Stage III 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 treatment exists for patients with stage III disease. The initial choice of palliative therapy is, therefore, dependent on the local expertise
with each modality. In patients with the Sézary syndrome (SS), a high
probability of extracutaneous involvement exists, and therefore systemic chemotherapy
is often given, though no proof is available that this affects survival.
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 (note that in this clinical setting, the skin is
easily injured; any of the topical therapies must be administered with extreme
caution):[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. PUVA may be used in conjunction with systemic
treatment. Maintenance therapy with PUVA is generally required to prolong
remission duration.[3] PUVA combined with interferon-alpha-2a is associated
with a high response rate.[4]
- TSEB. Electron radiation of
appropriate energies will penetrate only to the dermis, and thus the skin alone
can be treated without systemic effects. This therapy requires an excellent
radiation therapy facility with physics support, considerable technical
expertise, and precise dosimetry. The therapy can produce excellent
palliation with complete response rates of as much as 80%.[5,6]
- Local electron-beam radiation or orthovoltage radiation therapy may also
be used to palliate areas of bulky or symptomatic disease.
- Fludarabine, 2-chlorodeoxyadenosine, and pentostatin are active agents for
mycosis fungoides (MF) and SS.[7-10]
- Interferon-alpha alone or in combination with topical therapy, as evidenced in ECOG-1495.[8,11]
- Denileukin diftitox (interleukin-2 fusion toxin) for CD25 and MF.[12,13]
- Systemic chemotherapy (single agent or combination) often combined with
treatment directed at the skin.[1,14,15]
- Extracorporeal photochemotherapy.[16,17]
- Topical mechlorethamine (nitrogen mustard). This form of treatment may be
used palliatively or to supplement therapeutic approaches directed against
nodal or visceral disease. The overall complete remission rate of TNM
classification T4 patients is 20% to 40%. Treatments are usually continued for
2 to 3 years.[18,19]
- Bexarotene, an oral or topical retinoid.[20,21]
- Pegylated liposomal doxorubicin.[22]
- Vorinostat, an oral histone deacetylase inhibitor.[23]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III 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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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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Jones GW, Rosenthal D, Wilson LD: Total skin electron radiation for patients with erythrodermic cutaneous T-cell lymphoma (mycosis fungoides and the Sézary syndrome). Cancer 85 (9): 1985-95, 1999.
[PUBMED Abstract]
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Reddy S, Parker CM, Shidnia H, et al.: Total skin electron beam radiation therapy for mycosis fungoides. Am J Clin Oncol 15 (2): 119-24, 1992.
[PUBMED Abstract]
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Saven A, Carrera CJ, Carson DA, et al.: 2-Chlorodeoxyadenosine: an active agent in the treatment of cutaneous T-cell lymphoma. Blood 80 (3): 587-92, 1992.
[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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Foss FM, Ihde DC, Linnoila IR, et al.: Phase II trial of fludarabine phosphate and interferon alfa-2a in advanced mycosis fungoides/Sézary syndrome. J Clin Oncol 12 (10): 2051-9, 1994.
[PUBMED Abstract]
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Kurzrock R, Pilat S, Duvic M: Pentostatin therapy of T-cell lymphomas with cutaneous manifestations. J Clin Oncol 17 (10): 3117-21, 1999.
[PUBMED Abstract]
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Olsen EA, Bunn PA: Interferon in the treatment of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am 9 (5): 1089-107, 1995.
[PUBMED Abstract]
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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 19 (2): 376-88, 2001.
[PUBMED Abstract]
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Siegel RS, Pandolfino T, Guitart J, et al.: Primary cutaneous T-cell lymphoma: review and current concepts. J Clin Oncol 18 (15): 2908-25, 2000.
[PUBMED Abstract]
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Rosen ST, Foss FM: Chemotherapy for mycosis fungoides and the Sézary syndrome. Hematol Oncol Clin North Am 9 (5): 1109-16, 1995.
[PUBMED Abstract]
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Zackheim HS, Epstein EH Jr: Low-dose methotrexate for the Sézary syndrome. J Am Acad Dermatol 21 (4 Pt 1): 757-62, 1989.
[PUBMED Abstract]
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Edelson R, Berger C, Gasparro F, et al.: Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results. N Engl J Med 316 (6): 297-303, 1987.
[PUBMED Abstract]
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Heald PW, Perez MI, McKiernan G, et al.: Extracorporeal photochemotherapy for CTCL. Prog Clin Biol Res 337: 443-7, 1990.
[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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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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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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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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