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Journal of the American Academy of... Dec 2015Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small... (Review)
Review
BACKGROUND
Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small population of patients with cutaneous T-cell lymphoma.
OBJECTIVE
We aimed to study the clinicopathologic features and overall prognosis associated with LF in patients with mycosis fungoides and Sézary syndrome.
METHODS
We conducted a single-center retrospective study, reviewing 1338 patients with mycosis fungoides seen from 1987 to 2015 at a tertiary referral center for cutaneous T-cell lymphoma, and a systematic review of 14 patients in the literature.
RESULTS
We identified 10 patients with mycosis fungoides who developed LF. Folliculotropism was seen in all patients with LF who had facial biopsy specimens. Radiation was a beneficial therapy. Complete remission was achieved in 1 patient and overall 5-year survival was 26%. Systematic review of 10 additional patients showed that all patients with LF, including ours, had stage-IV disease and some degree of blood involvement, but not all met criteria for Sézary syndrome.
LIMITATIONS
This was a retrospective study with a small sample size.
CONCLUSION
LF is associated with stage-IV cutaneous T-cell lymphoma, is often accompanied by folliculotropism and blood involvement, and can be treated with local electron beam therapy.
Topics: Adult; Aged; Biopsy, Needle; Cancer Care Facilities; Cohort Studies; Combined Modality Therapy; Facies; Female; Follow-Up Studies; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Mycosis Fungoides; Neoplasm Invasiveness; Neoplasm Staging; Positron-Emission Tomography; Retrospective Studies; Risk Assessment; Skin Neoplasms; Survival Rate; Tertiary Care Centers; Treatment Outcome
PubMed: 26476898
DOI: 10.1016/j.jaad.2015.09.017 -
Cancer Treatment Reviews Sep 2014A variety of therapeutic options are available for mycosis fungoides, the most prevalent subtype of cutaneous T cell lymphomas, but thus far, no regimen has been proven... (Review)
Review
BACKGROUND
A variety of therapeutic options are available for mycosis fungoides, the most prevalent subtype of cutaneous T cell lymphomas, but thus far, no regimen has been proven to be curative. A combination of treatments is a well-established strategy to increase the therapeutic efficacy. However, data from clinical trials analyzing such combinations for the treatment of mycosis fungoides are scarce.
OBJECTIVE
To analyze the available evidence on combination therapies with emphasis on the combination of psoralen with UVA phototherapy (PUVA), interferon-alpha and bexarotene with another treatment.
METHODS
Systematic literature review of the databases Embase, Cochrane, Medline, and Medline in Process.
RESULTS
Combination of PUVA with interferon-alpha or retinoids did not result in an increased overall response rate. Addition of methotrexate but not retinoids to interferon-alpha may increase the overall response rate. Bexarotene was investigated in one trial each with vorinostat, methotrexate or gemcitabine, whereby only methotrexate possibly enhanced the effect of bexarotene.
CONCLUSION
For mycosis fungoides, no combination treatment has been demonstrated to be superior to monotherapy. Based on our analysis, we conclude that in certain clinical situations, patients may benefit from a combination of PUVA with interferon-alpha or a retinoid or a combination of the latter two. Furthermore, patients in advanced stages may benefit from the combination of methotrexate and interferon-alpha or bexarotene. Finally, the combination of bexarotene with either vorinostat or gemcitabine did not increase the overall response rate but resulted in more pronounced side effects and cannot be recommended.
Topics: Antineoplastic Combined Chemotherapy Protocols; Bexarotene; Combined Modality Therapy; Deoxycytidine; Ficusin; Humans; Hydroxamic Acids; Interferon-alpha; Methotrexate; Mycosis Fungoides; Phototherapy; Retinoids; Skin Neoplasms; Tetrahydronaphthalenes; Vorinostat; Gemcitabine
PubMed: 24997678
DOI: 10.1016/j.ctrv.2014.06.005