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Tonsillar follicular large B-cell lymphoma with IRF4 rearrangement causing sleep apnoea
  1. Yu-Ting Yu1,
  2. Seiji Sakata2,
  3. Kengo Takeuchi2,
  4. L Jeffrey Medeiros3,
  5. Kung-Chao Chang4
  1. 1Pathology, National Cheng Kung University, Tainan, Taiwan
  2. 2Division of Pathology, Japanese Foundation for Cancer Research, Koto, Tokyo, Japan
  3. 3Hematopathology, UT MD Anderson Cancer Center, Houston, Texas, USA
  4. 4Pathology, National Cheng Kung University Hospital, Tainan, Taiwan
  1. Correspondence to Professor Kung-Chao Chang, Pathology, National Cheng Kung University Hospital, Tainan 70428, Taiwan; changkc{at}mail.ncku.edu.tw

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Clinical question

An 8-year-old boy, without underlying disease, presented to the otolaryngologist with sleep apnoea and snoring. Physical examination showed bilateral tonsillar and adenoid hypertrophies and he underwent bilateral tonsillectomy and adenoidectomy. Review the high quality, interactive digital Aperio slide at http://virtualacp.com/JCPCases/jclinpath-2019-205915/ and consider your diagnosis.

What is your diagnosis?

  1. Follicular hyperplasia.

  2. Paediatric-type follicular lymphoma.

  3. Conventional follicular lymphoma.

  4. CD10-MUM1+follicular lymphoma.

  5. Large B cell lymphoma with IRF4 rearrangement.

Discussion

Pathologically, the follicles in the tonsils are numerous but only a subset is arranged in a back-to-back pattern (online supplementary figure S1). No abnormal follicle is found in the adenoid. These abnormal follicles show attenuated mantle zones, loss of polarity and starry-sky appearance (figure 1A), and are composed predominantly of medium-sized blasts (figure 1A, inset). Immunohistochemically, the cells in the abnormal follicles are positive for CD20, BCL-6 (figure 1B), BCL-2 (figure 1C) and MUM1/IRF4 (figure 1D) with a Ki-67 proliferative index of 20% to 30% (figure 1E). CD10 is however negative. An initial impression of paediatric-type follicular lymphoma was considered but the strong expression of MUM1/IRF4 suggests the alternative entity ─ follicular form of large B-cell lymphoma with IRF4 rearrangement.1 We, thus, performed a detailed fluorescence in situ hybridisation study using three-colour probes of mixed 6p25.3 region (IRF4) dual colour …

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