Abstract
Primary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), is a rare disease that infrequently involves the central nervous system (CNS), and it is even rarer in pediatric patients. Here, we report of a 13-year-old male with primary CNS PTCL-NOS who exhibited a malignant clinical course with recurrence after radiochemotherapy followed by bone marrow transplantation; he died 43 months after diagnosis. Pathology revealed the proliferation of cytotoxic T-cells and clonal T-cell receptor gene rearrangements. Although the optimal therapy for PTCL remains controversial, intensive radiochemotherapy may be required for some patients.
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Weisenburger DD, Savage KJ, Harris NL, Gascoyne RD, Jaffe ES, MacLennan KA, Rüdiger T, Pileri S, Nakamura S, Nathwani B, Campo E, Berger F, Coiffier B, Kim WS, Holte H, Federico M, Au WY, Tobinai K, Armitage JO, Vose JM, International Peripheral T-cell Lymphoma Project (2011) Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood 117:3402–3408
Al Mahmoud R, Weitzman S, Schechter T, Ngan B, Abdelhaleem M, Alexander S (2012) Peripheral T-cell lymphoma in children and adolescents: a single-institution experience. J Pediatr Hematol Oncol 34:611–616
Windsor R, Stiller C, Webb D (2008) Peripheral T-cell lymphoma in childhood: population-based experience in the United Kingdom over 20 years. Pediatr Blood Cancer 50:784–787
Lueth M, Stein H, Spors B, Henze G, Driever PH (2012) First case report of a peripheral T-cell lymphoma, not otherwise specified, of the central nervous system in a child. J Pediatr Hematol Oncol 34:e66–e68
Kato S, Takahashi E, Asano N, Tanaka T, Megahed N, Kinoshita T, Nakamura S (2012) Nodal cytotoxic molecule (CM)-positive Epstein–Barr virus (EBV)-associated peripheral T cell lymphoma (PTCL): a clinicopathological study of 26 cases. Histopathology 61:186–199
van Dongen JJ, Langerak AW, Bruggemann M, Evans PA, Hummel M, Lavender FL, Delabesse E, Davi F, Schuuring E, García-Sanz R, van Krieken JH, Droese J, González D, Bastard C, White HE, Spaargaren M, González M, Parreira A, Smith JL, Morgan GJ, Kneba M, Macintyre EA (2003) Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia 17:2257–2317
Takiyama A, Nishihara H, Tateishi U, Kimura T, Wang L, Marukawa K, Itoh T, Hashino S, Nagashima K, Tanaka S (2008) CNS lymphomatoid granulomatosis with lymph node and bone marrow involvements. Neuropathology 28:640–644
Lee DK, Chung CK, Kim HJ, Kim K, Choe G, Moon CW, Kim DY (2002) Multifocal primary CNS T cell lymphoma of the spinal cord. Clin Neuropathol 21:149–155
Liu D, Schelper RL, Carter DA, Poiesz BJ, Shrimpton AE, Frankel BM, Hutchison RE (2003) Primary central nervous system cytotoxic/suppressor T-cell lymphoma: report of a unique case and review of the literature. Am J Surg Pathol 27:682–688
Demetriades AK, McEvoy AW, Galloway M, Kitchen ND (2003) Peripheral T-cell lymphoma presenting as a solitary vermian mass. Br J Neurosurg 17:473–475
Harder A, Dudel C, Anagnostopoulos I, Hummel M, Brück W (2003) Molecular genetic diagnosis of a primary central nervous system T cell lymphoma. Acta Neuropathol 105:65–68
Ogura R, Aoki H, Natsumeda M, Shimizu H, Kobayashi T, Saito T, Takizawa J, Okamoto K, Hasegawa G, Umezu H, Ohshima K, Takahashi H, Fujii Y, Kakita A (2013) Epstein–Barr virus-associated primary central nervous system cytotoxic T-cell lymphoma. Neuropathology 33:436–441
Kato Y, Hayashi T, Kawai-Masaoka A, Ichimura T, Sasaki A, Uchino A, Nagamine Y, Tanahashi N (2014) Primary central nervous system cytotoxic T-cell lymphoma mimicking demyelinating disease. Intern Med 53:1197–1200
Kato S, Asano N, Miyata-Takata T, Takata K, Elsayed AA, Satou A, Takahashi E, Kinoshita T, Nakamura S (2015) T-cell receptor (TCR) phenotype of nodal Epstein–Barr virus (EBV)-positive cytotoxic T-cell lymphoma (CTL): a clinicopathologic study of 39 cases. Am J Surg Pathol 39:462–471
El-Mallawany NK, Frazer JK, Van Vlierberghe P, Ferrando AA, Perkins S, Lim M, Chu Y, Cairo MS (2012) Pediatric T- and NK-cell lymphomas: new biologic insights and treatment strategies. Blood Cancer J 2:e65
Bataille B, Delwail V, Menet E, Vandermarcq P, Ingrand P, Wager M, Guy G, Lapierre F (2000) Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg 92:261–266
Ferreri AJ, Reni M, Pasini F, Calderoni A, Tirelli U, Pivnik A, Aondio GM, Ferrarese F, Gomez H, Ponzoni M, Borisch B, Berger F, Chassagne C, Iuzzolino P, Carbone A, Weis J, Pedrinis E, Motta T, Jouvet A, Barbui T, Cavalli F, Blay JY (2002) A multicenter study of treatment of primary CNS lymphoma. Neurology 58:1513–1520
Lymphoma Study Group of Japanese Pathologists (2000) The world health organization classification of malignant lymphomas in Japan: incidence of recently recognized entities. Pathol Int 50:696–702
Choi JS, Nam DH, Ko YH, Seo JW, Choi YL, Suh YL, Ree HJ (2003) Primary central nervous system lymphoma in Korea: comparison of B- and T-cell lymphomas. Am J Surg Pathol 27:919–928
Shibamoto Y, Ogino H, Suzuki G, Takemoto M, Araki N, Isobe K, Tsuchida E, Nakamura K, Kenjo M, Suzuki K, Hosono M, Tokumaru S, Ishihara S, Kato E, Ii N, Hayabuchi N (2008) Primary central nervous system lymphoma in Japan: changes in clinical features, treatment, and prognosis during 1985–2004. Neuro Oncol 10:560–568
Asano N, Suzuki R, Kagami Y, Ishida F, Kitamura K, Fukutani H, Morishima Y, Takeuchi K, Nakamura S (2005) Clinicopathologic and prognostic significance of cytotoxic molecule expression in nodal peripheral T-cell lymphoma, unspecified. Am J Surg Pathol 29:1284–1293
Iqbal J, Weisenburger DD, Greiner TC, Vose JM, McKeithan T, Kucuk C, Geng H, Deffenbacher K, Smith L, Dybkaer K, Nakamura S, Seto M, Delabie J, Berger F, Loong F, Au WY, Ko YH, Sng I, Armitage JO, Chan WC (2010) Molecular signatures to improve diagnosis in peripheral T-cell lymphoma and prognostication in angioimmunoblastic T-cell lymphoma. Blood 115:1026–1036
Lim T, Kim SJ, Kim K, Lee JI, Lim do H, Lee DJ, Baek KK, Lee HY, Han B, Uhm JE, Ko YH, Kim WS (2011) Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes. Ann Hematol 90:1391–1398
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This work was supported by Japan Society for the Promotion of Science (JSPS) Grant-in-Aid for Scientific Research (C) (24590478) to Hiroyuki Momota.
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Momota, H., Kato, S., Fujii, M. et al. Primary peripheral T-cell lymphoma, not otherwise specified, of the central nervous system in a child. Brain Tumor Pathol 32, 281–285 (2015). https://doi.org/10.1007/s10014-015-0229-1
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DOI: https://doi.org/10.1007/s10014-015-0229-1