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Primary Cutaneous Small- to Medium-Sized CD4+ Pleomorphic T-Cell Lymphoma

A Retrospective Case Series and Review of the Provisional Cutaneous Lymphoma Category

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Abstract

Background and Objective: Primary cutaneous small- to medium-sized CD4+ pleomorphic T-cell lymphoma (PCSM-TCL) is a rare and provisional subcategory of cutaneous T-cell lymphoma, associated with a favorable prognosis. We present five cases of PCSM-TCL and review the literature to address questions surrounding its diagnosis and treatment.

Methods: A retrospective review was conducted of 353 patients with non-mycosis fungoides cutaneous lymphomas evaluated at the M.D. Anderson Cancer Center Cutaneous Lymphoma Clinic over 10 years. A retrospective search of the English literature using PubMed was conducted to identify additional cases.

Results: Only five patients had a diagnosis of PCSM-TCL. Three patients had unique multifocal presentations that responded well to topical therapy or excision. One patient with localized disease initially treated with radiation therapy had an aggressive relapse requiring systemic therapy. One patient had an isolated nodule that fully resolved with excision. The review of the English literature found 232 additional cases. The presentation of PCSM-TCL was heterogeneous and included solitary/localized papules, nodules, plaques, and tumors. Multiple differential diagnoses were considered and PCSM-TCL diagnosis was based on histopathologic features and clinical correlation. Treatment of solitary/localized lesions included excision or radiation therapy. Multifocal lesions were uncommon and associated with an aggressive course requiring systemic chemotherapy.

Conclusions: The heterogeneous presentation, variable histologic overlap with other lymphoproliferative disorders, and uncertain prognosis of PCSM-TCL creates diagnostic and management dilemmas for clinicians. For treatment, a distinction should be made between indolent and aggressive subtypes. Further investigation is needed to determine if PCSM-TCL is indeed a distinct lymphoma category and, additionally, if aggressive subtypes should remain part of the same diagnostic category.

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References

  1. Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: IARC Press, 2008

    Google Scholar 

  2. Kempf W, Sander CA. Classification of cutaneous lymphomas: an update. Histopathology 2010 Jan; 56 (1): 57–70

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

    Article  PubMed  CAS  Google Scholar 

  4. 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 Jul 1; 90 (1): 354–71

  5. von den Driesch P, Coors EA. Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a report of 3 cases stable for years. J Am Acad Dermatol 2002; 46(4): 531–5

    Article  PubMed  Google Scholar 

  6. Bekkenk MW, Vermeer MH, Jansen PM, et al. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood 2003 Sep 15; 102 (6): 2213–9

  7. Friedmann D, Wechsler J, Delfau MH, et al. Primary cutaneous pleomorphic small T-cell lymphoma: a review of 11 cases. The French Study Group on Cutaneous Lymphomas. Arch Dermatol 1995 Sep; 131 (9): 1009–15

    Google Scholar 

  8. Grogg KL, Jung S, Erickson LA, et al. Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma: a clonal T-cell lymphoproliferative disorder with indolent behavior. Mod Pathol 2008; 21: 708–15

    Article  PubMed  CAS  Google Scholar 

  9. Beltraminelli H, Leinweber B, Kerl H, et al. Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma: a cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance? A study of 136 cases. Am J Dermatopathol 2009; 31 (4): 317–22

    Article  PubMed  Google Scholar 

  10. Garcia-Herrera A, Colomo L, Camós M, et al. Primary cutaneous small/medium CD4+ T-cell lymphomas: a heterogeneous group of tumors with different clinicopathologic features and outcome. J Clin Oncol 2008; 26: 3364–71

    Article  PubMed  Google Scholar 

  11. Sterry W, Siebel A, Mielke V. HTLV-1-negative pleomorphic T-cell lymphoma of the skin: the clinicopathological correlations and natural history of 15 patients. Br J Dermatol 1992; 126: 456–62

    PubMed  CAS  Google Scholar 

  12. Rodriguez Pinilla SM, Roncador G, Rodriguez-Peralto JL, et al. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma expresses follicular T-cell markers. Am J Surg Pathol 2009 Jan; 33 (1): 81–

    Google Scholar 

  13. Kim YC, Vandersteen DP. Primary cutaneous pleomorphic small/medium-sized T-cell lymphoma in a young man. Br J Dermatol Apr 2001; 144 (4): 903–5

    Google Scholar 

  14. Boussault P, Tucker ML, Weschler J, et al. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma associated with an annular elastolytic giant cell granuloma. Br J Dermatol 2009; 160 (5): 1126–8

    Article  PubMed  CAS  Google Scholar 

  15. Khamaysi Z, Ben-Arieh Y, Epelbaum R, et al. Pleomorphic CD8+ small/medium size cutaneous T-cell lymphoma. Am J Dermatopathol 2006 Oct; 28 (5): 434–7

    Google Scholar 

  16. Petrella T, Maubec E, Cornillet-Lefebvre P, et al. Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma? Am J Surg Pathol 2007; 31 (12): 1887–92

    Article  PubMed  Google Scholar 

  17. Beljaards RC, Meijer CJ, Van der Putte SC, et al. Primary cutaneous T-cell lymphoma: clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and CD30-positive large cell lymphoma. J Pathol 1994; 172: 53–60

    Article  PubMed  CAS  Google Scholar 

  18. Suchak R, O’Connor S, McNamara C, et al. Indolent CD8-positive lymphoid proliferation on the face: part of the spectrum of primary cutaneous small-/ medium-sized pleomorphic T-cell lymphoma or a distinct entity? J Cutan Pathol 2010 Sep; 37 (9): 977–81

  19. Landa NG, Zelickson BD, Peters MS, et al. Lymphoma versus pseudolymphoma of the skin: gene rearrangement study of 21 cases with clinicopathologic correlation. J Am Acad Dermatol 1993 Dec; 29 (6): 945–53

    Google Scholar 

  20. Boer A, Tirumalae R, Bresch M, et al. Pseudoclonality in cutaneous pseudolymphomas: a pitfall in interpretation of rearrangement studies. Br J Dermatol 2008 Aug; 159 (2): 394–402

    Google Scholar 

  21. Magro CM, Crowson AN, Kovatich AJ, et al. Drug-induced reversible lymphoid dyscrasia: a clonal lymphomatoid dermatitis of memory and activated T cells. Hum Pathol 2003 Feb; 34 (2): 119–29

    Google Scholar 

  22. Bakels V, van Oostveen JW, van der Putte SC, et al. Immunophenotyping and gene rearrangement analysis provide additional criteria to differentiate between cutaneous T-cell lymphomas and pseudo-T-cell lymphomas. Am J Pathol 1997; 150 (6): 1941–9

    PubMed  CAS  Google Scholar 

  23. Setyadi HG, Nash JW, Duvic M. The solitary lymphomatous papule, nodule, or tumor. J Am Acad Dermatol 2007; 57 (6): 1072–83

    Article  PubMed  Google Scholar 

  24. Kerl H, Fink-Puches R, Cerroni L. Diagnostic criteria of primary cutaneous B-cell lymphomas and pseudolymphomas. Keio J Med 2001 Dec; 50 (4): 269–73

    Google Scholar 

  25. Kerl H, Kodama K, Cerroni L. Diagnostic principles and new developments in primary cutaneous B-cell lymphomas. J Dermatol Sci 2004; 34 (3): 167–75

    Article  PubMed  Google Scholar 

  26. Momtaz P, Zippin JH. Cutaneous T-cell lymphoma: a review of current therapies and the future therapeutic implications of chemokine biology. J Drugs Dermatol 2009; 8 (12): 1142–9

    PubMed  Google Scholar 

  27. Berthelot C, Rivera A, Duvic M. Skin directed therapy for mycosis fungoides: a review. J Drugs Dermatol 2008; 7 (7): 655–66

    PubMed  Google Scholar 

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Acknowledgments

No sources of funding were used to prepare this article. The authors have no conflicts of interest that are directly relevant to the content of this article.

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Authors and Affiliations

  1. Baylor College of Medicine, Houston, TX, USA

    Victoria L. Williams

  2. Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

    Carlos A. Torres-Cabala

  3. Department of Dermatology, The University of Texas M.D. Anderson Cancer Center, 1452, 77030, 1400 Pressler, Houston, TX, USA

    Madeleine Duvic

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  1. Victoria L. Williams
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  2. Carlos A. Torres-Cabala
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Williams, V.L., Torres-Cabala, C.A. & Duvic, M. Primary Cutaneous Small- to Medium-Sized CD4+ Pleomorphic T-Cell Lymphoma. Am J Clin Dermatol 12, 389–401 (2011). https://doi.org/10.2165/11590390-000000000-00000

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