Stage II Mycosis Fungoides/Sézary Syndrome
Current Clinical Trials
Note: Some citations in the text of this section are followed by a level of
evidence. The PDQ editorial boards use a formal ranking system to help the
reader judge the strength of evidence linked to the reported results of a
therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more
information.)
No curative therapy exists for patients with stage II disease. The
choice of initial palliative therapy is, therefore, dependent on the patient’s symptoms and
the local expertise with each modality.
A randomized study of 103 patients compared combined total-skin electron-beam radiation
(TSEB) plus combination chemotherapy with conservation therapy consisting of
sequential topical therapies.[1] In the latter group, combination
chemotherapy was reserved for symptomatic extracutaneous disease or for disease
refractory to topical therapies. Patients of any stage were eligible.
Although the complete response rate was higher with combined therapy, toxic
effects were considerably greater, and no difference was seen in disease-free
or overall survival between the two groups.[1][Level of evidence: 1iiA]
Standard treatment options:[2]
- Psoralen and ultraviolet A radiation (PUVA). Therapeutic trials with PUVA
have shown a 62% to 90% complete remission rate with early cutaneous stages
achieving the best responses. Maintenance therapy with PUVA is generally
required to prolong remission duration.[3,4] PUVA combined with interferon-alpha-2a is associated with a high response rate.[5]
- TSEB. Electron radiation of
appropriate energies will penetrate only to the dermis, and the skin
alone can be treated without systemic effects. This therapy requires a
radiation therapy facility with physics support, considerable technical
expertise, and precise dosimetry. The therapy can provide excellent palliation with
complete response rates of as much as 80%.[6-8]
- Topical mechlorethamine (nitrogen mustard). Topical application of
mechlorethamine has produced regression of cutaneous lesions with particular
efficacy in early stages of the disease. The overall complete remission rate is
related to skin stage; 25% to 75% of TNM classification T2, and as many as 50% of T3
patients have complete responses. Treatments are usually continued for 2 to 3
years.[6,9-11]
- Local electron-beam radiation or orthovoltage radiation therapy may also
be used to palliate areas of bulky or symptomatic disease.
- Interferon-alpha alone or in combination with topical therapy, as evidenced in the ECOG-1495 trial.[12]
- Bexarotene, an oral or topical retinoid.[13,14]
- Oral methotrexate.[15]
- Pegylated liposomal doxorubicin.[16]
- Vorinostat, an oral histone deacetylase inhibitor.[17]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II mycosis fungoides/Sezary syndrome. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References
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Kaye FJ, Bunn PA Jr, Steinberg SM, et al.: A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med 321 (26): 1784-90, 1989.
[PUBMED Abstract]
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Trautinger F, Knobler R, Willemze R, et al.: EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer 42 (8): 1014-30, 2006.
[PUBMED Abstract]
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Herrmann JJ, Roenigk HH Jr, Hurria A, et al.: Treatment of mycosis fungoides with photochemotherapy (PUVA): long-term follow-up. J Am Acad Dermatol 33 (2 Pt 1): 234-42, 1995.
[PUBMED Abstract]
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Querfeld C, Rosen ST, Kuzel TM, et al.: Long-term follow-up of patients with early-stage cutaneous T-cell lymphoma who achieved complete remission with psoralen plus UV-A monotherapy. Arch Dermatol 141 (3): 305-11, 2005.
[PUBMED Abstract]
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Kuzel TM, Roenigk HH Jr, Samuelson E, et al.: Effectiveness of interferon alfa-2a combined with phototherapy for mycosis fungoides and the Sézary syndrome. J Clin Oncol 13 (1): 257-63, 1995.
[PUBMED Abstract]
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Chinn DM, Chow S, Kim YH, et al.: Total skin electron beam therapy with or without adjuvant topical nitrogen mustard or nitrogen mustard alone as initial treatment of T2 and T3 mycosis fungoides. Int J Radiat Oncol Biol Phys 43 (5): 951-8, 1999.
[PUBMED Abstract]
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Quirós PA, Jones GW, Kacinski BM, et al.: Total skin electron beam therapy followed by adjuvant psoralen/ultraviolet-A light in the management of patients with T1 and T2 cutaneous T-cell lymphoma (mycosis fungoides). Int J Radiat Oncol Biol Phys 38 (5): 1027-35, 1997.
[PUBMED Abstract]
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Ysebaert L, Truc G, Dalac S, et al.: Ultimate results of radiation therapy for T1-T2 mycosis fungoides (including reirradiation). Int J Radiat Oncol Biol Phys 58 (4): 1128-34, 2004.
[PUBMED Abstract]
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Vonderheid EC, Tan ET, Kantor AF, et al.: Long-term efficacy, curative potential, and carcinogenicity of topical mechlorethamine chemotherapy in cutaneous T cell lymphoma. J Am Acad Dermatol 20 (3): 416-28, 1989.
[PUBMED Abstract]
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Hoppe RT, Abel EA, Deneau DG, et al.: Mycosis fungoides: management with topical nitrogen mustard. J Clin Oncol 5 (11): 1796-803, 1987.
[PUBMED Abstract]
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de Quatrebarbes J, Estève E, Bagot M, et al.: Treatment of early-stage mycosis fungoides with twice-weekly applications of mechlorethamine and topical corticosteroids: a prospective study. Arch Dermatol 141 (9): 1117-20, 2005.
[PUBMED Abstract]
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Foss FM, Ihde DC, Breneman DL, et al.: Phase II study of pentostatin and intermittent high-dose recombinant interferon alfa-2a in advanced mycosis fungoides/Sézary syndrome. J Clin Oncol 10 (12): 1907-13, 1992.
[PUBMED Abstract]
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Duvic M, Hymes K, Heald P, et al.: Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol 19 (9): 2456-71, 2001.
[PUBMED Abstract]
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Heald P, Mehlmauer M, Martin AG, et al.: Topical bexarotene therapy for patients with refractory or persistent early-stage cutaneous T-cell lymphoma: results of the phase III clinical trial. J Am Acad Dermatol 49 (5): 801-15, 2003.
[PUBMED Abstract]
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Zackheim HS, Kashani-Sabet M, McMillan A: Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol 49 (5): 873-8, 2003.
[PUBMED Abstract]
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Wollina U, Dummer R, Brockmeyer NH, et al.: Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer 98 (5): 993-1001, 2003.
[PUBMED Abstract]
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Olsen E, Kim YH, Kuzel T, et al.: Vorinostat (suberoylanilide hydroxamic acid, SAHA) is clinically active in advanced cutaneous T-cell lymphoma (CTLC): results of a phase IIb trial. [Abstract] J Clin Oncol 24 (Suppl 18): A-7500, 422s, 2006.
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