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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
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

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
Primary cutaneous B-cell lymphomas- clinical and histopathologic features, differential diagnosis, and treatment.
Chen ST, Barnes J, Duncan L
Semin Cutan Med Surg 2018 Mar;37(1):49-55. doi: 10.12788/j.sder.2018.014. PMID: 29719020
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: 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
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

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: 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 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
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
Primary cutaneous B-cell lymphomas- clinical and histopathologic features, differential diagnosis, and treatment.
Chen ST, Barnes J, Duncan L
Semin Cutan Med Surg 2018 Mar;37(1):49-55. doi: 10.12788/j.sder.2018.014. PMID: 29719020

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

Supplemental Content

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    Clinical resources

    Practice guidelines

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      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

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

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
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      Related literature resources in PubMed

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