Skip to main content
SpringerLink
Log in

Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type

  • Chapter
Atlas of Cutaneous Lymphomas

  • 1960 Accesses

Abstract

Primary cutaneous diffuse large B-cell lymphoma (pcDLBCL), leg type, is a rare B-cell lymphoma of post-germinal B cells. Although this lymphoma classically presents on the leg with erythematous or violaceous tumors or nodules, it can also present elsewhere on the body. Patients with pcDLBCL have a moderately poor prognosis and experience frequent systemic dissemination. Though small case series have reported dramatic improvements in survival with combination chemotherapy and rituximab, treatment is largely limited by patient age and functional status. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of pcDLBCL. The chapter closes with two clinical cases: one case of pcDLBCL occurring on the leg and one occurring on the scalp.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
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

Similar content being viewed by others

References

  1. Meijer CJLM, Vergier B, Duncan LM, Willemze R. Primary cutaneous DLBCL, leg type. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 242.

    Google Scholar 

  2. 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;113:5064–73.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Paulli M, Viglio A, Vivenza D, Capello D, Rossi D, Riboni R, et al. Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity. Hum Pathol. 2002;33:937–43.

    Article  PubMed  Google Scholar 

  4. Bessell EM, Humber CE, O’Connor S, English JSE, Perkins W, Dickinson PD, et al. Primary cutaneous B-cell lymphoma in Nottinghamshire U.K.: prognosis of subtypes defined in the WHO-EORTC classification. Br J Dermatol. 2012;167:1118–23.

    Article  CAS  PubMed  Google Scholar 

  5. Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood. 2005;106:2491–7.

    Article  CAS  PubMed  Google Scholar 

  6. 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;25:1581–7.

    Article  PubMed  Google Scholar 

  7. Vermeer MH, Geelen F, van Haselen CW, van Voorst Vader PC, Geerts M-L, van Vloten WA, et al. Primary cutaneous large B-cell lymphoma of the leg. Arch Dermatol. 1996;132:1304–8.

    Article  CAS  PubMed  Google Scholar 

  8. 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;87:719–25.

    Article  PubMed  Google Scholar 

  9. Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Bergier B, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type. Arch Dermatol. 2007;143:1144–50.

    Article  PubMed  Google Scholar 

  10. Kramer MH, Hermans J, Wijburg E, Philippo K, Geelen E, van Krieken JH, et al. Clinical relevance of BCL2, BCL6, and MYC rearrangements in diffuse large B-cell lymphoma. Blood. 1998;92:3152–62.

    CAS  PubMed  Google Scholar 

  11. 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;49:223–8.

    Article  PubMed  Google Scholar 

  12. 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;145:329–30.

    CAS  PubMed  Google Scholar 

  13. 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;112:1600–9.

    Article  CAS  PubMed  Google Scholar 

  14. Persky DO, Unger JM, Spier CM, Stea B, LeBlanc M, McCarty MJ, et al. Phase II study of rituximab plus three cycles of CHOP and involved-field radiotherapy for patients with limited-stage aggressive B-cell lymphoma: Southwest Oncology Group study 0014. J Clin Oncol. 2008;26:2258–63.

    Article  CAS  PubMed  Google Scholar 

  15. Miller TP, Dahlberg S, Cassady JR, Adelstein DJ, Spier CM, Grogan TM, et al. Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin’s lymphoma. N Engl J Med. 1998;339:21–6.

    Article  CAS  PubMed  Google Scholar 

  16. Posada-Garcia C, Florez A, Pardavila R, Garcia-Cruz A, Amador L, Alvarez M, et al. Primary cutaneous large B-cell lymphoma, leg type, successfully treated with rituximab plus chemotherapy. Eur J Dermatol. 2009;19:393–4.

    Google Scholar 

  17. Hallermann C, Niermann C, Fischer R-J, Schulze H-J. New prognostic relevant factors in primary cutaneous diffuse large B-cell lymphomas. J Am Acad Dermatol. 2007;56:588–97.

    Article  PubMed  Google Scholar 

  18. 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;139:515–35.

    Article  CAS  PubMed  Google Scholar 

  19. Desouki MM, Post GR, Cherry D, Lazarchick J. PAX-5: a valuable immunohistochemical marker in the differential diagnosis of lymphoid neoplasms. Clin Med Res. 2010;8:84–8.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  20. Hallermann C, Kaune KM, Gesk S, Martin-Subero JI, Gunawan B, Griesinger F, et al. Molecular cytogenetic analysis of chromosomal breakpoints in the IGH, MYC, BCL6, and MALT1 gene loci in primary cutaneous B-cell lymphomas. J Invest Dermatol. 2004;123:213–9.

    Article  CAS  PubMed  Google Scholar 

  21. 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;24:296–305.

    Article  CAS  PubMed  Google Scholar 

  22. 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;129:1149–55.

    Article  CAS  PubMed  Google Scholar 

  23. Espinet B, García-Herrera A, Gallardo F, Baró C, Salgado R, Servitje O, et al. FOXP1 molecular cytogenetics and protein expression analyses in primary cutaneous large B cell lymphoma, leg-type. Histol Histopathol. 2011;26:213–21.

    CAS  PubMed  Google Scholar 

  24. Grange F, Petrella T, Beylot-Barry M, Joly P, D’Incan M, Delaunay M, et al. Bcl-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas. Blood. 2004;103:3662–8.

    Article  CAS  PubMed  Google Scholar 

  25. Felcht M, Heck M, Weiss C, Becker JC, Dippel E, Müller CSL, et al. Expression of the T-cell regulatory marker FOXP3 in primary cutaneous large B-cell lymphoma tumour cells. Br J Dermatol. 2012;167:348–58.

    Article  CAS  PubMed  Google Scholar 

  26. Hoefnagel JJ, Dijkman R, Basso K, Jansen PM, Hallermann C, Willemze R, et al. Distinct types of primary cutaneous large B-cell lymphoma identified by gene expression profiling. Blood. 2005;105:3671–8.

    Article  CAS  PubMed  Google Scholar 

  27. Sterry W, Kruger GR, Steigelder G-K. Skin involvement in malignant B-cell lymphomas. J Dermatol Surg Oncol. 1984;10:276–7.

    Article  CAS  PubMed  Google Scholar 

  28. 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;107:265–76.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. Muniesa C, Pujol RM, Estrach MT, Gallardo F, García-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;66:650–4.

    Article  PubMed  Google Scholar 

  30. Demirkesen C, Tüzüner N, Esen T, Lebe B, Ozkal S. The expression of IgM is helpful in the differentiation of primary cutaneous diffuse large B cell lymphoma and follicle center lymphoma. Leuk Res. 2011;35:1269–72.

    Article  CAS  PubMed  Google Scholar 

  31. 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;34:1043–8.

    Article  PubMed  Google Scholar 

  32. Shimada K, Kinoshita T, Naoe T, Nakamura S. Presentation and management of intravascular large B-cell lymphoma. Lancet Oncol. 2009;10:895–902.

    Article  PubMed  Google Scholar 

  33. Ferreri AJM, Campo E, Seymour JF, Willemze R, Ilariucci F, Ambrosetti A, et al. Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the “cutaneous variant”. Br J Haematol. 2004;127:173–83.

    Article  PubMed  Google Scholar 

  34. Orwat DE, Batalis NI. Intravascular large B-cell lymphoma. Arch Pathol Lab Med. 2012;136:333–8.

    Article  PubMed  Google Scholar 

  35. Jaffe ES, Harris NL, Stein H, Campo E, Pileri SA, Swerdlow SH. Introduction and overview of the classification of the lymphoid neoplasms. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 158–66.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, Boston, MA, USA

    Amrita Goyal M.D.

  2. Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joi B. Carter M.D.

  3. Comprehensive Cutaneous Lymphoma Program, Department of Medicine, Massachusetts General Hospital Cancer Center, Boston, MA, USA

    Jeffrey A. Barnes M.D., Ph.D.

  4. Dermatopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Lyn McDivitt Duncan M.D.

Authors
  1. Amrita Goyal M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Joi B. Carter M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jeffrey A. Barnes M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Lyn McDivitt Duncan M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lyn McDivitt Duncan M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA

    Joi B. Carter

  2. Division of Health Sciences and Technology, Harvard Medical School, Boston, Massachusetts, USA

    Amrita Goyal

  3. Dermatopathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA

    Lyn McDivitt Duncan

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Goyal, A., Carter, J.B., Barnes, J.A., Duncan, L.M. (2015). Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type. In: Carter, J., Goyal, A., McDivitt Duncan, L. (eds) Atlas of Cutaneous Lymphomas. Springer, Cham. https://doi.org/10.1007/978-3-319-17217-0_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-17217-0_18

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17216-3

  • Online ISBN: 978-3-319-17217-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation