Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Primary cutaneous follicle center lymphoma

Eric Jacobsen, MD
Arnold S Freedman, MD
Rein Willemze, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD


Primary cutaneous B cell lymphoma (PCBCL) refers to those cases of B cell lymphoma that present in the skin when there is no evidence of extracutaneous disease at the time of diagnosis and after the completion of an initial staging evaluation. The revised 2016 World Health Organization (WHO) classification system defines three main subtypes of PCBCL [1]:

Primary cutaneous follicle center lymphoma (PCFCL)

Primary cutaneous large B cell lymphoma, leg type

Primary cutaneous marginal zone lymphoma (PCMZL), which is included in the broader category of extranodal marginal zone B cell lymphoma

These appear to be unique entities with differing clinical presentations, pathologic features, and prognosis. The approach to treatment also differs between subtypes. This topic review will discuss PCFCL. This entity was previously known as primary cutaneous follicle center cell lymphoma (PCFCCL) under the European Organization for Research and Treatment of Cancer (EORTC) classification and contains cases that may have previously be classified as cutaneous follicle center lymphoma or diffuse large B cell lymphoma under the 2001 WHO classification.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Sep 14, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375.
  2. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105:3768.
  3. Dores GM, Anderson WF, Devesa SS. Cutaneous lymphomas reported to the National Cancer Institute's surveillance, epidemiology, and end results program: applying the new WHO-European Organisation for Research and Treatment of Cancer classification system. J Clin Oncol 2005; 23:7246.
  4. Park JH, Shin HT, Lee DY, et al. World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphoma in Korea: a retrospective study at a single tertiary institution. J Am Acad Dermatol 2012; 67:1200.
  5. Fujita A, Hamada T, Iwatsuki K. Retrospective analysis of 133 patients with cutaneous lymphomas from a single Japanese medical center between 1995 and 2008. J Dermatol 2011; 38:524.
  6. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC Press, Lyon 2008.
  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.
  8. Fink-Puches R, Zenahlik P, Bäck B, et al. Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients. Blood 2002; 99:800.
  9. Senff NJ, Hoefnagel JJ, Jansen PM, 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.
  10. Mirza I, Macpherson N, Paproski S, et al. Primary cutaneous follicular lymphoma: an assessment of clinical, histopathologic, immunophenotypic, and molecular features. J Clin Oncol 2002; 20:647.
  11. Zinzani PL, Quaglino P, Pimpinelli N, et al. Prognostic factors in primary cutaneous B-cell lymphoma: the Italian Study Group for Cutaneous Lymphomas. J Clin Oncol 2006; 24:1376.
  12. 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.
  13. Massone C, Fink-Puches R, Laimer M, et al. Miliary and agminated-type primary cutaneous follicle center lymphoma: report of 18 cases. J Am Acad Dermatol 2011; 65:749.
  14. Hamilton SN, Wai ES, Tan K, et al. 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.
  15. Cerroni L, Kerl H. Primary cutaneous follicle center cell lymphoma. Leuk Lymphoma 2001; 42:891.
  16. Pimpinelli N, Santucci M, Mori M, et al. Primary cutaneous B-cell lymphoma: a clinically homogeneous entity? J Am Acad Dermatol 1997; 37:1012.
  17. Fung MA, Murphy MJ, Hoss DM, Grant-Kels JM. Practical evaluation and management of cutaneous lymphoma. J Am Acad Dermatol 2002; 46:325.
  18. Watabe H, Kawakami T, Soma Y, et al. Primary cutaneous T-cell-rich B-cell lymphoma in a zosteriform distribution associated with Epstein-Barr virus infection. J Dermatol 2002; 29:748.
  19. Gulia A, Saggini A, Wiesner T, 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; 65:991.
  20. Bergman R, Kurtin PJ, Gibson LE, et al. Clinicopathologic, immunophenotypic, and molecular characterization of primary cutaneous follicular B-cell lymphoma. Arch Dermatol 2001; 137:432.
  21. Grange F, Hedelin G, Joly P, et al. Prognostic factors in primary cutaneous lymphomas other than mycosis fungoides and the Sézary syndrome. The French Study Group on Cutaneous Lymphomas. Blood 1999; 93:3637.
  22. 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.
  23. de Leval L, Harris NL, Longtine J, et al. Cutaneous b-cell lymphomas of follicular and marginal zone types: use of Bcl-6, CD10, Bcl-2, and CD21 in differential diagnosis and classification. Am J Surg Pathol 2001; 25:732.
  24. Hoefnagel JJ, Dijkman R, Basso K, et al. Distinct types of primary cutaneous large B-cell lymphoma identified by gene expression profiling. Blood 2005; 105:3671.
  25. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 1994; 84:1361.
  26. Goodlad JR, Krajewski AS, Batstone PJ, et al. Primary cutaneous follicular lymphoma: a clinicopathologic and molecular study of 16 cases in support of a distinct entity. Am J Surg Pathol 2002; 26:733.
  27. Child FJ, Russell-Jones R, Woolford AJ, et al. Absence of the t(14;18) chromosomal translocation in primary cutaneous B-cell lymphoma. Br J Dermatol 2001; 144:735.
  28. Grange F, Petrella T, Beylot-Barry 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.
  29. Dijkman R, Tensen CP, Jordanova ES, 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.
  30. Menguy S, Gros A, Pham-Ledard A, et al. MYD88 Somatic Mutation Is a Diagnostic Criterion in Primary Cutaneous Large B-Cell Lymphoma. J Invest Dermatol 2016; 136:1741.
  31. Magro C, Crowson AN, Porcu P, Nuovo GJ. Automated kappa and lambda light chain mRNA expression for the assessment of B-cell clonality in cutaneous B-cell infiltrates: its utility and diagnostic application. J Cutan Pathol 2003; 30:504.
  32. Sterry W, Krüger GR, Steigleder GK. Skin involvement of malignant B-cell lymphomas. J Dermatol Surg Oncol 1984; 10:276.
  33. 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.
  34. Senff NJ, Noordijk EM, Kim YH, 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.
  35. Neelis KJ, Schimmel EC, Vermeer MH, et al. Low-dose palliative radiotherapy for cutaneous B- and T-cell lymphomas. Int J Radiat Oncol Biol Phys 2009; 74:154.
  36. http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf (Accessed on September 24, 2013).
  37. Suárez AL, Querfeld C, Horwitz S, et al. Primary cutaneous B-cell lymphomas: part II. Therapy and future directions. J Am Acad Dermatol 2013; 69:343.e1.
  38. Specht L, Dabaja B, Illidge T, et al. 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.
  39. Grange F, Bekkenk MW, Wechsler J, et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study. J Clin Oncol 2001; 19:3602.
  40. Fernández de Larrea C, Martínez-Pozo A, Mercadal S, et al. Initial features and outcome of cutaneous and non-cutaneous primary extranodal follicular lymphoma. Br J Haematol 2011; 153:334.
  41. Morales AV, Advani R, Horwitz SM, et al. Indolent primary cutaneous B-cell lymphoma: experience using systemic rituximab. J Am Acad Dermatol 2008; 59:953.
  42. Valencak J, Weihsengruber F, Rappersberger K, et al. Rituximab monotherapy for primary cutaneous B-cell lymphoma: response and follow-up in 16 patients. Ann Oncol 2009; 20:326.
  43. Brandenburg A, Humme D, Terhorst D, et al. Long-term outcome of intravenous therapy with rituximab in patients with primary cutaneous B-cell lymphomas. Br J Dermatol 2013; 169:1126.
  44. Peñate Y, Hernández-Machín B, Pérez-Méndez LI, et al. Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicentre study. The Spanish Working Group on Cutaneous Lymphoma. Br J Dermatol 2012; 167:174.
  45. Terhorst D, Mestel DS, Humme D, et al. Evaluation of different methods in the follow-up of patients with indolent types of primary cutaneous lymphomas. Br J Dermatol 2012; 166:1295.
  46. Abdul-Wahab A, Tang SY, Robson A, et al. Chromosomal anomalies in primary cutaneous follicle center cell lymphoma do not portend a poor prognosis. J Am Acad Dermatol 2014; 70:1010.
  47. Kodama K, Massone C, Chott A, et al. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 2005; 106:2491.
  48. Suárez AL, Pulitzer M, Horwitz S, et al. Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification. J Am Acad Dermatol 2013; 69:329.e1.