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Primary cutaneous marginal zone B-cell lymphoma

MedGen UID:
698594
•Concept ID:
C1275321
•
Neoplastic Process
Synonyms: C-MALT; cutaneous Immunocytoma; Marginal Zone B Cell Lymphoma of Skin; marginal zone B cell lymphoma of skin; Marginal Zone B Cell Lymphoma of the Skin; marginal zone B cell lymphoma of the skin; PCMZL; Primary Cutaneous Marginal Zone B Cell Lymphoma of Mucosa-Associated Lymphoid Tissue; Primary cutaneous marginal zone B cell lymphoma of mucosa-associated lymphoid tissue; primary cutaneous marginal zone B-cell lymphoma; Primary Cutaneous Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue; Primary cutaneous marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue; Primary Cutaneous Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue; Primary Cutaneous Marginal Zone Lymphoproliferative Disorder; SALT lymphoma; SALT Lymphoma; salt lymphoma; SALT type B-cell lymphoma; Skin-associated lymphoid tissue lymphoma; Skin-Associated Lymphoid Tissue Lymphoma; skin-associated lymphoid tissue lymphoma; Skin-associated lymphoid tissue type B-cell lymphoma
SNOMED CT: Primary cutaneous marginal zone B-cell lymphoma (404140004); SALT type B-cell lymphoma (404140004); Skin-associated lymphoid tissue type B-cell lymphoma (404140004); SALT lymphoma (128803008); Skin-associated lymphoid tissue lymphoma (128803008); Primary cutaneous marginal zone B-cell lymphoma (420028002)
 
Monarch Initiative: MONDO:0015813
Orphanet: ORPHA178536

Definition

A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous marginal zone B-cell lymphoma
Follow this link to review classifications for Primary cutaneous marginal zone B-cell lymphoma in Orphanet.

Professional guidelines

PubMed

Recent advances in primary cutaneous B-cell lymphomas.
Vermeer MH, Willemze R
Curr Opin Oncol 2014 Mar;26(2):230-6. doi: 10.1097/CCO.0000000000000060. PMID: 24463347
Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review.
Fernández-Guarino M, Ortiz-Romero PL, Fernández-Misa R, Montalbán C
Actas Dermosifiliogr 2014 Jun;105(5):438-45. Epub 2013 Mar 26 doi: 10.1016/j.ad.2012.10.021. PMID: 23540593
Primary cutaneous B-cell lymphoma: classification and treatment.
Willemze R
Curr Opin Oncol 2006 Sep;18(5):425-31. doi: 10.1097/01.cco.0000239879.31463.42. PMID: 16894288

Curated

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

Recent clinical studies

Etiology

Pediatric Cutaneous Hematologic Disorders: Cutaneous Lymphoma and Leukemia Cutis-Experience of a Tertiary-Care Pediatric Institution and Review of the Literature.
Colmant C, Demers MA, Hatami A, Coulombe J, McCuaig CC, Piram M, Marcoux D, Kokta V, Powell J
J Cutan Med Surg 2022 Jul-Aug;26(4):349-360. Epub 2022 Feb 15 doi: 10.1177/12034754221077694. PMID: 35167768
Dermoscopic and trichoscopic features of primary cutaneous lymphomas - systematic review.
Sławińska M, Sokołowska-Wojdyło M, Olszewska B, Nowicki RJ, Sobjanek M, Zalaudek I
J Eur Acad Dermatol Venereol 2021 Jul;35(7):1470-1484. Epub 2021 Apr 27 doi: 10.1111/jdv.17219. PMID: 33710688
Recent advances in primary cutaneous B-cell lymphomas.
Vermeer MH, Willemze R
Curr Opin Oncol 2014 Mar;26(2):230-6. doi: 10.1097/CCO.0000000000000060. PMID: 24463347
Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients.
Servitje O, Muniesa C, Benavente Y, Monsálvez V, Garcia-Muret MP, Gallardo F, Domingo-Domenech E, Lucas A, Climent F, Rodriguez-Peralto JL, Ortiz-Romero PL, Sandoval J, Pujol RM, Estrach MT
J Am Acad Dermatol 2013 Sep;69(3):357-65. Epub 2013 Jun 21 doi: 10.1016/j.jaad.2013.04.047. PMID: 23796549
Primary cutaneous marginal zone lymphoma.
Dalle S, Thomas L, Balme B, Dumontet C, Thieblemont C
Crit Rev Oncol Hematol 2010 Jun;74(3):156-62. Epub 2009 Oct 12 doi: 10.1016/j.critrevonc.2009.09.003. PMID: 19819717

Diagnosis

Pediatric Cutaneous Hematologic Disorders: Cutaneous Lymphoma and Leukemia Cutis-Experience of a Tertiary-Care Pediatric Institution and Review of the Literature.
Colmant C, Demers MA, Hatami A, Coulombe J, McCuaig CC, Piram M, Marcoux D, Kokta V, Powell J
J Cutan Med Surg 2022 Jul-Aug;26(4):349-360. Epub 2022 Feb 15 doi: 10.1177/12034754221077694. PMID: 35167768
Dermoscopic and trichoscopic features of primary cutaneous lymphomas - systematic review.
Sławińska M, Sokołowska-Wojdyło M, Olszewska B, Nowicki RJ, Sobjanek M, Zalaudek I
J Eur Acad Dermatol Venereol 2021 Jul;35(7):1470-1484. Epub 2021 Apr 27 doi: 10.1111/jdv.17219. PMID: 33710688
Complete resolution of primary cutaneous marginal zone B-cell lymphoma on the cheek of a 7-year-old boy with intralesional triamcinolone and tincture of time.
Skaljic M, Cotton CH, Reilly AF, Pillai V, Perman MJ
Pediatr Dermatol 2020 Jan;37(1):228-229. Epub 2019 Oct 30 doi: 10.1111/pde.14028. PMID: 31667886
Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart?
Amitay-Laish I, Tavallaee M, Kim J, Hoppe RT, Million L, Feinmesser M, Fenig E, Wolfe MEL, Hodak E, Kim YH
Br J Dermatol 2017 Apr;176(4):1010-1020. Epub 2017 Feb 20 doi: 10.1111/bjd.14932. PMID: 27501236
Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases.
Servitje O, Gallardo F, Estrach T, Pujol RM, Blanco A, Fernández-Sevilla A, Pétriz L, Peyrí J, Romagosa V
Br J Dermatol 2002 Dec;147(6):1147-58. doi: 10.1046/j.1365-2133.2002.04961.x. PMID: 12452864

Therapy

Complete resolution of primary cutaneous marginal zone B-cell lymphoma on the cheek of a 7-year-old boy with intralesional triamcinolone and tincture of time.
Skaljic M, Cotton CH, Reilly AF, Pillai V, Perman MJ
Pediatr Dermatol 2020 Jan;37(1):228-229. Epub 2019 Oct 30 doi: 10.1111/pde.14028. PMID: 31667886
Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart?
Amitay-Laish I, Tavallaee M, Kim J, Hoppe RT, Million L, Feinmesser M, Fenig E, Wolfe MEL, Hodak E, Kim YH
Br J Dermatol 2017 Apr;176(4):1010-1020. Epub 2017 Feb 20 doi: 10.1111/bjd.14932. PMID: 27501236
Recent advances in primary cutaneous B-cell lymphomas.
Vermeer MH, Willemze R
Curr Opin Oncol 2014 Mar;26(2):230-6. doi: 10.1097/CCO.0000000000000060. PMID: 24463347
Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review.
Fernández-Guarino M, Ortiz-Romero PL, Fernández-Misa R, Montalbán C
Actas Dermosifiliogr 2014 Jun;105(5):438-45. Epub 2013 Mar 26 doi: 10.1016/j.ad.2012.10.021. PMID: 23540593
Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients.
Servitje O, Muniesa C, Benavente Y, Monsálvez V, Garcia-Muret MP, Gallardo F, Domingo-Domenech E, Lucas A, Climent F, Rodriguez-Peralto JL, Ortiz-Romero PL, Sandoval J, Pujol RM, Estrach MT
J Am Acad Dermatol 2013 Sep;69(3):357-65. Epub 2013 Jun 21 doi: 10.1016/j.jaad.2013.04.047. PMID: 23796549

Prognosis

Follicular T-Helper Cells in Marginal Zone Lymphoma: Evidence of an Organoid Immune Response.
Robson A, Bakr F, Rashidghamat E, Willsmore ZN, Ally M, Greenblatt D, Barlow R, Wain EM, Child F, Esdaile B, Kempf W
Am J Dermatopathol 2021 Dec 1;43(12):e197-e203. doi: 10.1097/DAD.0000000000002017. PMID: 34231493
Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart?
Amitay-Laish I, Tavallaee M, Kim J, Hoppe RT, Million L, Feinmesser M, Fenig E, Wolfe MEL, Hodak E, Kim YH
Br J Dermatol 2017 Apr;176(4):1010-1020. Epub 2017 Feb 20 doi: 10.1111/bjd.14932. PMID: 27501236
Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients.
Servitje O, Muniesa C, Benavente Y, Monsálvez V, Garcia-Muret MP, Gallardo F, Domingo-Domenech E, Lucas A, Climent F, Rodriguez-Peralto JL, Ortiz-Romero PL, Sandoval J, Pujol RM, Estrach MT
J Am Acad Dermatol 2013 Sep;69(3):357-65. Epub 2013 Jun 21 doi: 10.1016/j.jaad.2013.04.047. PMID: 23796549
Primary cutaneous marginal zone lymphoma.
Dalle S, Thomas L, Balme B, Dumontet C, Thieblemont C
Crit Rev Oncol Hematol 2010 Jun;74(3):156-62. Epub 2009 Oct 12 doi: 10.1016/j.critrevonc.2009.09.003. PMID: 19819717
Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases.
Servitje O, Gallardo F, Estrach T, Pujol RM, Blanco A, Fernández-Sevilla A, Pétriz L, Peyrí J, Romagosa V
Br J Dermatol 2002 Dec;147(6):1147-58. doi: 10.1046/j.1365-2133.2002.04961.x. PMID: 12452864

Clinical prediction guides

Follicular T-Helper Cells in Marginal Zone Lymphoma: Evidence of an Organoid Immune Response.
Robson A, Bakr F, Rashidghamat E, Willsmore ZN, Ally M, Greenblatt D, Barlow R, Wain EM, Child F, Esdaile B, Kempf W
Am J Dermatopathol 2021 Dec 1;43(12):e197-e203. doi: 10.1097/DAD.0000000000002017. PMID: 34231493
Dermoscopic and trichoscopic features of primary cutaneous lymphomas - systematic review.
Sławińska M, Sokołowska-Wojdyło M, Olszewska B, Nowicki RJ, Sobjanek M, Zalaudek I
J Eur Acad Dermatol Venereol 2021 Jul;35(7):1470-1484. Epub 2021 Apr 27 doi: 10.1111/jdv.17219. PMID: 33710688
Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients.
Servitje O, Muniesa C, Benavente Y, Monsálvez V, Garcia-Muret MP, Gallardo F, Domingo-Domenech E, Lucas A, Climent F, Rodriguez-Peralto JL, Ortiz-Romero PL, Sandoval J, Pujol RM, Estrach MT
J Am Acad Dermatol 2013 Sep;69(3):357-65. Epub 2013 Jun 21 doi: 10.1016/j.jaad.2013.04.047. PMID: 23796549
Primary cutaneous marginal zone B-cell lymphoma: clinical and therapeutic features in 50 cases.
Hoefnagel JJ, Vermeer MH, Jansen PM, Heule F, van Voorst Vader PC, Sanders CJ, Gerritsen MJ, Geerts ML, Meijer CJ, Noordijk EM, Willemze R
Arch Dermatol 2005 Sep;141(9):1139-45. doi: 10.1001/archderm.141.9.1139. PMID: 16172311
Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases.
Servitje O, Gallardo F, Estrach T, Pujol RM, Blanco A, Fernández-Sevilla A, Pétriz L, Peyrí J, Romagosa V
Br J Dermatol 2002 Dec;147(6):1147-58. doi: 10.1046/j.1365-2133.2002.04961.x. PMID: 12452864

Recent systematic reviews

Dermoscopic and trichoscopic features of primary cutaneous lymphomas - systematic review.
Sławińska M, Sokołowska-Wojdyło M, Olszewska B, Nowicki RJ, Sobjanek M, Zalaudek I
J Eur Acad Dermatol Venereol 2021 Jul;35(7):1470-1484. Epub 2021 Apr 27 doi: 10.1111/jdv.17219. PMID: 33710688

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    Curated

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

    Reviews

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