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Primary cutaneous lymphoma

MedGen UID:
220981
•Concept ID:
C1302772
•
Neoplastic Process
Synonyms: cutaneous (skin) lymphoma; Primary Cutaneous Lymphoma; primary cutaneous lymphoma; Primary Skin Lymphoma; primary skin lymphoma
SNOMED CT: Primary cutaneous lymphoma (400001003); Primary cutaneous lymphoma (419392005)
 
Monarch Initiative: MONDO:0018898
Orphanet: ORPHA542

Definition

Cutaneous lymphoma is a heterogeneous entity with respect to its clinical and pathological features, evolutive profile, prognosis, molecular aetiology and response to therapy. These specifications have been taken into account in recent classifications, which have placed particular importance on the prognostic implications of these different entities. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous lymphoma

Professional guidelines

PubMed

Update in Diagnosis and Management of Primary Cutaneous B-Cell Lymphomas.
Krenitsky A, Klager S, Hatch L, Sarriera-Lazaro C, Chen PL, Seminario-Vidal L
Am J Clin Dermatol 2022 Sep;23(5):689-706. Epub 2022 Jul 19 doi: 10.1007/s40257-022-00704-0. PMID: 35854102
Diagnosis and Management of Cutaneous B-cell Lymphoma.
Pinter-Brown LC
Dermatol Clin 2015 Oct;33(4):835-40. doi: 10.1016/j.det.2015.05.003. PMID: 26433853
Staging and management of cutaneous T-cell lymphoma.
Scarisbrick JJ
Clin Exp Dermatol 2006 Mar;31(2):181-6. doi: 10.1111/j.1365-2230.2005.02019.x. PMID: 16487086

Recent clinical studies

Etiology

Recent advances in cutaneous lymphoma-implications for current and future classifications.
Goodlad JR, Cerroni L, Swerdlow SH
Virchows Arch 2023 Jan;482(1):281-298. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03421-5. PMID: 36278991Free PMC Article
Primary cutaneous lymphoma: the 2018 update of the WHO-EORTC classification.
Willemze R
Presse Med 2022 Mar;51(1):104126. Epub 2022 May 13 doi: 10.1016/j.lpm.2022.104126. PMID: 35569698
The clinico-pathological spectrum of primary cutaneous lymphoma other than mycosis fungoides/Sezary syndrome.
Oschlies I, King RL, Dotlic S, Montes-Moreno S, Ponzoni M, Traverse-Glehen A, Calaminici M, Ferry JA, Ott G, Goodlad JR
Virchows Arch 2020 May;476(5):683-699. Epub 2019 Nov 28 doi: 10.1007/s00428-019-02713-7. PMID: 31781845
AEDV Primary Cutaneous Lymphoma Registry.
Estrach T, Servitje O, Ortiz-Romero PL
Actas Dermosifiliogr 2017 Apr;108(3):181-183. doi: 10.1016/j.ad.2017.02.008. PMID: 28347423
Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma.
Olsen EA
Dermatol Clin 2015 Oct;33(4):643-54. doi: 10.1016/j.det.2015.06.001. PMID: 26433839

Diagnosis

Recent advances in cutaneous lymphoma-implications for current and future classifications.
Goodlad JR, Cerroni L, Swerdlow SH
Virchows Arch 2023 Jan;482(1):281-298. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03421-5. PMID: 36278991Free PMC Article
Primary cutaneous lymphoma: the 2018 update of the WHO-EORTC classification.
Willemze R
Presse Med 2022 Mar;51(1):104126. Epub 2022 May 13 doi: 10.1016/j.lpm.2022.104126. PMID: 35569698
Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC.
Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, Schwartz L, Horwitz S, Guitart J, Zic J, Kim YH, Wood GS, Duvic M, Ai W, Girardi M, Gru A, Guenova E, Hodak E, Hoppe R, Kempf W, Kim E, Lechowicz MJ, Ortiz-Romero P, Papadavid E, Quaglino P, Pittelkow M, Prince HM, Sanches JA, Sugaya M, Vermeer M, Zain J, Knobler R, Stadler R, Bagot M, Scarisbrick J
Blood 2022 Aug 4;140(5):419-437. doi: 10.1182/blood.2021012057. PMID: 34758074Free PMC Article
Diagnosis and Management of Cutaneous B-cell Lymphoma.
Pinter-Brown LC
Dermatol Clin 2015 Oct;33(4):835-40. doi: 10.1016/j.det.2015.05.003. PMID: 26433853
Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma.
Olsen EA
Dermatol Clin 2015 Oct;33(4):643-54. doi: 10.1016/j.det.2015.06.001. PMID: 26433839

Therapy

Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC.
Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, Schwartz L, Horwitz S, Guitart J, Zic J, Kim YH, Wood GS, Duvic M, Ai W, Girardi M, Gru A, Guenova E, Hodak E, Hoppe R, Kempf W, Kim E, Lechowicz MJ, Ortiz-Romero P, Papadavid E, Quaglino P, Pittelkow M, Prince HM, Sanches JA, Sugaya M, Vermeer M, Zain J, Knobler R, Stadler R, Bagot M, Scarisbrick J
Blood 2022 Aug 4;140(5):419-437. doi: 10.1182/blood.2021012057. PMID: 34758074Free PMC Article
Brentuximab vedotin in CD30(+) primary cutaneous T-cell lymphomas: a review and analysis of existing data.
Enos TH, Feigenbaum LS, Wickless HW
Int J Dermatol 2017 Dec;56(12):1400-1405. Epub 2017 Aug 1 doi: 10.1111/ijd.13696. PMID: 28762479
Renaissance of Low-Dose Radiotherapy Concepts for Cutaneous Lymphomas.
Dabaja B
Oncol Res Treat 2017;40(5):255-260. Epub 2017 Apr 6 doi: 10.1159/000470845. PMID: 28380490
CD4 + primary cutaneous small/medium-sized pleomorphic T-cell lymphoma: a retrospective case series and review of literature.
James E, Sokhn JG, Gibson JF, Carlson K, Subtil A, Girardi M, Wilson LD, Foss F
Leuk Lymphoma 2015 Apr;56(4):951-7. doi: 10.3109/10428194.2014.938331. PMID: 24996443
Staging and management of cutaneous T-cell lymphoma.
Scarisbrick JJ
Clin Exp Dermatol 2006 Mar;31(2):181-6. doi: 10.1111/j.1365-2230.2005.02019.x. PMID: 16487086

Prognosis

Primary Cutaneous B-Cell Lymphomas with Large Cell Morphology: A Practical Review.
Ronchi A, Vitiello P, D'Abbronzo G, Caccavale S, Argenziano G, Sica A, Alfano R, Savarese G, Berretta M, Cozzolino I, Franco R
Int J Mol Sci 2023 Mar 25;24(7) doi: 10.3390/ijms24076204. PMID: 37047176Free PMC Article
Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC.
Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, Schwartz L, Horwitz S, Guitart J, Zic J, Kim YH, Wood GS, Duvic M, Ai W, Girardi M, Gru A, Guenova E, Hodak E, Hoppe R, Kempf W, Kim E, Lechowicz MJ, Ortiz-Romero P, Papadavid E, Quaglino P, Pittelkow M, Prince HM, Sanches JA, Sugaya M, Vermeer M, Zain J, Knobler R, Stadler R, Bagot M, Scarisbrick J
Blood 2022 Aug 4;140(5):419-437. doi: 10.1182/blood.2021012057. PMID: 34758074Free PMC Article
Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations.
Cocks M, Porcu P, Wick MR, Gru AA
Surg Pathol Clin 2019 Sep;12(3):783-803. doi: 10.1016/j.path.2019.03.006. PMID: 31352988
Aggressive cutaneous T-cell lymphomas.
Junkins-Hopkins Md JM
Semin Diagn Pathol 2017 Jan;34(1):44-59. Epub 2016 Dec 24 doi: 10.1053/j.semdp.2016.11.004. PMID: 28187925
Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma.
Olsen EA
Dermatol Clin 2015 Oct;33(4):643-54. doi: 10.1016/j.det.2015.06.001. PMID: 26433839

Clinical prediction guides

The role of (18)F-FDG PET/CT in primary cutaneous lymphoma: an educational review.
McDonald EO, Amanullah AA, Park PSU, Song W, Werner TJ, Alavi A, Revheim ME
Ann Nucl Med 2023 Jun;37(6):328-348. Epub 2023 Apr 24 doi: 10.1007/s12149-023-01830-3. PMID: 37095393Free PMC Article
Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years.
Falkenhain-López D, Muniesa C, Estrach MT, Morillo-Andújar M, Peñate Y, Acebo E, Pujol RM, García-Muret MP, Machan S, Medina S, Botella-Estrada R, Fernández de Misa R, Blanes M, Flórez A, Pérez-Paredes G, Izu R, Yanguas I, Silva-Díaz E, Pérez-Ferriols A, Prieto-Torres L, Zayas A, Parera-Amer ME, Pérez A, Aspe L, Román C, Sánchez-Caminero MP, Bassas-Vila J, Domínguez-Auñón JD, Calzado L, Navedo M, Ortiz-Prieto A, Servitje O, Polo-Rodríguez I, Torres I, Hernández-Hernández MN, Mitxelena-Eceiza J, García-Vázquez A, García-Doval I, Ortiz-Romero PL
Actas Dermosifiliogr 2023 Apr;114(4):291-298. Epub 2022 Dec 15 doi: 10.1016/j.ad.2022.11.010. PMID: 36529273
Primary cutaneous indolent B-cell lymphomas - a retrospective multicenter analysis and a review of literature.
Olszewska-Szopa M, Sobas M, Laribi K, Bao Perez L, Drozd-Sokołowska J, Subocz E, Joks M, Zduniak K, Gajewska M, de Nalecz AK, Romejko-Jarosińska J, Kumiega B, Waszczuk-Gajda A, Wróbel T, Czyz A
Acta Oncol 2021 Oct;60(10):1361-1368. Epub 2021 Aug 4 doi: 10.1080/0284186X.2021.1956689. PMID: 34346830
Aggressive cutaneous T-cell lymphomas.
Junkins-Hopkins Md JM
Semin Diagn Pathol 2017 Jan;34(1):44-59. Epub 2016 Dec 24 doi: 10.1053/j.semdp.2016.11.004. PMID: 28187925
Diagnosis and Management of Cutaneous B-cell Lymphoma.
Pinter-Brown LC
Dermatol Clin 2015 Oct;33(4):835-40. doi: 10.1016/j.det.2015.05.003. PMID: 26433853

Recent systematic reviews

The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series.
Surmanowicz P, Doherty S, Sivanand A, Parvinnejad N, Deschenes J, Schneider M, Hardin J, Gniadecki R
Dermatology 2021;237(4):618-628. Epub 2020 Dec 16 doi: 10.1159/000511473. PMID: 33326960
Borrelia burgdorferi in primary cutaneous lymphomas: a systematic review and meta-analysis.
Travaglino A, Varricchio S, Pace M, Russo D, Picardi M, Baldo A, Staibano S, Mascolo M
J Dtsch Dermatol Ges 2020 Dec;18(12):1379-1384. Epub 2020 Oct 8 doi: 10.1111/ddg.14289. PMID: 33029842

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