Adèle de Masson1, Maxime Battistella2, Marie-Dominique Vignon-Pennamen3, Bénédicte Cavelier-Balloy3, Frédéric Mouly4, Michel Rybojad4, Jean-David Bouaziz1, Antoine Petit4, Anne Saussine4, Sophie Ronceray5, François Le Gall5, Caroline Ram-Wolff4, Philippe Assouly4, Olivier Dereure6, Pascal Joly7, Alexiane Dallot8, Alain Dupuy9, Céleste Lebbé1, Isabelle Moulonguet4, Jacqueline Rivet3, Anne Janin2, Martine Bagot10. 1. Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France. 2. Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France; INSERM UMR-S928, Paris, France. 3. Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. 4. Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. 5. Laboratoire de Pathologie, Hôpital Pontchaillou, Rennes, France. 6. Service de Dermatologie, Hôpital Saint-Eloi, Montpellier, France. 7. Service de Dermatologie, Hôpital Charles-Nicolle, Rouen, France. 8. Service de Dermatologie, Hôpital Robert-Ballanger, Aulnay, France. 9. Service de Dermatologie, Hôpital Pontchaillou, Rennes, France. 10. Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France. Electronic address: martine.bagot@sls.aphp.fr.
Abstract
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.
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.