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Cutaneous Mature T-Cell and NK-Cell Neoplasm

MedGen UID:: 317556
•Concept ID:: C1707552
•: Neoplastic Process

Definition

A group of neoplasms composed of T-lymphocytes with a mature (peripheral/post-thymic) immunophenotypic profile and/or NK-cells affecting the skin. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCutaneous Mature T-Cell and NK-Cell Neoplasm

Disease
- Disorder by Site
  - Hematopoietic and Lymphoid System Disorder
    - Hematologic disorder
      - Hematopoietic and lymphoid cell neoplasm
        Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
        Cutaneous Mature T-Cell and NK-Cell Neoplasm
        Cutaneous Adult T-Cell Leukemia/Lymphoma
        Cutaneous Angioimmunoblastic T-Cell Lymphoma
        Cutaneous Nasal Type Extranodal NK/T-Cell Lymphoma
        Lymphomatoid papulosis
        Lymphomatoid Papulosis with DUSP22-IRF4 Gene Rearrangement
        Type A Lymphomatoid Papulosis
        Type B Lymphomatoid Papulosis
        Type C Lymphomatoid Papulosis
        Type D Lymphomatoid Papulosis
        Type E Lymphomatoid Papulosis
        Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder
        Borderline Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder
        Anaplastic Large Cell Lymphoma, Lymphomatoid Papulosis-Like Histology
        Primary cutaneous anaplastic large cell lymphoma
        Adult Primary Cutaneous Anaplastic Large Cell Lymphoma
        Childhood Primary Cutaneous Anaplastic Large Cell Lymphoma
        Recurrent Primary Cutaneous Anaplastic Large Cell Lymphoma
        Refractory Primary Cutaneous Anaplastic Large Cell Lymphoma
        Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder
        Primary cutaneous T-cell lymphoma
        Advanced Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Early Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Mycosis fungoides
        Mycosis Fungoides by AJCC v7 Stage
        Pagetoid Reticulosis
        Recurrent Mycosis Fungoides
        Refractory Mycosis Fungoides
        Transformed Mycosis Fungoides
        Mycosis fungoides variant
        Folliculotropic mycosis fungoides
        Granulomatous slack skin disease
        Primary Cutaneous Peripheral T-Cell Lymphoma, Rare Subtype
        Primary Cutaneous Acral CD8-Positive T-Cell Lymphoma
        Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
        Primary cutaneous gamma-delta t-cell lymphoma
        Recurrent Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Recurrent Subcutaneous Panniculitis-Like T-Cell Lymphoma
        Refractory Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Refractory Subcutaneous Panniculitis-Like T-Cell Lymphoma
        Sezary syndrome
        Recurrent Sezary Syndrome
        Refractory Sezary Syndrome
        Subcutaneous panniculitis-like T-cell lymphoma

Professional guidelines

PubMed

Treatment algorithms for mature T-cell and natural killer-cell neoplasms.

Tse E, Kwong YL
Future Oncol 2011 Sep;7(9):1101-12. doi: 10.2217/fon.11.84. PMID: 21919697

Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma).

Dearden CE, Johnson R, Pettengell R, Devereux S, Cwynarski K, Whittaker S, McMillan A; British Committee for Standards in Haematology
Br J Haematol 2011 May;153(4):451-85. Epub 2011 Apr 11 doi: 10.1111/j.1365-2141.2011.08651.x. PMID: 21480860

Classification and treatment of rare and aggressive types of peripheral T-cell/natural killer-cell lymphomas of the skin.

Rezania D, Sokol L, Cualing HD
Cancer Control 2007 Apr;14(2):112-23. doi: 10.1177/107327480701400204. PMID: 17387296

See all (7)

Recent clinical studies

Etiology

Allogeneic Hematopoietic Stem Cell Transplantation for Mature T/NK-Cell Lymphomas in Children.

Klimentova M, Perminova M, Shelikhova L, Abugova Y, Kobyzeva D, Pershin D, Balashov D, Myakova N, Maschan A, Maschan M
Transplant Cell Ther 2024 Apr;30(4):437.e1-437.e11. Epub 2024 Jan 27 doi: 10.1016/j.jtct.2024.01.077. PMID: 38286354

Transplantation.

Zain J
Cancer Treat Res 2019;176:269-287. doi: 10.1007/978-3-319-99716-2_13. PMID: 30596223

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145

Allogeneic transplantation in T-cell lymphomas.

Schmitz N, Wu HS, Glass B
Semin Hematol 2014 Jan;51(1):67-72. Epub 2013 Nov 28 doi: 10.1053/j.seminhematol.2013.11.010. PMID: 24468318

Treatment algorithms for mature T-cell and natural killer-cell neoplasms.

Tse E, Kwong YL
Future Oncol 2011 Sep;7(9):1101-12. doi: 10.2217/fon.11.84. PMID: 21919697

See all (29)

Diagnosis

Allogeneic Hematopoietic Stem Cell Transplantation for Mature T/NK-Cell Lymphomas in Children.

Expression pattern and diagnostic utility of BCL11B in mature T- and NK-cell neoplasms.

Fang H, Khoury JD, Torres-Cabala CA, Ng SB, Xu J, El Hussein S, Hu S, Vega F, Li S, Tang Z, Tang G, Medeiros LJ, Wang W
Pathology 2022 Dec;54(7):893-899. Epub 2022 Jun 29 doi: 10.1016/j.pathol.2022.04.012. PMID: 35864006

Mature (non-anaplastic, non-cutaneous) T-/NK-cell lymphomas in children, adolescents and young adults: state of the science.

Flower A, Xavier AC, Cairo MS
Br J Haematol 2019 May;185(3):418-435. Epub 2019 Feb 1 doi: 10.1111/bjh.15767. PMID: 30706450

Transplantation.

Zain J
Cancer Treat Res 2019;176:269-287. doi: 10.1007/978-3-319-99716-2_13. PMID: 30596223

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145

See all (29)

Therapy

Treating relapsed/refractory mature T- and NK-cell neoplasms with tislelizumab: a multicenter open-label phase 2 study.

Bachy E, Savage KJ, Huang H, Kwong YL, Gritti G, Zhang Q, Liberati AM, Cao J, Yang H, Hao S, Hu J, Zhou K, Petrini M, Russo F, Zhang H, Sang W, Ji J, Ferreri AJM, Damaj GL, Liu H, Zhang W, Ke X, Ghiggi C, Huang S, Li X, Yao H, Paik J, Novotny W, Zhou W, Zhu H, Zinzani PL
Blood Adv 2023 Aug 22;7(16):4435-4447. doi: 10.1182/bloodadvances.2022009575. PMID: 37276067 Free PMC Article

Mature (non-anaplastic, non-cutaneous) T-/NK-cell lymphomas in children, adolescents and young adults: state of the science.

Flower A, Xavier AC, Cairo MS
Br J Haematol 2019 May;185(3):418-435. Epub 2019 Feb 1 doi: 10.1111/bjh.15767. PMID: 30706450

Transplantation.

Zain J
Cancer Treat Res 2019;176:269-287. doi: 10.1007/978-3-319-99716-2_13. PMID: 30596223

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145

Treatment algorithms for mature T-cell and natural killer-cell neoplasms.

Tse E, Kwong YL
Future Oncol 2011 Sep;7(9):1101-12. doi: 10.2217/fon.11.84. PMID: 21919697

See all (16)

Prognosis

Mature (non-anaplastic, non-cutaneous) T-/NK-cell lymphomas in children, adolescents and young adults: state of the science.

Flower A, Xavier AC, Cairo MS
Br J Haematol 2019 May;185(3):418-435. Epub 2019 Feb 1 doi: 10.1111/bjh.15767. PMID: 30706450

The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms.

Matutes E
Int J Lab Hematol 2018 May;40 Suppl 1:97-103. doi: 10.1111/ijlh.12817. PMID: 29741263

Allogeneic transplantation in T-cell lymphomas.

Schmitz N, Wu HS, Glass B
Semin Hematol 2014 Jan;51(1):67-72. Epub 2013 Nov 28 doi: 10.1053/j.seminhematol.2013.11.010. PMID: 24468318

Treatment algorithms for mature T-cell and natural killer-cell neoplasms.

Tse E, Kwong YL
Future Oncol 2011 Sep;7(9):1101-12. doi: 10.2217/fon.11.84. PMID: 21919697

Pathology and biology of peripheral T-cell lymphomas.

de Leval L, Gaulard P
Histopathology 2011 Jan;58(1):49-68. doi: 10.1111/j.1365-2559.2010.03704.x. PMID: 21261683

See all (26)

Clinical prediction guides

Epidemiology of mature T/NK-cell lymphomas in Germany - A representative cross-sectional study based on SHI claims data.

Assaf C, Dobos G, Zech IM, Doess A, May M, Jadasz J
J Dtsch Dermatol Ges 2023 Nov;21(11):1320-1327. Epub 2023 Oct 16 doi: 10.1111/ddg.15187. PMID: 37845021

Treating relapsed/refractory mature T- and NK-cell neoplasms with tislelizumab: a multicenter open-label phase 2 study.

A randomised controlled trial of long NY-ESO-1 peptide-pulsed autologous dendritic cells with or without alpha-galactosylceramide in high-risk melanoma.

Dasyam N, Sharples KJ, Barrow C, Huang Y, Bauer E, Mester B, Wood CE, Authier-Hall A, Dzhelali M, Ostapowicz T, Kumar R, Lowe J, Maxwell A, Burn OK, Williams GM, Carley SE, Caygill G, Jones J, Chan STS, Hinder VA, Macapagal J, McCusker M, Weinkove R, Brimble MA, Painter GF, Findlay MP, Dunbar PR, Gasser O, Hermans IF
Cancer Immunol Immunother 2023 Jul;72(7):2267-2282. Epub 2023 Mar 7 doi: 10.1007/s00262-023-03400-y. PMID: 36881133 Free PMC Article

Cutaneous lymphoma in Japan, 2012-2017: A nationwide study.

Fujii K, Hamada T, Shimauchi T, Asai J, Fujisawa Y, Ihn H, Katoh N
J Dermatol Sci 2020 Mar;97(3):187-193. Epub 2020 Jan 25 doi: 10.1016/j.jdermsci.2020.01.010. PMID: 32033869

Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma).

See all (17)

MedGen

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Cutaneous Mature T-Cell and NK-Cell Neoplasm

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Practice guidelines

Related information

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