Table of Contents Purpose of This PDQ Summary General Information Cellular Classification Stage Information
Treatment Option Overview Stage I Mycosis Fungoides/Sézary Syndrome Stage II Mycosis Fungoides/Sézary Syndrome Stage III Mycosis Fungoides/Sézary Syndrome Stage IV Mycosis Fungoides/Sézary Syndrome Recurrent Mycosis Fungoides/Sézary Syndrome Get More Information From NCI Changes to This Summary (05/22/2008) More Information
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of mycosis fungoides and the Sézary syndrome. This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board.
Information about the following is included in this summary:
- Prognostic factors.
- Cellular classification.
- Staging.
- Treatment options by cancer stage.
This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Some of the reference citations in the summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.
This summary is available in a patient version, written in less technical language, and in Spanish.
Back to Top General Information
Mycosis fungoides and the Sézary syndrome (MF/SS) are neoplasias of malignant
T lymphocytes that usually possess the helper/inducer cell surface phenotype.
These kinds of neoplasms initially present as skin involvement and as such have been
classified as cutaneous T-cell lymphomas.[1] These types of lymphomas are included in the
Revised European-American Lymphoma classification as low grade T-cell
lymphomas, which should be distinguished from other T-cell lymphomas that
involve the skin, such as anaplastic large cell lymphoma (CD30 positive),
peripheral T-cell lymphoma (CD30 negative, with no epidermal involvement),
adult T-cell leukemia/lymphoma (usually with systemic involvement), or
subcutaneous panniculitic T-cell lymphoma.[2,3] These histologic types
of T-cell lymphomas are discussed in another PDQ summary. (Refer to the PDQ
summary on Adult Non-Hodgkin Lymphoma Treatment for more information.) In
addition, a number of benign or very indolent conditions can be confused with
mycosis fungoides. Consultation with a pathologist who has
expertise in distinguishing these conditions is important.[4]
The prognosis of patients with MF/SS is based on the extent of disease at
presentation (stage).[5] The presence of lymphadenopathy and involvement of
peripheral blood and viscera increase in likelihood with worsening cutaneous
involvement and define poor prognostic groups.[6] The median survival
following diagnosis varies according to stage. Patients with stage IA disease
have a median survival of 20 or more years. The majority of deaths for this
group are not caused by, nor are they related to, MF.[7] In contrast, more
than 50% of patients with stage III through stage IV disease die of MF, with a
median survival of less than 5 years.[5-7]
A report on 1,798 patients from the SEER database found an increase in second malignancies (standardized incidence ratio of 1.32; 95% confidence interval, 1.15–1.52), especially for Hodgkin and non-Hodgkin lymphoma and for myeloma.[8]
Typically, the natural history of MF is indolent.[9] Symptoms of the disease
may present for long periods, an average of 2 to 10 years, as waxing and waning
cutaneous eruptions prior to biopsy confirmation. MF/SS is treatable with
available topical and/or systemic therapies. Curative modalities, however, have
thus far proven elusive, with the possible exception of patients with minimal
disease confined to the skin.
Cutaneous disease typically progresses from an eczematous patch/plaque stage
covering less than 10% of the body surface (T1) to plaque stage covering
10% or more of the body surface (T2), and finally to tumors
(T3) that frequently undergo necrotic ulceration.[4,10] SS is an advanced form
of MF with generalized erythroderma (T4) and peripheral blood involvement at
presentation. Cytologic transformation from a low-grade lymphoma to a high-grade
lymphoma sometimes occurs during the course of these diseases and is associated
with a poor prognosis.[11-13] A common cause of death during the tumor phase is
sepsis from Pseudomonas aeruginosa or Staphylococcus aureus caused by chronic skin
infection with staph species and subsequent systemic infections.[10]
References
-
Girardi M, Heald PW, Wilson LD: The pathogenesis of mycosis fungoides. N Engl J Med 350 (19): 1978-88, 2004.
[PUBMED Abstract]
-
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 90 (1): 354-71, 1997.
[PUBMED Abstract]
-
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 84 (5): 1361-92, 1994.
[PUBMED Abstract]
-
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]
-
Zackheim HS, Amin S, Kashani-Sabet M, et al.: Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients. J Am Acad Dermatol 40 (3): 418-25, 1999.
[PUBMED Abstract]
-
de Coninck EC, Kim YH, Varghese A, et al.: Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides. J Clin Oncol 19 (3): 779-84, 2001.
[PUBMED Abstract]
-
Kim YH, Jensen RA, Watanabe GL, et al.: Clinical stage IA (limited patch and plaque) mycosis fungoides. A long-term outcome analysis. Arch Dermatol 132 (11): 1309-13, 1996.
[PUBMED Abstract]
-
Huang KP, Weinstock MA, Clarke CA, et al.: Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: evidence from population-based and clinical cohorts. Arch Dermatol 143 (1): 45-50, 2007.
[PUBMED Abstract]
-
Diamandidou E, Cohen PR, Kurzrock R: Mycosis fungoides and Sezary syndrome. Blood 88 (7): 2385-409, 1996.
[PUBMED Abstract]
-
Lorincz AL: Cutaneous T-cell lymphoma (mycosis fungoides) Lancet 347 (9005): 871-6, 1996.
[PUBMED Abstract]
-
Schechter GP, Sausville EA, Fischmann AB, et al.: Evaluation of circulating malignant cells provides prognostic information in cutaneous T cell lymphoma. Blood 69 (3): 841-9, 1987.
[PUBMED Abstract]
-
Dmitrovsky E, Matthews MJ, Bunn PA, et al.: Cytologic transformation in cutaneous T cell lymphoma: a clinicopathologic entity associated with poor prognosis. J Clin Oncol 5 (2): 208-15, 1987.
[PUBMED Abstract]
-
Kim YH, Bishop K, Varghese A, et al.: Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome. Arch Dermatol 131 (9): 1003-8, 1995.
[PUBMED Abstract]
Back to Top Cellular Classification
The histologic diagnosis of mycosis fungoides and the Sézary syndrome (MF/SS)
is usually difficult to determine in the initial stages of the disease and may require the
review of multiple biopsies by an experienced pathologist.
A definitive diagnosis from a skin biopsy requires the presence of MF/SS cells
(convoluted lymphocytes), a band-like upper dermal infiltrate, and epidermal
infiltrations with Pautrier abscesses (collections of neoplastic
lymphocytes). A definitive diagnosis of SS may be made from a peripheral blood
evaluation when skin biopsies are consistent with the diagnosis. Circulating Sézary cells can be confirmed by T-cell receptor gene analysis.[1]
References
-
Fraser-Andrews EA, Russell-Jones R, Woolford AJ, et al.: Diagnostic and prognostic importance of T-cell receptor gene analysis in patients with Sézary syndrome. Cancer 92 (7): 1745-52, 2001.
[PUBMED Abstract]
Back to Top Stage Information
The stages that follow are defined by TNM classification. Peripheral blood
involvement with mycosis fungoides or Sézary syndrome (MF/SS) cells is correlated with more
advanced skin stage, lymph node and visceral involvement, and shortened
survival but probably provides no independent prognostic information beyond
that associated with TNM staging. In a multivariate analysis, the two most
important prognostic factors are the presence of visceral disease and type of
skin involvement.
The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define mycosis fungoides.[1]
TNM(B) Definitions
Primary tumor (T)
- T1: Limited patch/plaque (<10% of skin surface involved)
- T2: Generalized patch/plaque (≥10% of skin surface involved)
- T3: Cutaneous tumors (one or more)
- T4: Generalized erythroderma
(with or without patches, plaques, or tumors)
[Note: Pathology of T1–T4 is diagnostic of cutaneous T-cell lymphoma (CTCL). When characteristics of more than one T-type tumor exist, both are recorded and the highest is used for staging, for example, T4(3).]
Regional lymph nodes (N)
- N0: Lymph nodes clinically uninvolved; the pathology is negative for
CTCL
- N1: Lymph nodes clinically enlarged, histologically uninvolved; the pathology is negative for CTCL
- N2: Lymph nodes clinically unenlarged, histologically involved; the pathology is positive for
CTCL
- N3: Lymph nodes enlarged and histologically involved; the pathology is positive for CTCL
Distant metastasis (M)
- M0: No visceral disease
- M1: Visceral disease present
The TNM classification includes a subcategory for patients with CTCL:
Blood involvement (B)
- B0: No circulating atypical cells (<1000 Sézary cells [CD4 + CD7-]/mL)
- B1: Circulating atypical cells (≥1000 Sézary cells [CD4 + CD7-]/mL)
TNM Stage Groupings
Stage IA
Stage IB
Stage IIA
Stage IIB
Stage IIIA
Stage IIIB
Stage IVA
- T1–T4, N2, M0
- T1–T4, N3, M0
Stage IVB
- T1–T4, N0, M1
- T1–T4, N1, M1
- T1–T4, N2, M1
- T1–T4, N3, M1
References
-
Lymphoid neoplasms. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 393-406.
Back to Top Treatment Option Overview
Treatment options for patients with mycosis fungoides and the Sézary syndrome (MF/SS) include:[1,2]
- Topical corticosteroids.
- Topical
chemotherapy with mechlorethamine (nitrogen mustard) or carmustine (BCNU).
- Psoralen and ultraviolet A radiation (PUVA).
- Ultraviolet B radiation (UVB)
- Total-skin electron-beam
radiation (TSEB).
- Radiation of symptomatic skin lesions.
- Interferon-alpha
alone or in combination with topical therapy.
- Single-agent and multiagent
chemotherapy.
- Bexarotene (topical gel or oral); retinoids.
- Denileukin diftitox (recombinant fusion protein of diptheria toxin fragments and interleukin-2 sequences).
- Combined modality treatment.
These types of treatments produce
remissions, but long-term remissions are uncommon. Treatment, therefore, is considered palliative for most patients,
though major symptomatic improvement is regularly achieved. Survival in excess of 8 years, however, is common. All patients with MF/SS are
candidates for clinical trials evaluating new approaches to treatment.
Current areas of interest in clinical trials for MF confined to the skin
include combined modality therapies containing both topical and systemic agents
such as TSEB combined with chemotherapy, topical mechlorethamine or PUVA
combined with interferon, or wide-field radiation techniques with PUVA.
Reports are available of activity for extracorporeal photochemotherapy using
psoralen; interferon-gamma or interferon-alpha; pentostatin; retinoids; fludarabine;
acyclovir; 2-chlorodeoxyadenosine; serotherapy with unlabeled,
toxin-labeled, or radiolabeled monoclonal antibodies; cell surface receptor
ligand-toxin fusion protein; and, methotrexate.[3-12]
Antigen-specific vaccination using dendritic cells [13] and ultraviolet-B phototherapy [14] are also under clinical evaluation.
References
-
Bunn PA Jr, Hoffman SJ, Norris D, et al.: Systemic therapy of cutaneous T-cell lymphomas (mycosis fungoides and the Sézary syndrome). Ann Intern Med 121 (8): 592-602, 1994.
[PUBMED Abstract]
-
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]
-
Kaplan EH, Rosen ST, Norris DB, et al.: Phase II study of recombinant human interferon gamma for treatment of cutaneous T-cell lymphoma. J Natl Cancer Inst 82 (3): 208-12, 1990.
[PUBMED Abstract]
-
Heald P, Rook A, Perez M, et al.: Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy. J Am Acad Dermatol 27 (3): 427-33, 1992.
[PUBMED Abstract]
-
Rosen ST, Zimmer AM, Goldman-Leikin R, et al.: Radioimmunodetection and radioimmunotherapy of cutaneous T cell lymphomas using an 131I-labeled monoclonal antibody: an Illinois Cancer Council Study. J Clin Oncol 5 (4): 562-73, 1987.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
Maier T, Tun-Kyi A, Tassis A, et al.: Vaccination of patients with cutaneous T-cell lymphoma using intranodal injection of autologous tumor-lysate-pulsed dendritic cells. Blood 102 (7): 2338-44, 2003.
[PUBMED Abstract]
-
Abe M, Ohnishi K, Kan C, et al.: Ultraviolet-B phototherapy is successful in Japanese patients with early-stage mycosis fungoides. J Dermatol 30 (11): 789-96, 2003.
[PUBMED Abstract]
Back to Top Stage I Mycosis Fungoides/Sézary Syndrome
Since several forms of treatment can produce complete resolution of skin
lesions in this stage, the choice of therapy is dependent on local expertise
and the facilities available. With therapy, the survival of patients with
stage IA disease can be expected to be the same as age and gender-matched
controls.[1,2]
Standard treatment options:[3]
- 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. Continued maintenance therapy with PUVA at more
protracted intervals is generally required to prolong remission duration.[4-6]
PUVA combined with interferon-alpha-2a is associated with a high response
rate.[7]
- Total-skin electron-beam radiation (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 considerable
technical expertise to deliver, can result in short- and long-term cutaneous
toxic effects, and is not widely available. Based on the long-term survival of
these early stage patients, electron-beam radiation therapy is sometimes used with
curative intent.[8-10] Long-term disease-free survival can be achieved in
patients with unilesional mycosis fungoides treated with local radiation
therapy.[11]
- Topical mechlorethamine (nitrogen mustard). Topical application of
mechlorethamine has produced regression of cutaneous lesions, with particular
efficacy in early stages of disease. The overall complete remission rate is
related to skin stage; 50% to 80% of TNM classification T1, and 25% to 75% of
T2 patients have complete responses. Treatments are usually continued for 2 to
3 years. Continuous 5-year disease-free survival may be possible in as many as 33% of T1 patients.[8,12-14]
- Local electron-beam radiation or orthovoltage radiation therapy may be
used to palliate areas of bulky or symptomatic skin disease.
- Interferon-alpha alone or in combination with topical therapy, as evidenced in the ECOG-1495 trial.
- Bexarotene, an oral or topical retinoid.[15,16]
- Oral methotrexate.[17]
- Pegylated liposomal doxorubicin.[18]
- Vorinostat, an oral histone deacetylase inhibitor.[19]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I 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
-
Kim YH, Jensen RA, Watanabe GL, et al.: Clinical stage IA (limited patch and plaque) mycosis fungoides. A long-term outcome analysis. Arch Dermatol 132 (11): 1309-13, 1996.
[PUBMED Abstract]
-
Zackheim HS, Amin S, Kashani-Sabet M, et al.: Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients. J Am Acad Dermatol 40 (3): 418-25, 1999.
[PUBMED Abstract]
-
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]
-
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]
-
Ramsay DL, Lish KM, Yalowitz CB, et al.: Ultraviolet-B phototherapy for early-stage cutaneous T-cell lymphoma. Arch Dermatol 128 (7): 931-3, 1992.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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]
-
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]
-
Micaily B, Miyamoto C, Kantor G, et al.: Radiotherapy for unilesional mycosis fungoides. Int J Radiat Oncol Biol Phys 42 (2): 361-4, 1998.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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.
Back to Top Stage II Mycosis Fungoides/Sézary Syndrome
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
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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.
Back to Top Stage III Mycosis Fungoides/Sézary Syndrome
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
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
Rosen ST, Foss FM: Chemotherapy for mycosis fungoides and the Sézary syndrome. Hematol Oncol Clin North Am 9 (5): 1109-16, 1995.
[PUBMED Abstract]
-
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]
-
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]
-
Heald PW, Perez MI, McKiernan G, et al.: Extracorporeal photochemotherapy for CTCL. Prog Clin Biol Res 337: 443-7, 1990.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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.
Back to Top Stage IV Mycosis Fungoides/Sézary Syndrome
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.)
The use of single alkylating agents has produced objective responses in 60% of
patients with a duration of less than 6 months. One of the alkylating agents
(e.g., mechlorethamine, cyclophosphamide, or chlorambucil), or the antimetabolite
methotrexate is the most frequently used. Single agents have not been shown to
cure any patients, and insufficient data exist to determine if these
agents prolong survival. Combination chemotherapy is not definitely superior
to single agents. Even in stage IV disease, treatments directed at the skin
may provide significant palliation.
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. 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 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. This therapy can produce excellent
palliation and may be combined with systemic treatment.[5]
- 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 Sézary syndrome.[6-8]
- Interferon-alpha alone or in combination with topical therapy, as evidenced in the ECOG-1495 trial.[7,9]
- Denileukin diftitox (interleukin-2 fusion toxin) for CD25 and MF.[10,11]
- Systemic chemotherapy: chlorambucil plus prednisone, mechlorethamine,
cyclophosphamide, methotrexate, and combination chemotherapy. [1,12,13]
- 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 in 243 patients
was 64% and was related to stage; as many as 35% of stage IV patients had complete
responses. Treatments are usually continued for 2 to 3 years.[14,15]
- Extracorporeal photochemotherapy alone [16-18] or in combination with
TSEB.[19]
- Serotherapy with monoclonal antibodies.[20,21]
- Bexarotene, an oral or topical retinoid.[22,23]
- Pegylated liposomal doxorubicin.[24]
- Vorinostat, an oral histone deacetylase inhibitor.[25]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV 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
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
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]
-
Rosen ST, Foss FM: Chemotherapy for mycosis fungoides and the Sézary syndrome. Hematol Oncol Clin North Am 9 (5): 1109-16, 1995.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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]
-
Heald PW, Perez MI, McKiernan G, et al.: Extracorporeal photochemotherapy for CTCL. Prog Clin Biol Res 337: 443-7, 1990.
[PUBMED Abstract]
-
Fraser-Andrews E, Seed P, Whittaker S, et al.: Extracorporeal photopheresis in Sézary syndrome. No significant effect in the survival of 44 patients with a peripheral blood T-cell clone. Arch Dermatol 134 (8): 1001-5, 1998.
[PUBMED Abstract]
-
Palareti G, Maccaferri M, Manotti C, et al.: Fibrinogen assays: a collaborative study of six different methods. C.I.S.M.E.L. Comitato Italiano per la Standardizzazione dei Metodi in Ematologia e Laboratorio. Clin Chem 37 (5): 714-9, 1991.
[PUBMED Abstract]
-
Knox SJ, Levy R, Hodgkinson S, et al.: Observations on the effect of chimeric anti-CD4 monoclonal antibody in patients with mycosis fungoides. Blood 77 (1): 20-30, 1991.
[PUBMED Abstract]
-
Rosen ST, Zimmer AM, Goldman-Leikin R, et al.: Radioimmunodetection and radioimmunotherapy of cutaneous T cell lymphomas using an 131I-labeled monoclonal antibody: an Illinois Cancer Council Study. J Clin Oncol 5 (4): 562-73, 1987.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
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.
Back to Top Recurrent Mycosis Fungoides/Sézary Syndrome
The treatment of relapsed patients with cutaneous T-cell lymphomas involves the joint decisions of the dermatologist, medical oncologist, and
radiation oncologist. It may be possible to re-treat localized areas of
relapse with additional electron-beam radiation or possibly to repeat total-skin electron-beam radiation therapy.[1] Photon radiation to bulky skin or
nodal masses may prove beneficial. If these options are not possible, then
continued topical treatment with other modalities such as mechlorethamine or
psoralen and ultraviolet A radiation (PUVA) may be warranted to relieve
cutaneous symptoms.
Clinical trials, if possible, should be considered as the next therapeutic
option. Options include:[2]
- PUVA combined with interferon-alpha-2a is associated with a high
response rate.[3]
- Extracorporeal photochemotherapy has produced tumor
regression in patients resistant to other therapies.[4,5]
- Radioimmunotherapy
using an I-131-labeled monoclonal antibody directed against a T-cell antigen has
produced brief responses in a clinical trial.[6]
- The interleukin-2 fusion toxin, denileukin diftitox,
is given monthly with response rates of about 30% to 40% for patients with CD25
and mycosis fungoides (MF).[7]
- Bexarotene is a retinoid available in an oral or topical form with
activity in patients with recurrent MF, as evidenced in the L-1069 trial.[8,9]
- New purine nucleoside phosphorylase inhibitors such as
peldesine are under clinical evaluation.[10]
- Allogeneic or autologous bone
marrow transplantation is also under clinical evaluation.[11]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent 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
-
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 32 (5): 1445-9, 1995.
[PUBMED Abstract]
-
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]
-
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]
-
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]
-
Heald PW, Perez MI, McKiernan G, et al.: Extracorporeal photochemotherapy for CTCL. Prog Clin Biol Res 337: 443-7, 1990.
[PUBMED Abstract]
-
Rosen ST, Zimmer AM, Goldman-Leikin R, et al.: Radioimmunodetection and radioimmunotherapy of cutaneous T cell lymphomas using an 131I-labeled monoclonal antibody: an Illinois Cancer Council Study. J Clin Oncol 5 (4): 562-73, 1987.
[PUBMED Abstract]
-
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]
-
Miller VA, Benedetti FM, Rigas JR, et al.: Initial clinical trial of a selective retinoid X receptor ligand, LGD1069. J Clin Oncol 15 (2): 790-5, 1997.
[PUBMED Abstract]
-
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]
-
Henderson B, Poole S, Wilson M: Microbial/host interactions in health and disease: who controls the cytokine network? Immunopharmacology 35 (1): 1-21, 1996.
[PUBMED Abstract]
-
Molina A, Zain J, Arber DA, et al.: Durable clinical, cytogenetic, and molecular remissions after allogeneic hematopoietic cell transplantation for refractory Sezary syndrome and mycosis fungoides. J Clin Oncol 23 (25): 6163-71, 2005.
[PUBMED Abstract]
Back to Top Get More Information From NCI
Call 1-800-4-CANCER
For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
Search the NCI Web site
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
Back to Top Changes to This Summary (05/22/2008)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
Back to Top More Information
About PDQ
Additional PDQ Summaries
Important:
This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
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