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Int J Clin Exp Pathol 2013;6(9):1919-1928

Case Report
Primary sacral non-germinal center type diffuse large B-cell lymphoma with MYC
translocation: a case report and a review of the literature

Asami Shimada, Kei-Ji Sugimoto, Mutsumi Wakabayashi, Hidenori Imai, Yasunobu Sekiguchi, Noriko Nakamura, Tomohiro Sawada, Yasunori
Ota, Norio Komatsu, Masaaki Noguchi

Department of Hematology, Juntendo University Urayasu Hospital, Urayasu, Japan; Department of Hematology, juntendo university school of
medicine, Tokyo, Japan; Department of Clinical Laboratory, Juntendo University Urayasu Hospital, Urayasu, Japan; Department of Pathology,
Research Hospital, the Institute of Medical Science, the University of Tokyo, Tokyo, Japan

Received July 16, 2013; Accepted July 30, 2013; Epub August 15, 2013; Published September 1, 2013

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. (IJCEP1307026).

Keywords: Primary sacral lymphoma, non-germinal center DLBCL, CD10-negative, MYC, paraffin-embedded tissue section-fluorescence
in-situ hybridization

Address correspondence to: Dr. Kei-Ji Sugimoto, Department of Hematology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu,
279-0021, Japan. Tel: +81-47-353-3111; Fax: +81-47-381-5054; E-mail: keijis@juntendo.ac.jp