, Ahn HS (2012) Successful treatment of primary central nervous system lymphoma without irradiation in children: single center experience. J Korean Med Sci 27(11):1378–1384

    Article  PubMed  PubMed Central  Google Scholar 

  7. Makino K, Nakamura H, Yano S, Kuratsu J-I (2007) Pediatric primary CNS lymphoma: long term survival after treatment with radiation monotherapy. Acta Neurochir 149(3):295–297 discussion 297-298

    Article  PubMed  CAS  Google Scholar 

  8. Kai Y, Kuratsu J, Ushio Y (1998) Primary malignant lymphoma of the brain in childhood. Neurol Med Chir (Tokyo) 38(4):232–237

    Article  CAS  Google Scholar 

  9. Benson R, Mallick S, Purkait S et al (2016) Primary pediatric mid-brain lymphoma: report of a rare pediatric tumor in a rare location. World J Clin Cases 4(12):419–422

    Article  PubMed  PubMed Central  Google Scholar 

  10. Oeffinger KC, Hudson MM (2004) Long-term complications following childhood and adolescent cancer: foundations for providing risk-based health care for survivors. CA Cancer J Clin 54(4):208–236

    Article  PubMed  Google Scholar 

  11. Thiel E, Korfel A, Martus P, Kanz L, Griesinger F, Rauch M, Röth A, Hertenstein B, von Toll T, Hundsberger T, Mergenthaler HG, Leithäuser M, Birnbaum T, Fischer L, Jahnke K, Herrlinger U, Plasswilm L, Nägele T, Pietsch T, Bamberg M, Weller M (2010) High-dose methotrexate with or without whole brain radiotherapy for primary CNS lymphoma (G-PCNSL-SG-1): a phase 3, randomised, non-inferiority trial. Lancet Oncol 11(11):1036–1047

    Article  PubMed  CAS  Google Scholar 

  12. Ferreri AJM, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G, International Extranodal Lymphoma Study Group (IELSG) (2016) Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial. Lancet Haematol 3(5):e217–e227

    Article  PubMed  Google Scholar 

  13. Shah GD, Yahalom J, Correa DD, Lai RK, Raizer JJ, Schiff D, LaRocca R, Grant B, DeAngelis LM, Abrey LE (2007) Combined immunochemotherapy with reduced whole-brain radiotherapy for newly diagnosed primary CNS lymphoma. J Clin Oncol 25(30):4730–4735

    Article  PubMed  CAS  Google Scholar 

  14. Morris PG, Correa DD, Yahalom J, Raizer JJ, Schiff D, Grant B, Grimm S, Lai RK, Reiner AS, Panageas K, Karimi S, Curry R, Shah G, Abrey LE, DeAngelis LM, Omuro A (2013) Rituximab, methotrexate, procarbazine, and vincristine followed by consolidation reduced-dose whole-brain radiotherapy and cytarabine in newly diagnosed primary CNS lymphoma: final results and long-term outcome. J Clin Oncol 31(31):3971–3979

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  15. Goldman S, Smith L, Anderson JR, Perkins S, Harrison L, Geyer MB, Gross TG, Weinstein H, Bergeron S, Shiramizu B, Sanger W, Barth M, Zhi J, Cairo MS (2013) Rituximab and FAB/LMB 96 chemotherapy in children with stage III/IV B-cell non-Hodgkin lymphoma: a Children’s Oncology Group report. Leukemia 27(5):1174–1177

    Article  PubMed  CAS  Google Scholar 

  16. Goldman S, Smith L, Galardy P (2014) Rituximab with chemotherapy in children and adolescents with central nervous system and/or bone marrow-positive Burkitt lymphoma/leukaemia: a Children’s Oncology Group Report. Br J Haematol 167(3):394–401

    Article  PubMed  PubMed Central  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiotherapy & Oncology, All India Institute of Medical Sciences, New Delhi, 110029, India

    Narayan Adhikari & Ahitagni Biswas

  2. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India

    Sameer Bakhshi

  3. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Gaurav Khanna & Vaishali Suri

Authors
  1. Narayan Adhikari
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ahitagni Biswas
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sameer Bakhshi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gaurav Khanna
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Vaishali Suri
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ahitagni Biswas.

Ethics declarations

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Informed consent

Informed consent was taken from the patient’s guardian before surgery and administration of chemotherapy and radiotherapy.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Adhikari, N., Biswas, A., Bakhshi, S. et al. A rare case of paediatric primary central nervous system lymphoma treated with high-dose methotrexate and rituximab-based chemoimmunotherapy and whole brain radiotherapy followed by tumour bed boost with three-dimensional conformal radiation technique. Childs Nerv Syst 34, 1777–1783 (2018). https://doi.org/10.1007/s00381-018-3807-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-018-3807-9

Keywords

Search

Navigation