NCCN Guidelines Version 3.2012 Mycosis Fungoides/Sezary ...

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NCCN Guidelines Version 3.2012 Mycosis Fungoides/Sezary Syndrome STAGE PRIMARY TREATMENTp ( MFSS-2) Stage IV Sezary syndrome Non Sezary or Visceral disease (solid organ) � See Suggested Treatment Regimens � Systemic Therapies (SYST-CAT A) (MFSS-A) � Combination Therapies See Suggested Treatment Regimens - Systemic Therapies (SYST-CAT B) or ( SYST-CAT C) ff or multiagent chemotherapy ± RT for local controlgg CR/PR q or inadequate response Refractory diseaser or progression CR/PR q or inadequate response Refractory diseaser or progression Relapse or persistent disease � Consider allogeneic transplant, aa as appropriate Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Version 3.2012, 07/09/12 © National Comprehensive Cancer Network, Inc. 2012, All rights reserved. The NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN . ® ® NCCN Guidelines Index NHL Table of Contents Discussion � See Suggested Treatment Regimens - Systemic Therapies (SYST-CAT B) (MFSS-A) � Alemtuzumabee � Clinical trial Relapse or persistent disease � Consider allogeneic transplant, aa as appropriate Clinical trial pIt is preferred that treatment occur at centers with expertise in the management of the disease. qPatients achieving a response should be considered for maintenance or taper regimens to optimize response duration. Patients who relapse often respond well to the same treatment. Patients with a PR should be treated with the other options in the primary treatment list to improve response before moving onto treatment for refractory disease. Patients with relapse or persistent disease after initial primary treatment may be candidates for clinical trials. rRefractory or intolerant to multiple previous therapies. aaThe role of allogeneic HSCT is controversial. See discussion for further details. eeLower doses of alemtuzumab administered subcutaneously has shown lower incidence of infectious complications. ffPatients with stage IV non-Sezary/visceral disease may present with more aggressive growth characteristics. If there is no evidence of more aggressive growth, systemic therapies from SYST-CAT B are appropriate. If aggressive growth is seen, then agents listed in SYST-CAT C are preferred. ggConsider adjuvant systemic biologic therapy ( SYST-CAT A) after chemotherapy to improve response duration. MFSS-8
SKIN-DIRECTED THERAPIES NCCN Guidelines Version 3.2012 Mycosis Fungoides/Sezary Syndrome SUGGESTED TREATMENT REGIMENSa SYSTEMIC THERAPIES For limited/localized skin involvement (Skin- Category A (SYST-CAT A) Limited/Local) � Retinoids (bexarotene, all-trans retinoic � Topical corticosteroidsb acid, isotretinoin [13-cis-retinoic acid], � Topical chemotherapy ( mechlorethamine acitretin) [nitrogen mustard] , carmustine) � Interferons (IFN-alpha, IFN-gamma) � Local radiation ( 12-36 Gy) � HDAC-inhibitors (vorinostat, romidepsin) e � Topical retinoids (bexarotene, tazarotene) � Extracorporeal photopheresisf � Phototherapy (UVB, nbUVB for patch/thin � Denileukin diftitox plaques; PUVA for thicker plaques) c � Topical imiquimod � Methotrexate ( � 100 mg q week) Category B (SYST-CAT B) For generalized skin involvement (Skin- � First-line therapies Generalized) � Liposomal doxorubicin � Topical corticosteroidsb � Gemcitabine � Topical chemotherapy (mechlorethamine � Second-line therapies [ nitrogen mustard] , carmustine) � Phototherapy (UVB, nbUVB, for patch/thin plaques; PUVA for thicker plaques) c � Total skin electron beam therapy (30-36 Gy) d (reserved for those with severe skin symptoms or generalized thick plaque or tumor disease, or poor response to other therapies) � Chlorambucil � Pentostatin � Etoposide � Cyclophosphamide � Temozolomide � Methotrexate (>100 mg q week) � Bortezomib � Low dose pralatrexate COMBINATION THERAPIES Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Version 3.2012, 07/09/12 © National Comprehensive Cancer Network, Inc. 2012, All rights reserved. The NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN . ® ® NCCN Guidelines Index NHL Table of Contents Discussion Category C (SYST-CAT C) g SYSTEMIC THERAPIES (continued) � Liposomal doxorubicin � Gemcitabine � Denileukin diftitox � Romidepsin � Low or standard dose pralatrexate � See regimens listed on TCEL-Bh Skin-directed + Systemic � Phototherapy + retinoide � Phototherapy + IFN � Phototherapy + photopheresisf � Total skin electron beam + photopheresisf Systemic + Systemic � Retinoid + IFN � Bexarotene + denileukin diftitox � Photopheresis f + retinoid � Photopheresis f + IFN � Photopheresis f + retinoid + IFN e a Safety of combining TSEBT with systemic retinoids or HDAC-inhibitors, such as See references for regimens MFSS-A 2 of 4 , MFSS-A 3 of 4, and MFSS-A 4 of 4 b vorinostat or romidepsin or combining phototherapy with vorinostat or romidepsin is Long-term use of topical steroid may be associated with skin atrophy and/or striae unknown. formation. This risk worsens with increased potency of the steroid. High-potency fPhotopheresis may be more appropriate as systemic therapy in patients with some steroid used on large skin surfaces may lead to systemic absorption. c blood involvement (B1 or B2). Cumulative dose of UV is associated with increased risk of UV-associated skin gPatients with large cell transformed (LCT) MF and stage IV non-Sezary/visceral neoplasms; thus, phototherapy may not be appropriate in patients with history of disease may present with more aggressive growth characteristics. In general, extensive squamoproliferative skin neoplasms or basal cell carcinomas or who agents listed in SYST-CAT C are preferred in these circumstances. have had melanoma. h d Combination regimens are generally reserved for patients with relapsed/refratory or It is common practice to follow TSEBT with systemic therapies such as interferon extracutaneous disease. or bexarotene to maintain response. MFSS-A 1 of 4
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