PMID- 30719810 OWN - NLM STAT- MEDLINE DCOM- 20190709 LR - 20190709 IS - 1440-1827 (Electronic) IS - 1320-5463 (Linking) VI - 69 IP - 3 DP - 2019 Mar TI - Anaplastic large cell lymphoma with TP63 rearrangement: A dismal prognosis. PG - 155-159 LID - 10.1111/pin.12758 [doi] AB - Anaplastic large cell lymphoma (ALCL) with TP63 rearrangement is a new entity and has the most dismal prognosis in all types of ALCL. This might be due to the resulting fusion protein having N-terminal truncated p63 with high oncogenic ability. Since this N-terminal domain has the function of tumor suppressor activity, the mechanism for high oncogenic capacity is thought to be the dominant negative function. Here, we report two ALCL cases with TP63 rearrangement that was each given too short a prognosis (Case 1 and 2: four and six months) in spite of intensive treatment. Immunohistochemically, p63 was highly expressed, and a sprit signal was detected using a TP63 break apart fluorescence in situ hybridization (FISH) in each case. Additionally, a poor prognostic marker of ALCL, all cytotoxic molecules (TIA-1, Granzyme B, and Perforin) were also expressed in almost all ALCL cells. Taken together, we suggest that not only the dominant negative function of N-truncated p63 but also the effect of cytotoxic molecules may influence the dismal prognosis of ALCL with TP63 rearrangement. CI - (c) 2019 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd. FAU - Yamashita, Takahisa AU - Yamashita T AD - Department of Pathology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Higashi, Morihiro AU - Higashi M AD - Department of Pathology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Kawano, Ryutaro AU - Kawano R AD - Department of Pathology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Momose, Shuji AU - Momose S AD - Department of Pathology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Tokuhira, Michihide AU - Tokuhira M AD - Department of Hematology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Kizaki, Masahiro AU - Kizaki M AD - Department of Hematology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. FAU - Tamaru, Jun-Ichi AU - Tamaru JI AD - Department of Pathology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama, 3508550, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20190205 PL - Australia TA - Pathol Int JT - Pathology international JID - 9431380 RN - 0 (T-Cell Intracellular Antigen-1) RN - 0 (TIA1 protein, human) RN - 0 (TP63 protein, human) RN - 0 (Transcription Factors) RN - 0 (Tumor Suppressor Proteins) RN - EC 2.7.10.1 (Protein-Tyrosine Kinases) RN - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases) RN - EC 3.4.21.- (Granzymes) SB - IM MH - Aged MH - Female MH - Granzymes MH - Hodgkin Disease/diagnosis/*pathology MH - Humans MH - In Situ Hybridization, Fluorescence/methods MH - Lymphoma, Large-Cell, Anaplastic/diagnosis/*metabolism/*pathology MH - Male MH - Prognosis MH - Protein-Tyrosine Kinases/metabolism MH - Receptor Protein-Tyrosine Kinases/metabolism MH - T-Cell Intracellular Antigen-1/metabolism MH - Transcription Factors/*metabolism MH - Tumor Suppressor Proteins/*metabolism OTO - NOTNLM OT - Anaplastic large cell lymphoma (ALCL) OT - FISH OT - TP63 OT - cytotoxic molecule EDAT- 2019/02/06 06:00 MHDA- 2019/07/10 06:00 CRDT- 2019/02/06 06:00 PHST- 2018/05/17 00:00 [received] PHST- 2018/12/02 00:00 [accepted] PHST- 2019/02/06 06:00 [pubmed] PHST- 2019/07/10 06:00 [medline] PHST- 2019/02/06 06:00 [entrez] AID - 10.1111/pin.12758 [doi] PST - ppublish SO - Pathol Int. 2019 Mar;69(3):155-159. doi: 10.1111/pin.12758. Epub 2019 Feb 5.