title: Primary sacral non-germinal center type diffuse large B-cell lymphoma with MYC translocation: a case report and a review of the literature.
abstract: An 85-year-old man presented with pain and numbness in the left buttock, and physical examination revealed an approximately 7 cm mass extending from the first to the third sacral vertebrae; biopsy of the mass led to the diagnosis of CD10-negative, BCL6-weakly positive, MUM1-positive, non-germinal center (non-GC) type diffuse large B-cell lymphoma (DLBCL). Furthermore, serological testing showed negative results for Epstein-Barr virus (EBV) infection, and fluorescence in situ hybridization (FISH) revealed a MYC translocation. Radiographs showed no remarkable osteolytic bone destruction, and the patient was staged with Stage IAE. After 8 cycles of rituximab therapy and 6 cycles of CHOP therapy, complete remission has been maintained until now, approximately 1 year after the treatment. Primary sacral lymphoma is very rare, with only 6 reported cases, including the present one. A review of the reported cases revealed that the disease predominantly affects elderly men, is usually non-GC-type DLBCL and stage IAE, measures approximately 2-7 cm in diameter in general, and does not show early recurrence after chemotherapy or chemoradiotherapy. There is no report in the literature yet of primary sacral DLBCL with MYC translocation, and this is the first case report. On the other hand, 35 cases of CD10-negative DLBCL with MYC translocation, including the present one, have been reported, and a review of the reported cases showed that the disease predominantly affects Asians, middle-aged or elderly men, shows positivity for either BCL6 or MUM1 and negativity for EBV, and has a high international prognostic index and poor prognosis.
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Asami
authors.initials: A
authors.lastname: Shimada
authors.forename: Kei-Ji
authors.initials: KJ
authors.lastname: Sugimoto
authors.forename: Mutsumi
authors.initials: M
authors.lastname: Wakabayashi
authors.forename: Hidenori
authors.initials: H
authors.lastname: Imai
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Sekiguchi
authors.forename: Noriko
authors.initials: N
authors.lastname: Nakamura
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Sawada
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ota
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
authors.forename: Masaaki
authors.initials: M
authors.lastname: Noguchi
mesh_terms: D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics*; D001706:Biopsy; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D016403:Lymphoma, Large B-Cell, Diffuse / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D010641:Phenotype; D049268:Positron-Emission Tomography; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D012074:Remission Induction; D012447:Sacrum / Q000473:pathology; D013125:Spinal Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; C489427:MYC protein, human; D016271:Proto-Oncogene Proteins c-myc
keywords: CD10-negative; MYC; Primary sacral lymphoma; non-germinal center DLBCL; paraffin-embedded tissue section-fluorescence in-situ hybridization
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