Journal of the American Academy of Dermatology · 2014
Syringotropic mycosis fungoides: clinical and histologic features, response to treatment, and outcome in 19 patients.
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de Masson Adèle, Battistella Maxime, Vignon-Pennamen Marie-Dominique, Cavelier-Balloy Bénédicte, Mouly Frédéric, Rybojad Michel, Bouaziz Jean-David, Petit Antoine, Saussine Anne, Ronceray Sophie, Le Gall François, Ram-Wolff Caroline, Assouly Philippe, Dereure Olivier, Joly Pascal, Dallot Alexiane, Dupuy Alain, Lebbé Céleste, Moulonguet Isabelle, Rivet Jacqueline, Janin Anne, Bagot Martine
Аннотация
BACKGROUND: A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the follicular subtype of MF in the World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.
OBJECTIVE: We sought to determine the clinicopathologic features and disease course of patients with STMF.
METHODS: A retrospective study was conducted to identify patients with STMF from 1998 to 2013.
RESULTS: Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n=16, 84%) with associated alopecia (n=12, 63%) and/or punctuated aspect (n=12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P=.02).
LIMITATIONS: Retrospective setting is a limitation.
CONCLUSIONS: In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.
OBJECTIVE: We sought to determine the clinicopathologic features and disease course of patients with STMF.
METHODS: A retrospective study was conducted to identify patients with STMF from 1998 to 2013.
RESULTS: Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n=16, 84%) with associated alopecia (n=12, 63%) and/or punctuated aspect (n=12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P=.02).
LIMITATIONS: Retrospective setting is a limitation.
CONCLUSIONS: In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.