Syringotropic mycosis fungoides: clinical and histologic features, response to treatment, and outcome in 19 patients

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.

Keywords: cutaneous T-cell lymphoma; follicular; folliculotropism; mycosis fungoides; syringotropic; syringotropism.