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Primary cutaneous follicle center lymphoma

MedGen UID:
234180
•Concept ID:
C1333171
•
Neoplastic Process
Synonyms: Crosti's Disease; Crosti's disease; Crosti's lymphoma; Cutaneous Follicle Center Lymphoma; cutaneous follicle center lymphoma; Cutaneous Follicle Centre Lymphoma; cutaneous follicle centre lymphoma; PCFCL; Primary cutaneous follicle center cell lymphoma; Primary Cutaneous Follicle Center Lymphoma; primary cutaneous follicle center lymphoma; Primary cutaneous follicular center B-cell lymphoma; Reticulohistiocytoma of the Dorsum; Reticulohistiocytoma of the dorsum
SNOMED CT: Crosti's lymphoma (404143002); Primary cutaneous follicle center cell lymphoma (419662008); Primary cutaneous follicular center B-cell lymphoma (404143002); Primary cutaneous follicle center lymphoma (404143002); Primary cutaneous follicle center lymphoma (419662008)
 
Monarch Initiative: MONDO:0015814
Orphanet: ORPHA178540

Definition

A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and typically do not ulcerate. The neoplastic cells express pan B cell markers and Bcl-6, and typically lack Bcl-2. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
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
Cutaneous B-cell lymphomas: Update on diagnosis, risk-stratification, and management.
Fava P, Roccuzzo G, Alberti-Violetti S, Grandi V, Pileri A, Pimpinelli N, Berti E, Quaglino P
Presse Med 2022 Mar;51(1):104109. Epub 2022 Jan 11 doi: 10.1016/j.lpm.2022.104109. PMID: 35026390

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

Recent clinical studies

Etiology

Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
What is new about primary cutaneous B cell lymphomas.
Blanchard M, Hoetzenecker W, Blanchard G, Guenova E
Ital J Dermatol Venerol 2023 Jun;158(3):205-216. doi: 10.23736/S2784-8671.23.07572-2. PMID: 37278499
Rituximab Treatment of Primary Cutaneous Follicle Center Lymphoma: A Retrospective Review.
Wang S, Perlmutter JW, Johnston J, Nugent Z, Wiseman M
J Cutan Med Surg 2022 Nov-Dec;26(6):604-612. Epub 2022 Sep 22 doi: 10.1177/12034754221126119. PMID: 36134749Free PMC Article
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
Primary Cutaneous B-cell Lymphomas.
Hope CB, Pincus LB
Clin Lab Med 2017 Sep;37(3):547-574. doi: 10.1016/j.cll.2017.05.009. PMID: 28802500

Diagnosis

Cutaneous B-Cell Lymphomas.
Villasenor-Park J, Chung J, Kim EJ
Hematol Oncol Clin North Am 2024 Oct;38(5):1111-1131. Epub 2024 Jul 23 doi: 10.1016/j.hoc.2024.05.017. PMID: 39048407
Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Follicular lymphoma and marginal zone lymphoma: how many diseases?
Laurent C, Cook JR, Yoshino T, Quintanilla-Martinez L, Jaffe ES
Virchows Arch 2023 Jan;482(1):149-162. Epub 2022 Nov 17 doi: 10.1007/s00428-022-03432-2. PMID: 36394631Free PMC Article
Cutaneous B-Cell Lymphoma.
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672
Primary Cutaneous Follicle Center Lymphoma.
Skala SL, Hristov B, Hristov AC
Arch Pathol Lab Med 2018 Nov;142(11):1313-1321. doi: 10.5858/arpa.2018-0215-RA. PMID: 30407851

Therapy

Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Rituximab Treatment of Primary Cutaneous Follicle Center Lymphoma: A Retrospective Review.
Wang S, Perlmutter JW, Johnston J, Nugent Z, Wiseman M
J Cutan Med Surg 2022 Nov-Dec;26(6):604-612. Epub 2022 Sep 22 doi: 10.1177/12034754221126119. PMID: 36134749Free PMC Article
Cutaneous B-cell lymphomas - pathogenesis, diagnostic workup, and therapy.
Nicolay JP, Wobser M
J Dtsch Dermatol Ges 2016 Dec;14(12):1207-1224. doi: 10.1111/ddg.13164. PMID: 27992127
Primary cutaneous B-cell lymphomas: part II. Therapy and future directions.
Suárez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL
J Am Acad Dermatol 2013 Sep;69(3):343.e1-11; quiz 355-6. doi: 10.1016/j.jaad.2013.06.011. PMID: 23957985
Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management.
Sokol L, Naghashpour M, Glass LF
Cancer Control 2012 Jul;19(3):236-44. doi: 10.1177/107327481201900308. PMID: 22710899

Prognosis

Cutaneous B-Cell Lymphomas.
Villasenor-Park J, Chung J, Kim EJ
Hematol Oncol Clin North Am 2024 Oct;38(5):1111-1131. Epub 2024 Jul 23 doi: 10.1016/j.hoc.2024.05.017. PMID: 39048407
Profiling of Copy Number Alterations Using Low-Coverage Whole-Genome Sequencing Informs Differential Diagnosis and Prognosis in Primary Cutaneous Follicle Center Lymphoma.
Bátai B, Kiss L, Varga L, Nagy Á, Househam J, Baker AM, László T, Udvari A, Horváth R, Nagy T, Csomor J, Szakonyi J, Schneider T, Graham TA, Alpár D, Fitzgibbon J, Szepesi Á, Bödör C
Mod Pathol 2024 May;37(5):100465. Epub 2024 Mar 7 doi: 10.1016/j.modpat.2024.100465. PMID: 38460675Free PMC Article
Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Cutaneous B-Cell Lymphoma.
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672
Primary Cutaneous Follicle Center Lymphoma.
Skala SL, Hristov B, Hristov AC
Arch Pathol Lab Med 2018 Nov;142(11):1313-1321. doi: 10.5858/arpa.2018-0215-RA. PMID: 30407851

Clinical prediction guides

Rituximab Treatment of Primary Cutaneous Follicle Center Lymphoma: A Retrospective Review.
Wang S, Perlmutter JW, Johnston J, Nugent Z, Wiseman M
J Cutan Med Surg 2022 Nov-Dec;26(6):604-612. Epub 2022 Sep 22 doi: 10.1177/12034754221126119. PMID: 36134749Free PMC Article
Navigating the cutaneous B-cell lymphomas: avoiding the rocky shoals.
Jaffe ES
Mod Pathol 2020 Jan;33(Suppl 1):96-106. Epub 2019 Oct 25 doi: 10.1038/s41379-019-0385-7. PMID: 31653979
Primary cutaneous B-cell lymphoma: A single-center 5-year experience.
Jacob LA, Asati V, Lakshmaiah KC, Govind BK, Lokanatha D, Babu SM, Lokesh KN, Rudresh AH, Rajeev LK, Mulchandani NJ, Anand A, Koppaka D, Mysore SN
Indian J Cancer 2018 Apr-Jun;55(2):134-137. doi: 10.4103/ijc.IJC_418_17. PMID: 30604723
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
Primary cutaneous B-cell lymphomas: part II. Therapy and future directions.
Suárez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL
J Am Acad Dermatol 2013 Sep;69(3):343.e1-11; quiz 355-6. doi: 10.1016/j.jaad.2013.06.011. PMID: 23957985

Recent systematic reviews

Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management.
Sokol L, Naghashpour M, Glass LF
Cancer Control 2012 Jul;19(3):236-44. doi: 10.1177/107327481201900308. PMID: 22710899

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

    Reviews

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