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
- 151 Downloads
Primary central nervous system lymphomas (PCNSL) are rare in the paediatric population.
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.
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.
KeywordsPaediatric Primary CNS lymphoma Chemoimmunotherapy Whole brain radiotherapy
Compliance with ethical standards
Conflict of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
Informed consent was taken from the patient’s guardian before surgery and administration of chemotherapy and radiotherapy.
- 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):4643–4648CrossRefPubMedGoogle 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):346–352CrossRefPubMedPubMedCentralGoogle 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–e241CrossRefPubMedPubMedCentralGoogle 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):1036–1047CrossRefPubMedGoogle 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–e227CrossRefPubMedGoogle 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):3971–3979CrossRefPubMedPubMedCentralGoogle 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):1174–1177CrossRefPubMedGoogle Scholar