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Lymphoepithelioid Variant Peripheral T-Cell Lymphoma

MedGen UID:
301211
•Concept ID:
C1621719
•
Neoplastic Process
Synonyms: Lennert lymphoma; Lennert Lymphoma; Lennert Variant Peripheral T-Cell Lymphoma; Lennert's lymphoma; Lennert's Lymphoma; Lennert's Variant Peripheral T-Cell Lymphoma; Lymphoepithelioid Cell Variant Peripheral T-Cell Lymphoma; Lymphoepithelioid lymphoma; Lymphoepithelioid lymphoma (clinical)
SNOMED CT: Lymphoepithelioid lymphoma (76481008); Lennert's lymphoma (76481008); Lymphoepithelioid lymphoma (clinical) (109976000); Lymphoepithelioid lymphoma (109976000); Lennert's lymphoma (109976000); Lennert lymphoma (76481008)

Definition

A variant of peripheral T-cell lymphoma, not otherwise specified. It is characterized by the presence of neoplastic small lymphocytes infiltrating the lymph nodes in a diffuse and less frequently interfollicular pattern. There is an associated proliferation of epithelioid histiocytes forming confluent clusters. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphoepithelioid Variant Peripheral T-Cell Lymphoma

Recent clinical studies

Etiology

Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases.
Khanlari M, Ramos JC, Sanchez SP, Cho-Vega JH, Amador A, Campuzano-Zuluaga G, Vega F, Chapman JR
Mod Pathol 2018 Jul;31(7):1046-1063. Epub 2018 Feb 15 doi: 10.1038/s41379-018-0037-3. PMID: 29449683Free PMC Article
Expression of follicular helper T cell markers in nodal peripheral T cell lymphomas: a tissue microarray analysis of 162 cases.
Zhan HQ, Li XQ, Zhu XZ, Lu HF, Zhou XY, Chen Y
J Clin Pathol 2011 Apr;64(4):319-24. Epub 2011 Feb 17 doi: 10.1136/jcp.2010.084459. PMID: 21330314
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104
Nodal peripheral T-cell lymphomas and, in particular, their lymphoepithelioid (Lennert's) variant are often derived from CD8(+) cytotoxic T-cells.
Geissinger E, Odenwald T, Lee SS, Bonzheim I, Roth S, Reimer P, Wilhelm M, Müller-Hermelink HK, Rüdiger T
Virchows Arch 2004 Oct;445(4):334-43. Epub 2004 Jul 29 doi: 10.1007/s00428-004-1077-2. PMID: 15480768
Peripheral T-cell lymphomas: histologic, immunohistologic, and clinical characterization.
Weis JW, Winter MW, Phyliky RL, Banks PM
Mayo Clin Proc 1986 Jun;61(6):411-26. doi: 10.1016/s0025-6196(12)61975-2. PMID: 3086632

Diagnosis

Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature.
Yin Y, Liu H, Luo M, Yu G, Yin W, Li P
Diagn Pathol 2023 Feb 3;18(1):12. doi: 10.1186/s13000-023-01297-w. PMID: 36737805Free PMC Article
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104
Cutaneous involvement in the lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified (Lennert lymphoma). Report of a case and review of the literature.
Summers TA Jr, Rush W, Aguilera N, Lupton G
J Cutan Pathol 2009 Oct;36 Suppl 1:25-30. doi: 10.1111/j.1600-0560.2008.01203.x. PMID: 19775391
Lymphoepithelioid cell lymphoma (Lennert's lymphoma). Report of a case with fine needle aspiration cytology.
Vaíllo Vinagre A, Gutiérrez Martín A, Pérez Barrios A, Alberti Masgrau N, Ruiz Liso JM
Acta Cytol 2004 Mar-Apr;48(2):234-8. doi: 10.1159/000326323. PMID: 15085759
Peripheral T-cell lymphomas: histologic, immunohistologic, and clinical characterization.
Weis JW, Winter MW, Phyliky RL, Banks PM
Mayo Clin Proc 1986 Jun;61(6):411-26. doi: 10.1016/s0025-6196(12)61975-2. PMID: 3086632

Therapy

Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature.
Yin Y, Liu H, Luo M, Yu G, Yin W, Li P
Diagn Pathol 2023 Feb 3;18(1):12. doi: 10.1186/s13000-023-01297-w. PMID: 36737805Free PMC Article
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104

Prognosis

Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104
Peripheral T-cell lymphomas: histologic, immunohistologic, and clinical characterization.
Weis JW, Winter MW, Phyliky RL, Banks PM
Mayo Clin Proc 1986 Jun;61(6):411-26. doi: 10.1016/s0025-6196(12)61975-2. PMID: 3086632

Clinical prediction guides

Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104
Implications for differential diagnosis of lung cancer-associated lymphadenopathy in lymphoepithelioid cell lymphoma (Lennert's lymphoma) arising simultaneously with lung cancer: a case report.
Hoshi R, Furuta N, Horai T, Takeuchi K, Ishikawa Y, Satoh Y
Acta Cytol 2010 Mar-Apr;54(2):197-201. doi: 10.1159/000325009. PMID: 20391979
Lymphoepithelioid cell lymphoma (Lennert's lymphoma). Report of a case with fine needle aspiration cytology.
Vaíllo Vinagre A, Gutiérrez Martín A, Pérez Barrios A, Alberti Masgrau N, Ruiz Liso JM
Acta Cytol 2004 Mar-Apr;48(2):234-8. doi: 10.1159/000326323. PMID: 15085759
Lennert's lymphoma: a variant of cytotoxic T-cell lymphoma?
Yamashita Y, Nakamura S, Kagami Y, Hasegawa Y, Kojima H, Nagasawa T, Mori N
Am J Surg Pathol 2000 Dec;24(12):1627-33. doi: 10.1097/00000478-200012000-00006. PMID: 11117783
Angioimmunoblastic lymphadenopathy--type of T-cell lymphoma with a high content of epithelioid cells. Histopathology and comparison with lymphoepithelioid cell lymphoma.
Patsouris E, Noël H, Lennert K
Am J Surg Pathol 1989 Apr;13(4):262-75. doi: 10.1097/00000478-198904000-00002. PMID: 2648876

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