Journal of Blood Medicine (Jan 2023)

Successful CNS-Centric Therapeutic Management and Genomic Profiling of Primary Cranial Vault Diffuse Large B-Cell Lymphoma

  • Davis J,
  • Kimbrough EO,
  • Alhaj Moustafa M,
  • Jiang L,
  • Gupta V,
  • Parent E,
  • Tun HW

Journal volume & issue
Vol. Volume 14
pp. 49 – 55


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Jordan Davis,1 ErinMarie O Kimbrough,1 Muhamad Alhaj Moustafa,1 Liuyan Jiang,2 Vivek Gupta,3 Ephraim Parent,3 Han W Tun1 1Division of Hematology and Oncology, Mayo Clinic, Jacksonville, FL, USA; 2Department of Pathology, Mayo Clinic, Jacksonville, FL, USA; 3Department of Radiology, Mayo Clinic, Jacksonville, FL, USACorrespondence: Han W Tun, Division of Hematology and Oncology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL, 32224, USA, Tel +1 904 953 7290, Fax +1 904 953 2315, Email [email protected]: Primary cranial vault lymphoma (PCVL) is a rare lymphoma involving the skull with or without extra- and intracranial extension. Most cases of PCVL are diffuse large B-cell lymphoma (DLBCL). We report a case of primary cranial vault diffuse large B-cell lymphoma (PCV-DLBCL) that was successfully treated with anthracycline-based chemoimmunotherapy (CIT) alternating with central nervous system (CNS)-directed CIT with high-dose methotrexate and high-dose cytarabine. CNS-centric therapy was given for suspected cerebral cortical involvement and presumed elevated risk of CNS recurrence. The patient has remained in complete remission for 4.25 years following treatment. We suggest that PCV-DLBCL is potentially curable with CNS-directed therapy. Additionally, we provide genomic profiling results indicating an indeterminate cell of origin and multiple genetic mutations which are not frequently seen in DLBCL.Keywords: diffuse large B-cell lymphoma, central nervous system, calvarium, chemotherapy, scalp lesions