Shimada Asami, Sugimoto Kei-Ji, Wakabayashi Mutsumi, Imai Hidenori, Sekiguchi Yasunobu, Nakamura Noriko, Sawada Tomohiro, Ota Yasunori, Komatsu Norio, Noguchi Masaaki
Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
Int J Clin Exp Pathol. 2013 Aug 15;6(9):1919-28. eCollection 2013.
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.
一名85岁男性因左臀部疼痛和麻木就诊，体格检查发现一个约7 cm的肿块，从第一骶椎延伸至第三骶椎；对该肿块进行活检后诊断为CD10阴性、BCL6弱阳性、MUM1阳性的非生发中心（非GC）型弥漫性大B细胞淋巴瘤（DLBCL）。此外，血清学检测显示EB病毒（EBV）感染呈阴性，荧光原位杂交（FISH）显示存在MYC易位。X线片显示无明显溶骨性骨质破坏，患者分期为IAE期。经过8个周期的利妥昔单抗治疗和6个周期的CHOP治疗后，至今已维持完全缓解，治疗后约1年。原发性骶骨淋巴瘤非常罕见，包括本病例在内仅报道过6例。对已报道病例的回顾显示，该病主要影响老年男性，通常为非GC型DLBCL且为IAE期，一般直径约2 - 7 cm，化疗或放化疗后无早期复发。文献中尚无原发性骶骨DLBCL伴MYC易位的报道，这是首例病例报告。另一方面，包括本病例在内，已报道35例CD10阴性DLBCL伴MYC易位，对已报道病例的回顾显示，该病主要影响亚洲人、中年或老年男性，BCL6或MUM1呈阳性，EBV呈阴性，国际预后指数高且预后差。
