Skip to main content

Primary Cutaneous B-cell Lymphomas: FL, MCL, Differential Diagnosis

  • Chapter
  • First Online:
Follicular Lymphoma and Mantle Cell Lymphoma

Part of the book series: Molecular and Translational Medicine ((MOLEMED))

  • 446 Accesses

Abstract

Primary cutaneous B-cell lymphomas comprise a small group of lymphoproliferative disorders that range in behavior from indolent, localized skin-limited tumors with survival approximating 100% to aggressive malignancies with a propensity for extracutaneous dissemination. The relatively indolent primary cutaneous marginal zone lymphoma and primary cutaneous follicle center lymphoma both show significant overlap with cutaneous lymphoid hyperplasias and often require strong correlations with clinical findings in order to establish a definitive diagnosis. These lymphomas can exhibit significant histologic and sometimes molecular overlap with their extracutaneous counterparts, MALT lymphoma and follicular lymphoma, respectively. A multidisciplinary evaluation may therefore be required in order to permit for proper categorization. Furthermore, these entities must be distinguished from other mature B-cell lymphomas that can involve the skin, including chronic lymphocytic leukemia/small cell lymphoma and mantle cell lymphoma. At the opposite end of the clinical spectrum is primary cutaneous diffuse large B-cell lymphoma, leg type, which is occasionally challenging to distinguish from follicle center lymphomas with a preponderance of large cells and may be phenotypically identical to some of the extracutaneous diffuse large B-cell lymphomas that secondarily involve the skin. The distinction of leg-type lymphoma from follicle center lymphoma is critical since leg-type lymphomas are clinically aggressive, and there is ongoing discussion regarding how to classify rare, morphologically or immunophenotypically ambiguous large cell lymphomas. Our present understanding of the histomorphologic, immunophenotypic, and molecular aberrances underpinning these unusual and diagnostically challenging lymphoproliferative entities is discussed herein with an emphasis on differential diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 119.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Willemze R, Kerl H, Sterry W, Berti E, Cerroni L, Chimenti S, et al. EORTC classification for primary cutaneous lymphomas: a proposal from the cutaneous lymphoma study Group of the European Organization for research and treatment of cancer. Blood. 1997;90(1):354–71.

    CAS  PubMed  Google Scholar 

  2. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Bradford PT, Devesa SS, Anderson WF, Toro JR. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood. 2009;113(21):5064–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Gru AA, Hurley MY, Salavaggione AL, Brodell L, Sheinbein D, Anadkat M, et al. Cutaneous mantle cell lymphoma: a clinicopathologic review of 10 cases. J Cutan Pathol. 2016;43(12):1112–20.

    Article  PubMed  Google Scholar 

  5. Takino H, Li C, Hu S, Kuo TT, Geissinger E, Muller-Hermelink HK, et al. Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathological study of cases from Asia, Germany, and the United States. Mod Pathol. 2008;21(12):1517–26.

    Article  CAS  PubMed  Google Scholar 

  6. Cerroni L, Zochling N, Putz B, Kerl H. Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol. 1997;24(8):457–61.

    Article  CAS  PubMed  Google Scholar 

  7. Guitart J, Deonizio J, Bloom T, Martinez-Escala ME, Kuzel TM, Gerami P, et al. High incidence of gastrointestinal tract disorders and autoimmunity in primary cutaneous marginal zone B-cell lymphomas. JAMA Dermatol. 2014;150(4):412–8.

    Article  PubMed  Google Scholar 

  8. Edinger JT, Kant JA, Swerdlow SH. Cutaneous marginal zone lymphomas have distinctive features and include 2 subsets. Am J Surg Pathol. 2010;34(12):1830–41.

    Article  PubMed  Google Scholar 

  9. Hoefnagel JJ, Vermeer MH, Jansen PM, Heule F, van Voorst Vader PC, Sanders CJ, et al. Primary cutaneous marginal zone B-cell lymphoma: clinical and therapeutic features in 50 cases. Arch Dermatol. 2005;141(9):1139–45.

    Article  CAS  PubMed  Google Scholar 

  10. Dalle S, Thomas L, Balme B, Dumontet C, Thieblemont C. Primary cutaneous marginal zone lymphoma. Crit Rev Oncol Hematol. 2010;74(3):156–62.

    Article  PubMed  Google Scholar 

  11. Cerroni L, Signoretti S, Hofler G, Annessi G, Putz B, Lackinger E, et al. Primary cutaneous marginal zone B-cell lymphoma: a recently described entity of low-grade malignant cutaneous B-cell lymphoma. Am J Surg Pathol. 1997;21(11):1307–15.

    Article  CAS  PubMed  Google Scholar 

  12. Amitay-Laish I, Tavallaee M, Kim J, Hoppe RT, Million L, Feinmesser M, et al. Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart? Br J Dermatol. 2017;176(4):1010–20.

    Article  CAS  PubMed  Google Scholar 

  13. Servitje O, Gallardo F, Estrach T, Pujol RM, Blanco A, Fernandez-Sevilla A, et al. Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases. Br J Dermatol. 2002;147(6):1147–58.

    Article  CAS  PubMed  Google Scholar 

  14. Baldassano MF, Bailey EM, Ferry JA, Harris NL, Duncan LM. Cutaneous lymphoid hyperplasia and cutaneous marginal zone lymphoma: comparison of morphologic and immunophenotypic features. Am J Surg Pathol. 1999;23(1):88–96.

    Article  CAS  PubMed  Google Scholar 

  15. Zinzani PL, Quaglino P, Pimpinelli N, Berti E, Baliva G, Rupoli S, et al. Prognostic factors in primary cutaneous B-cell lymphoma: the Italian study Group for Cutaneous Lymphomas. J Clin Oncol. 2006;24(9):1376–82.

    Article  PubMed  Google Scholar 

  16. Gerami P, Wickless SC, Rosen S, Kuzel TM, Ciurea A, Havey J, et al. Applying the new TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome in primary cutaneous marginal zone lymphoma. J Am Acad Dermatol. 2008;59(2):245–54.

    Article  PubMed  Google Scholar 

  17. Hatem J, Schrank-Hacker AM, Watt CD, Morrissette JJ, Rubin AI, Kim EJ, et al. Marginal zone lymphoma-derived interfollicular diffuse large B-cell lymphoma harboring 20q12 chromosomal deletion and missense mutation of BIRC3 gene: a case report. Diagn Pathol. 2016;11(1):137.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Goyal A, Carter JB, Pashtan I, Gallotto S, Wang I, Isom S, et al. Very low-dose versus standard dose radiation therapy for indolent primary cutaneous B-cell lymphomas: a retrospective study. J Am Acad Dermatol. 2018;78(2):408–10.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Oertel M, Elsayad K, Weishaupt C, Steinbrink K, Eich HT. De-escalated radiotherapy for indolent primary cutaneous B-cell lymphoma. Strahlenther Onkol. 2020;196:126.

    Article  PubMed  Google Scholar 

  20. Christensen L, Cooper K, Honda K, Mansur D. Relapse rates in patients with unilesional primary cutaneous B-cell lymphoma treated with radiation therapy: a single-institution experience. Br J Dermatol. 2018;179(5):1172–3.

    Article  CAS  PubMed  Google Scholar 

  21. NCCN. NCCN Guidelines for Primary Cutaneous B-cell Lymphomas. Available from: https://www.nccn.org/professionals/physician_gls/pdf/pcbcl.pdf.

  22. van Maldegem F, van Dijk R, Wormhoudt TA, Kluin PM, Willemze R, Cerroni L, et al. The majority of cutaneous marginal zone B-cell lymphomas expresses class-switched immunoglobulins and develops in a T-helper type 2 inflammatory environment. Blood. 2008;112(8):3355–61.

    Article  PubMed  CAS  Google Scholar 

  23. Guitart J. Rethinking primary cutaneous marginal zone lymphoma: shifting the focus to the cause of the infiltrate. J Cutan Pathol. 2015;42(9):600–3.

    Article  PubMed  Google Scholar 

  24. Duncan LM, LeBoit PE. Are primary cutaneous immunocytoma and marginal zone lymphoma the same disease? Am J Surg Pathol. 1997;21(11):1368–72.

    Article  CAS  PubMed  Google Scholar 

  25. Schafernak KT, Variakojis D, Goolsby CL, Tucker RM, Martinez-Escala ME, Smith FA, et al. Clonality assessment of cutaneous B-cell lymphoid proliferations: a comparison of flow cytometry immunophenotyping, molecular studies, and immunohistochemistry/in situ hybridization and review of the literature. Am J Dermatopathol. 2014;36(10):781–95.

    Article  PubMed  Google Scholar 

  26. Tsukamoto N, Kojima M, Uchiyama T, Takeuchi T, Karasawa M, Murakami H, et al. Primary cutaneous CD5+ marginal zone B-cell lymphoma resembling the plasma cell variant of Castleman’s disease. Case Rep APMIS. 2007;115(12):1426–31.

    Google Scholar 

  27. Magro CM, Yang A, Fraga G. Blastic marginal zone lymphoma: a clinical and pathological study of 8 cases and review of the literature. Am J Dermatopathol. 2013;35(3):319–26.

    Article  PubMed  Google Scholar 

  28. Jaffe ES. Navigating the cutaneous B-cell lymphomas: avoiding the rocky shoals. Mod Pathol. 2019;33:96.

    Article  PubMed  Google Scholar 

  29. Swerdlow SH. Cutaneous marginal zone lymphomas. Semin Diagn Pathol. 2017;34(1):76–84.

    Article  PubMed  Google Scholar 

  30. Schmid U, Eckert F, Griesser H, Steinke C, Cogliatti SB, Kaudewitz P, et al. Cutaneous follicular lymphoid hyperplasia with monotypic plasma cells. A clinicopathologic study of 18 patients. Am J Surg Pathol. 1995;19(1):12–20.

    Article  CAS  PubMed  Google Scholar 

  31. Nihal M, Mikkola D, Horvath N, Gilliam AC, Stevens SR, Spiro TP, et al. Cutaneous lymphoid hyperplasia: a lymphoproliferative continuum with lymphomatous potential. Hum Pathol. 2003;34(6):617–22.

    Article  PubMed  Google Scholar 

  32. Streubel B, Vinatzer U, Lamprecht A, Raderer M, Chott A. T(3;14)(p14.1;q32) involving IGH and FOXP1 is a novel recurrent chromosomal aberration in MALT lymphoma. Leukemia. 2005;19(4):652–8.

    Article  CAS  PubMed  Google Scholar 

  33. Streubel B, Lamprecht A, Dierlamm J, Cerroni L, Stolte M, Ott G, et al. T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. Blood. 2003;101(6):2335–9.

    Article  CAS  PubMed  Google Scholar 

  34. Maurus K, Appenzeller S, Roth S, Kuper J, Rost S, Meierjohann S, et al. Panel sequencing reveals recurrent genetic FAS alterations in primary cutaneous marginal zone lymphoma. J Invest Dermatol. 2018;138:1573.

    Article  CAS  PubMed  Google Scholar 

  35. Levin C, Mirzamani N, Zwerner J, Kim Y, Schwartz EJ, Sundram U. A comparative analysis of cutaneous marginal zone lymphoma and cutaneous chronic lymphocytic leukemia. Am J Dermatopathol. 2012;34(1):18–23.

    Article  PubMed  Google Scholar 

  36. Geyer JT, Ferry JA, Longtine JA, Flotte TJ, Harris NL, Zukerberg LR. Characteristics of cutaneous marginal zone lymphomas with marked plasmacytic differentiation and a T cell-rich background. Am J Clin Pathol. 2010;133(1):59–69.

    Article  PubMed  Google Scholar 

  37. Carlsen ED, Swerdlow SH, Cook JR, Gibson SE. Class-switched primary cutaneous marginal zone lymphomas are frequently IgG4-positive and have features distinct from IgM-positive cases. Am J Surg Pathol. 2019;43(10):1403–12.

    Article  PubMed  Google Scholar 

  38. Brenner I, Roth S, Puppe B, Wobser M, Rosenwald A, Geissinger E. Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression. Mod Pathol. 2013;26(12):1568–76.

    Article  CAS  PubMed  Google Scholar 

  39. Nakashima MO, Durkin L, Bodo J, Lin J, Quintanilla-Martinez L, Fu K, et al. Utility and diagnostic pitfalls of SOX11 monoclonal antibodies in mantle cell lymphoma and other lymphoproliferative disorders. Appl Immunohistochem Mol Morphol. 2014;22(10):720–7.

    Article  CAS  PubMed  Google Scholar 

  40. Streubel B, Simonitsch-Klupp I, Mullauer L, Lamprecht A, Huber D, Siebert R, et al. Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. Leukemia. 2004;18(10):1722–6.

    Article  CAS  PubMed  Google Scholar 

  41. Hedayat AA, Carter JB, Lansigan F, LeBlanc RE. Epidermotropic presentation by splenic B-cell lymphoma: the importance of clinical-pathologic correlation. J Cutan Pathol. 2018;45(4):299–304.

    Article  PubMed  Google Scholar 

  42. Mollejo M, Algara P, Mateo MS, Sanchez-Beato M, Lloret E, Medina MT, et al. Splenic small B-cell lymphoma with predominant red pulp involvement: a diffuse variant of splenic marginal zone lymphoma? Histopathology. 2002;40(1):22–30.

    Article  CAS  PubMed  Google Scholar 

  43. Magro CM, Momtahen S, Coleman M, Grossman ME. Epidermotropic CXCR3 positive marginal zone lymphoma: a distinctive clinical histopathological entity potentially originating in the skin; it does not always indicate splenic marginal zone lymphoma. Dermatol Online J. 2019;25(7):13030/qt4207n83g.

    Article  PubMed  Google Scholar 

  44. Jullie ML, Carlotti M, Vivot A Jr, Beylot-Barry M, Ortonne N, Frouin E, et al. CD20 antigen may be expressed by reactive or lymphomatous cells of transformed mycosis fungoides: diagnostic and prognostic impact. Am J Surg Pathol. 2013;37(12):1845–54.

    Article  PubMed  Google Scholar 

  45. Gibson SE, Swerdlow SH, Craig FE, Surti U, Cook JR, Nalesnik MA, et al. EBV-positive extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in the posttransplant setting: a distinct type of posttransplant lymphoproliferative disorder? Am J Surg Pathol. 2011;35(6):807–15.

    Article  PubMed  Google Scholar 

  46. Gong S, Crane GM, McCall CM, Xiao W, Ganapathi KA, Cuka N, et al. Expanding the spectrum of EBV-positive marginal zone lymphomas: a lesion associated with diverse immunodeficiency settings. Am J Surg Pathol. 2018;42(10):1306–16.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, van Baarlen J, Blokx WA, et al. Reclassification of 300 primary cutaneous B-cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol. 2007;25(12):1581–7.

    Article  PubMed  Google Scholar 

  48. Hamilton SN, Wai ES, Tan K, Alexander C, Gascoyne RD, Connors JM. Treatment and outcomes in patients with primary cutaneous B-cell lymphoma: the BC Cancer Agency experience. Int J Radiat Oncol Biol Phys. 2013;87(4):719–25.

    Article  PubMed  Google Scholar 

  49. Swerdlow SH, Quintanilla-Martinez L, Willemze R, Kinney MC. Cutaneous B-cell lymphoproliferative disorders: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol. 2013;139(4):515–35.

    Article  CAS  PubMed  Google Scholar 

  50. Gulia A, Saggini A, Wiesner T, Fink-Puches R, Argenyi Z, Ferrara G, et al. Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: implications for early diagnosis and treatment. J Am Acad Dermatol. 2011;65(5):991–1000.

    Article  PubMed  Google Scholar 

  51. Aldarweesh FA, Treaba DO. Primary cutaneous follicle centre lymphoma with Hodgkin and reed-Sternberg like cells: a case report and review of the literature. Case Rep Hematol. 2017;2017:9549428.

    PubMed  PubMed Central  Google Scholar 

  52. Dilly M, Ben-Rejeb H, Vergier B, Feldis M, Toty L, Nohra O, et al. Primary cutaneous follicle center lymphoma with Hodgkin and reed-Sternberg-like cells: a new histopathologic variant. J Cutan Pathol. 2014;41(10):797–801.

    Article  PubMed  Google Scholar 

  53. Li L, Majerowski J, Sokumbi O. Cutaneous spindled follicle center cell lymphoma with abundant mucin: a diagnostic pitfall. J Cutan Pathol. 2020;47:394.

    Article  PubMed  Google Scholar 

  54. Ries S, Barr R, LeBoit P, McCalmont T, Waldman J. Cutaneous sarcomatoid B-cell lymphoma. Am J Dermatopathol. 2007;29(1):96–8.

    Article  PubMed  Google Scholar 

  55. Cerroni L, El-Shabrawi-Caelen L, Fink-Puches R, LeBoit PE, Kerl H. Cutaneous spindle-cell B-cell lymphoma: a morphologic variant of cutaneous large B-cell lymphoma. Am J Dermatopathol. 2000;22(4):299–304.

    Article  CAS  PubMed  Google Scholar 

  56. Charli-Joseph Y, Cerroni L, LeBoit PE. Cutaneous spindle-cell B-cell lymphomas: most are neoplasms of follicular center cell origin. Am J Surg Pathol. 2015;39(6):737–43.

    Article  PubMed  Google Scholar 

  57. Verdanet E, Dereure O, Rene C, Tempier A, Benammar-Hafidi A, Gallo M, et al. Diagnostic value of STMN1, LMO2, HGAL, AID expression and 1p36 chromosomal abnormalities in primary cutaneous B cell lymphomas. Histopathology. 2017;71(4):648–60.

    Article  PubMed  Google Scholar 

  58. Szablewski V, Ingen-Housz-Oro S, Baia M, Delfau-Larue MH, Copie-Bergman C, Ortonne N. Primary cutaneous follicle center lymphomas expressing BCL2 protein frequently harbor BCL2 gene break and may present 1p36 deletion: a study of 20 cases. Am J Surg Pathol. 2016;40(1):127–36.

    Article  PubMed  Google Scholar 

  59. Gango A, Batai B, Varga M, Kapczar D, Papp G, Marschalko M, et al. Concomitant 1p36 deletion and TNFRSF14 mutations in primary cutaneous follicle center lymphoma frequently expressing high levels of EZH2 protein. Virchows Arch. 2018;473(4):453–62.

    Article  CAS  PubMed  Google Scholar 

  60. Vergier B, Belaud-Rotureau MA, Benassy MN, Beylot-Barry M, Dubus P, Delaunay M, et al. Neoplastic cells do not carry bcl2-JH rearrangements detected in a subset of primary cutaneous follicle center B-cell lymphomas. Am J Surg Pathol. 2004;28(6):748–55.

    Article  PubMed  Google Scholar 

  61. Kim BK, Surti U, Pandya A, Cohen J, Rabkin MS, Swerdlow SH. Clinicopathologic, immunophenotypic, and molecular cytogenetic fluorescence in situ hybridization analysis of primary and secondary cutaneous follicular lymphomas. Am J Surg Pathol. 2005;29(1):69–82.

    Article  PubMed  Google Scholar 

  62. Streubel B, Scheucher B, Valencak J, Huber D, Petzelbauer P, Trautinger F, et al. Molecular cytogenetic evidence of t(14;18)(IGH;BCL2) in a substantial proportion of primary cutaneous follicle center lymphomas. Am J Surg Pathol. 2006;30(4):529–36.

    Article  PubMed  Google Scholar 

  63. Servitje O, Climent F, Colomo L, Ruiz N, Garcia-Herrera A, Gallardo F, et al. Primary cutaneous vs secondary cutaneous follicular lymphomas: a comparative study focused on BCL2, CD10, and t(14;18) expression. J Cutan Pathol. 2019;46(3):182–9.

    Article  PubMed  Google Scholar 

  64. Katzenberger T, Kalla J, Leich E, Stocklein H, Hartmann E, Barnickel S, et al. A distinctive subtype of t(14;18)-negative nodal follicular non-Hodgkin lymphoma characterized by a predominantly diffuse growth pattern and deletions in the chromosomal region 1p36. Blood. 2009;113(5):1053–61.

    Article  CAS  PubMed  Google Scholar 

  65. Schmidt J, Gong S, Marafioti T, Mankel B, Gonzalez-Farre B, Balague O, et al. Genome-wide analysis of pediatric-type follicular lymphoma reveals low genetic complexity and recurrent alterations of TNFRSF14 gene. Blood. 2016;128(8):1101–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  66. Vermeer MH, Geelen FA, van Haselen CW, van Voorst Vader PC, Geerts ML, van Vloten WA, et al. Primary cutaneous large B-cell lymphomas of the legs. A distinct type of cutaneous B-cell lymphoma with an intermediate prognosis. Dutch Cutaneous Lymphoma Working Group. Arch Dermatol. 1996;132(11):1304–8.

    Article  CAS  PubMed  Google Scholar 

  67. Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Vergier B, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol. 2007;143(9):1144–50.

    Article  PubMed  Google Scholar 

  68. Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood. 2006;107(1):265–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Koens L, Vermeer MH, Willemze R, Jansen PM. IgM expression on paraffin sections distinguishes primary cutaneous large B-cell lymphoma, leg type from primary cutaneous follicle center lymphoma. Am J Surg Pathol. 2010;34(7):1043–8.

    Article  PubMed  Google Scholar 

  70. Robson A, Shukur Z, Ally M, Kluk J, Liu K, Pincus L, et al. Immunocytochemical p63 expression discriminates between primary cutaneous follicle centre cell and diffuse large B cell lymphoma-leg type, and is of the TAp63 isoform. Histopathology. 2016;69(1):11–9.

    Article  PubMed  Google Scholar 

  71. Sterry W, Kruger GR, Steigleder GK. Skin involvement of malignant B-cell lymphomas. J Dermatol Surg Oncol. 1984;10(4):276–7.

    Article  CAS  PubMed  Google Scholar 

  72. Muniesa C, Pujol RM, Estrach MT, Gallardo F, Garcia-Muret MP, Climent J, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type and secondary cutaneous involvement by testicular B-cell lymphoma share identical clinicopathological and immunophenotypical features. J Am Acad Dermatol. 2012;66(4):650–4.

    Article  PubMed  Google Scholar 

  73. Zhou XA, Louissaint A Jr, Wenzel A, Yang J, Martinez-Escala ME, Moy AP, et al. Genomic analyses identify recurrent alterations in immune evasion genes in diffuse large B-cell lymphoma, leg type. J Invest Dermatol. 2018;138(11):2365–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Brogan BL, Zic JA, Kinney MC, Hu JY, Hamilton KS, Greer JP. Large B-cell lymphoma of the leg: clinical and pathologic characteristics in a north American series. J Am Acad Dermatol. 2003;49(2):223–8.

    Article  PubMed  Google Scholar 

  75. Grange F, Maubec E, Bagot M, Beylot-Barry M, Joly P, Dalle S, et al. Treatment of cutaneous B-cell lymphoma, leg type, with age-adapted combinations of chemotherapies and rituximab. Arch Dermatol. 2009;145(3):329–30.

    Article  CAS  PubMed  Google Scholar 

  76. Senff NJ, Noordijk EM, Kim YH, Bagot M, Berti E, Cerroni L, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112(5):1600–9.

    Article  CAS  PubMed  Google Scholar 

  77. Senff NJ, Hoefnagel JJ, Neelis KJ, Vermeer MH, Noordijk EM, Willemze R, et al. Results of radiotherapy in 153 primary cutaneous B-cell lymphomas classified according to the WHO-EORTC classification. Arch Dermatol. 2007;143(12):1520–6.

    Article  PubMed  Google Scholar 

  78. Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Blood. 2004;103(1):275–82.

    Article  CAS  PubMed  Google Scholar 

  79. Pham-Ledard A, Cappellen D, Martinez F, Vergier B, Beylot-Barry M, Merlio JP. MYD88 somatic mutation is a genetic feature of primary cutaneous diffuse large B-cell lymphoma, leg type. J Invest Dermatol. 2012;132(8):2118–20.

    Article  CAS  PubMed  Google Scholar 

  80. Pham-Ledard A, Beylot-Barry M, Barbe C, Leduc M, Petrella T, Vergier B, et al. High frequency and clinical prognostic value of MYD88 L265P mutation in primary cutaneous diffuse large B-cell lymphoma, leg-type. JAMA Dermatol. 2014;150(11):1173–9.

    Article  PubMed  Google Scholar 

  81. Koens L, Zoutman WH, Ngarmlertsirichai P, Przybylski GK, Grabarczyk P, Vermeer MH, et al. Nuclear factor-kappaB pathway-activating gene aberrancies in primary cutaneous large B-cell lymphoma, leg type. J Invest Dermatol. 2014;134(1):290–2.

    Article  CAS  PubMed  Google Scholar 

  82. Mareschal S, Pham-Ledard A, Viailly PJ, Dubois S, Bertrand P, Maingonnat C, et al. Identification of somatic mutations in primary cutaneous diffuse large B-cell lymphoma, leg type by massive parallel sequencing. J Invest Dermatol. 2017;137(9):1984–94.

    Article  CAS  PubMed  Google Scholar 

  83. Menguy S, Frison E, Prochazkova-Carlotti M, Dalle S, Dereure O, Boulinguez S, et al. Double-hit or dual expression of MYC and BCL2 in primary cutaneous large B-cell lymphomas. Mod Pathol. 2018;31(8):1332–42.

    Article  CAS  PubMed  Google Scholar 

  84. Menguy S, Laharanne E, Prochazkova-Carlotti M, Gros A, Vergier B, Parrens M, et al. Challenges in assessing MYC rearrangement in primary cutaneous diffuse large B-cell lymphoma, leg-type. Am J Surg Pathol. 2020;44:424.

    Article  PubMed  Google Scholar 

  85. Son SM, Ha SY, Yoo HY, Oh D, Kim SJ, Kim WS, et al. Prognostic impact of MYC protein expression in central nervous system diffuse large B-cell lymphoma: comparison with MYC rearrangement and MYC mRNA expression. Mod Pathol. 2017;30(1):4–14.

    Article  CAS  PubMed  Google Scholar 

  86. Nosrati A, Monabati A, Sadeghipour A, Radmanesh F, Safaei A, Movahedinia S. MYC, BCL2, and BCL6 rearrangements in primary central nervous system lymphoma of large B cell type. Ann Hematol. 2019;98(1):169–73.

    Article  CAS  PubMed  Google Scholar 

  87. Schrader AMR, Jansen PM, Vermeer MH, Kleiverda JK, Vermaat JSP, Willemze R. High incidence and clinical significance of MYC rearrangements in primary cutaneous diffuse large B-cell lymphoma, leg type. Am J Surg Pathol. 2018;42(11):1488–94.

    Article  PubMed  Google Scholar 

  88. Scott DW, King RL, Staiger AM, Ben-Neriah S, Jiang A, Horn H, et al. High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements with diffuse large B-cell lymphoma morphology. Blood. 2018;131(18):2060–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  89. Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019;133(16):1703–14.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  90. Wilcox RA. Cutaneous B-cell lymphomas: 2019 update on diagnosis, risk stratification, and management. Am J Hematol. 2018;93(11):1427–30.

    Article  PubMed  Google Scholar 

  91. Menguy S, Beylot-Barry M, Parrens M, Ledard AP, Frison E, Comoz F, et al. Primary cutaneous large B-cell lymphomas: relevance of the 2017 World Health Organization classification: clinicopathological and molecular analyses of 64 cases. Histopathology. 2019;74(7):1067–80.

    Article  PubMed  Google Scholar 

  92. Senff NJ, Zoutman WH, Vermeer MH, Assaf C, Berti E, Cerroni L, et al. Fine-mapping chromosomal loss at 9p21: correlation with prognosis in primary cutaneous diffuse large B-cell lymphoma, leg type. J Invest Dermatol. 2009;129(5):1149–55.

    Article  CAS  PubMed  Google Scholar 

  93. Belaud-Rotureau MA, Marietta V, Vergier B, Mainhaguiet G, Turmo M, Idrissi Y, et al. Inactivation of p16INK4a/CDKN2A gene may be a diagnostic feature of large B cell lymphoma leg type among cutaneous B cell lymphomas. Virchows Arch. 2008;452(6):607–20.

    Article  CAS  PubMed  Google Scholar 

  94. Dijkman R, Tensen CP, Jordanova ES, Knijnenburg J, Hoefnagel JJ, Mulder AA, et al. Array-based comparative genomic hybridization analysis reveals recurrent chromosomal alterations and prognostic parameters in primary cutaneous large B-cell lymphoma. J Clin Oncol. 2006;24(2):296–305.

    Article  CAS  PubMed  Google Scholar 

  95. Tsang HC, Mathew S, Magro CM. An aggressive primary cutaneous follicle center lymphoma with c-MYC translocation and CDKN2A (9p21) deletion: a case report and review of the literature. Am J Dermatopathol. 2017;39(3):e44–e9.

    Article  PubMed  Google Scholar 

  96. Sen F, Medeiros LJ, Lu D, Jones D, Lai R, Katz R, et al. Mantle cell lymphoma involving skin: cutaneous lesions may be the first manifestation of disease and tumors often have blastoid cytologic features. Am J Surg Pathol. 2002;26(10):1312–8.

    Article  PubMed  Google Scholar 

  97. Marti RM, Campo E, Bosch F, Palou J, Estrach T. Cutaneous lymphocyte-associated antigen (CLA) expression in a lymphoblastoid mantle cell lymphoma presenting with skin lesions. Comparison with other clinicopathologic presentations of mantle cell lymphoma. J Cutan Pathol. 2001;28(5):256–64.

    Article  CAS  PubMed  Google Scholar 

  98. Lynch DW, Verma R, Larson E, Geis MC, Jassim AD. Primary cutaneous mantle cell lymphoma with blastic features: report of a rare case with special reference to staging and effectiveness of chemotherapy. J Cutan Pathol. 2012;39(4):449–53.

    Article  PubMed  Google Scholar 

  99. Canpolat F, Tas E, Albayrak Sonmez A, Oktay M, Eskioglu F, Alper M. Cutaneous presentation of mantle cell lymphoma. Acta Derm Venereol. 2010;90(5):548–50.

    Article  PubMed  Google Scholar 

  100. Bertero M, Novelli M, Fierro MT, Bernengo MG. Mantle zone lymphoma: an immunohistologic study of skin lesions. J Am Acad Dermatol. 1994;30(1):23–30.

    Article  CAS  PubMed  Google Scholar 

  101. Hrgovic I, Hartmann S, Steffen B, Vogl T, Kaufmann R, Meissner M. Cutaneous involvement as a rare first sign of systemic mantle cell lymphoma: a case report and review of the literature. Mol Clin Oncol. 2016;4(5):728–32.

    Article  PubMed  PubMed Central  Google Scholar 

  102. Fulton E, Alapat D, Mabry A, Gao L, Shalin SC. CLL/SLL with initial presentation as a longstanding cutaneous plaque: a rare case. J Cutan Pathol. 2016;43(8):717–21.

    Article  PubMed  Google Scholar 

  103. Tapia G, Mate JL, Fuente MJ, Navarro JT, Fernandez-Figueras MT, Junca J, et al. Cutaneous presentation of chronic lymphocytic leukemia as unique extramedullar involvement in a patient with normal peripheral blood lymphocyte count (monoclonal B-cell lymphocytosis). J Cutan Pathol. 2013;40(8):740–4.

    Article  PubMed  Google Scholar 

  104. Lin P, Jones D, Dorfman DM, Medeiros LJ. Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement. Am J Surg Pathol. 2000;24(11):1480–90.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Robert E. LeBlanc .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

LeBlanc, R.E. (2020). Primary Cutaneous B-cell Lymphomas: FL, MCL, Differential Diagnosis. In: Kaur, P. (eds) Follicular Lymphoma and Mantle Cell Lymphoma. Molecular and Translational Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-49741-5_3

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-49741-5_3

  • Published:

  • Publisher Name: Humana, Cham

  • Print ISBN: 978-3-030-49740-8

  • Online ISBN: 978-3-030-49741-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics