Skip to main content
SpringerLink
Log in

Cutaneous Lymphomas

  • Chapter
  • First Online:
Radiation Therapy for Extranodal Lymphomas

  • 607 Accesses

Abstract

Primary cutaneous lymphoma (PCL) is the second most common type of extranodal lymphoma. PCL has different clinical behavior and prognosis than those of extranodal lymphomas arising from other sites, and requires a different pretreatment evaluation system and treatment strategy. All patients with PCL should be reviewed by a multidisciplinary team to decide an appropriate treatment strategy. PCL has three major disease entities including T-cell and natural-killer (NK)-cell lymphoma, B-cell lymphoma, and precursor hematological neoplasm. Mycosis fungoides is the most common type of cutaneous T-cell lymphoma, and its clinical behavior is indolent with slow progression. The treatment strategies of mycosis fungoides are based on a “Stage-based” approach, and “Expectant policy” is a reasonable treatment option for early stage disease. Total skin electron beam therapy (TSEBT) and adjuvant therapy may be applied for the patients with stage IB-IIB. The European Organization for Research and Treatment of Cancer proposed the technical guidelines of TSEBT. The standards of care for PCLs, other than mycosis fungoides and Sézary syndrome, have not been established because of their infrequency. The treatment strategies for each PCL subtype should be decided according to disease subtype, stage, clinical features, and patient’s condition.

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 139.99
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. Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sezary syndrome. Blood. 2009;114:4337–53.

    Article  CAS  PubMed  Google Scholar 

  2. Smith BD, Wilson LD. Cutaneous lymphoma. Curr Probl Cancer. 2008;32:43–87.

    Article  PubMed  Google Scholar 

  3. Mazzeo E, Rubino L, Buglione M, et al. The current management of mycosis fungoides and Sezary syndrome and the role of radiotherapy: principles and indications. Rep Pract Oncol Radiother. 2014;19:77–91.

    Article  PubMed  Google Scholar 

  4. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–85.

    Article  CAS  PubMed  Google Scholar 

  5. Groves FD, Linet MS, Travis LB, Devesa SS. Cancer surveillance series: non-Hodgkin's lymphoma incidence by histologic subtype in the United States from 1978 through 1995. J Natl Cancer Inst. 2000;92:1240–51.

    Article  CAS  PubMed  Google Scholar 

  6. Sander CA, Flaig MJ, Jaffe ES. Cutaneous manifestations of lymphoma: a clinical guide based on the WHO classification. World Health Organization. Clin Lymphoma. 2001;2:86–100. discussion 1-2

    Article  CAS  PubMed  Google Scholar 

  7. Willemze R, Kerl H, Sterry W, 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;90:354–71.

    CAS  PubMed  Google Scholar 

  8. Willemze R, Dreyling M, Group EGW. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21(Suppl 5):v177–80.

    Article  PubMed  Google Scholar 

  9. Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood. 2011;118:4024–35.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Olsen EA, Whittaker S, Kim YH, et al. Clinical end points and response criteria in mycosis fungoides and Sezary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol. 2011;29:2598–607.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Kim YH, Willemze R, Pimpinelli N, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:479–84.

    Article  CAS  PubMed  Google Scholar 

  12. Jones GW, Kacinski BM, Wilson LD, et al. Total skin electron radiation in the management of mycosis fungoides: Consensus of the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Project Group. J Am Acad Dermatol. 2002;47:364–70.

    Article  PubMed  Google Scholar 

  13. Scarisbrick JJ, Prince HM, Vermeer MH, et al. Cutaneous Lymphoma International Consortium Study of Outcome in advanced stages of mycosis fungoides and Sezary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol. 2015;33:3766–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Trautinger F, Knobler R, Willemze R, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome. Eur J Cancer. 2006;42:1014–30.

    Article  PubMed  Google Scholar 

  15. Whittaker SJ, Marsden JR, Spittle M, Russell Jones R, British Association of D, Group UKCL. Joint British Association of Dermatologists and U.K. Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol. 2003;149:1095–107.

    Article  CAS  PubMed  Google Scholar 

  16. Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:1713–22.

    Article  CAS  PubMed  Google Scholar 

  17. Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sezary syndrome. Lancet. 2008;371:945–57.

    Article  CAS  PubMed  Google Scholar 

  18. Olsen EA, Rook AH, Zic J, et al. Sezary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011;64:352–404.

    Article  PubMed  Google Scholar 

  19. Wilcox RA. Cutaneous T-cell lymphoma: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014;89:837–51.

    Article  PubMed  Google Scholar 

  20. Vonderheid EC, Bernengo MG, Burg G, et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol. 2002;46:95–106.

    Article  PubMed  Google Scholar 

  21. Bekkenk MW, Geelen FA, van Voorst Vader PC, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. 2000;95:3653–61.

    CAS  PubMed  Google Scholar 

  22. Scarisbrick JJ, Kim YH, Whittaker SJ, et al. Prognostic factors, prognostic indices and staging in mycosis fungoides and Sezary syndrome: where are we now? Br J Dermatol. 2014;170:1226–36.

    Article  CAS  PubMed  Google Scholar 

  23. Smith BD, Wilson LD. Cutaneous lymphomas. Semin Radiat Oncol. 2007;17:158–68.

    Article  PubMed  Google Scholar 

  24. Olsen E, Duvic M, Frankel A, et al. Pivotal phase III trial of two dose levels of denileukin diftitox for the treatment of cutaneous T-cell lymphoma. J Clin Oncol. 2001;19:376–88.

    Article  CAS  PubMed  Google Scholar 

  25. Kamstrup MR, Lindahl LM, Gniadecki R, et al. Low-dose total skin electron beam therapy as a debulking agent for cutaneous T-cell lymphoma: an open-label prospective phase II study. Br J Dermatol. 2012;166:399–404.

    Article  CAS  PubMed  Google Scholar 

  26. Smith BD, Smith GL, Cooper DL, Wilson LD. The cutaneous B-cell lymphoma prognostic index: a novel prognostic index derived from a population-based registry. J Clin Oncol. 2005;23:3390–5.

    Article  PubMed  Google Scholar 

  27. Eich HT, Eich D, Micke O, et al. Long-term efficacy, curative potential, and prognostic factors of radiotherapy in primary cutaneous B-cell lymphoma. Int J Radiat Oncol Biol Phys. 2003;55:899–906.

    Article  PubMed  Google Scholar 

  28. Mian M, Marcheselli L, Luminari S, et al. CLIPI: a new prognostic index for indolent cutaneous B cell lymphoma proposed by the International Extranodal Lymphoma Study Group (IELSG 11). Ann Hematol. 2011;90:401–8.

    Article  PubMed  Google Scholar 

  29. Kazmierska J. Clinical results of the total skin electron irradiation of the mycosis fungoides in adults. Conventional fractionation and low dose schemes. Rep Pract Oncol Radiother. 2014;19:99–103.

    Article  PubMed  Google Scholar 

  30. Hoppe RT, Harrison C, Tavallaee M, et al. Low-dose total skin electron beam therapy as an effective modality to reduce disease burden in patients with mycosis fungoides: results of a pooled analysis from 3 phase-II clinical trials. J Am Acad Dermatol. 2015;72:286–92.

    Article  PubMed  Google Scholar 

  31. Becker M, Hoppe RT, Knox SJ. Multiple courses of high-dose total skin electron beam therapy in the management of mycosis fungoides. Int J Radiat Oncol Biol Phys. 1995;32:1445–9.

    Article  CAS  PubMed  Google Scholar 

  32. Yamaguchi M, Tobinai K, Oguchi M, et al. Phase I/II study of concurrent chemoradiotherapy for localized nasal natural killer/T-cell lymphoma: Japan Clinical Oncology Group Study JCOG0211. J Clin Oncol. 2009;27:5594–600.

    Article  CAS  PubMed  Google Scholar 

  33. Teckie S, Qi S, Lovie S, et al. Long-term outcomes and patterns of relapse of early-stage extranodal marginal zone lymphoma treated with radiation therapy with curative intent. Int J Radiat Oncol Biol Phys. 2015;92:130–7.

    Article  PubMed  Google Scholar 

  34. Specht L, Dabaja B, Illidge T, Wilson LD, Hoppe RT, International Lymphoma Radiation Oncology G. Modern radiation therapy for primary cutaneous lymphomas: field and dose guidelines from the International Lymphoma Radiation Oncology Group. Int J Radiat Oncol Biol Phys. 2015;92:32–9.

    Article  PubMed  Google Scholar 

  35. Non-Hodgkin’s lymphoma. Version 2.2015 NCCN clinical practice guidelines in oncology; 2015.

    Google Scholar 

  36. Neelis KJ, Schimmel EC, Vermeer MH, Senff NJ, Willemze R, Noordijk EM. Low-dose palliative radiotherapy for cutaneous B- and T-cell lymphomas. Int J Radiat Oncol Biol Phys. 2009;74:154–8.

    Article  PubMed  Google Scholar 

  37. Cotter GW, Baglan RJ, Wasserman TH, Mill W. Palliative radiation treatment of cutaneous mycosis fungoides—a dose response. Int J Radiat Oncol Biol Phys. 1983;9:1477–80.

    Article  CAS  PubMed  Google Scholar 

  38. Elsayad K, Kriz J, Moustakis C, et al. Total skin electron beam for primary cutaneous T-cell lymphoma. Int J Radiat Oncol Biol Phys. 2015;93:1077–86.

    Article  PubMed  Google Scholar 

  39. Thomas TO, Agrawal P, Guitart J, et al. Outcome of patients treated with a single-fraction dose of palliative radiation for cutaneous T-cell lymphoma. Int J Radiat Oncol Biol Phys. 2013;85:747–53.

    Article  PubMed  Google Scholar 

  40. Piotrowski T, Milecki P, Skorska M, Fundowicz D. Total skin electron irradiation techniques: a review. Postepy Dermatol Alergol. 2013;30:50–5.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Heumann TR, Esiashvili N, Parker S, et al. Total skin electron therapy for cutaneous T-cell lymphoma using a modern dual-field rotational technique. Int J Radiat Oncol Biol Phys. 2015;92:183–91.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Chen Z, Agostinelli AG, Wilson LD, Nath R. Matching the dosimetry characteristics of a dual-field Stanford technique to a customized single-field Stanford technique for total skin electron therapy. Int J Radiat Oncol Biol Phys. 2004;59:872–85.

    Article  PubMed  Google Scholar 

  43. Morris SL, McGovern M, Bayne S, Wain M, Child F, Whittaker S. Results of a 5-week schedule of modern total skin electron beam radiation therapy. Int J Radiat Oncol Biol Phys. 2013;86:936–41.

    Article  PubMed  Google Scholar 

  44. Chan DV, Aneja S, Honda K, et al. Radiation therapy in the management of unilesional primary cutaneous T-cell lymphomas. Br J Dermatol. 2012;166:1134–7.

    Article  CAS  PubMed  Google Scholar 

  45. 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 

Download references

Author information

Authors and Affiliations

  1. Department of Radiation Oncology, Juntendo University, 2-1-1 Hongo, Bunkyo, Tokyo, 112-0012, Japan

    Naoto Shikama M.D., Ph.D. & Kazunari Miyazawa M.D.

Authors
  1. Naoto Shikama M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazunari Miyazawa M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Naoto Shikama M.D., Ph.D. .

Editor information

Editors and Affiliations

  1. Department of Radiation Oncology, Juntendo University Faculty of Medicine, Tokyo, Japan

    Keisuke Sasai

  2. Department of Radiation Oncology, The Cancer Institute Hospital, Tokyo, Japan

    Masahiko Oguchi

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer Japan

About this chapter

Cite this chapter

Shikama, N., Miyazawa, K. (2017). Cutaneous Lymphomas. In: Sasai, K., Oguchi, M. (eds) Radiation Therapy for Extranodal Lymphomas. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56435-5_9

Download citation

  • DOI: https://doi.org/10.1007/978-4-431-56435-5_9

  • Published:

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-56433-1

  • Online ISBN: 978-4-431-56435-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation