BACKGROUND: Granulomatous mycosis fungoides (GMF) is an uncommon variant of mycosis fungoides (MF). OBJECTIVE: We sought to analyze the relative frequency, clinicopathologic characteristics, prognosis, and therapeutic responses of GMF. METHODS: We conducted a retrospective case-control study of patients with GMF and age- and stage-matched patients with classic MF between 1981 and 2012. RESULTS: A total of 27 patients with GMF were identified, representing 6.3% of all patients with MF at our center. Skin manifestations were similar to classic MF having an atypical lichenoid CD4(+) CD8(-) lymphocytic infiltrate with interstitial histiocytes and/or perivascular granulomas with giant cells. Fewer patients with GMF achieved a partial response or complete response with topical (57% vs 83%; P = .002) or ultraviolet light (62% vs 90%; P = .006) therapy. The 5- and 10-year progression-free survival rates were significantly lower in patients with GMF (59% and 33%) compared with patients with classic MF (84% and 56%; P = .02), but overall survival was similar between groups (86% and 72% vs 85% and 85%; P = .54). LIMITATIONS: The retrospective methodology may underestimate the frequency of GMF. The median follow-up time may be too short to detect possible differences in overall survival. CONCLUSION: More frequent disease progression and poorer response to skin-directed therapies were observed in patients with GMF. Our findings may be helpful in selecting the most appropriate treatment for these patients.
BACKGROUND:Granulomatous mycosis fungoides (GMF) is an uncommon variant of mycosis fungoides (MF). OBJECTIVE: We sought to analyze the relative frequency, clinicopathologic characteristics, prognosis, and therapeutic responses of GMF. METHODS: We conducted a retrospective case-control study of patients with GMF and age- and stage-matched patients with classic MF between 1981 and 2012. RESULTS: A total of 27 patients with GMF were identified, representing 6.3% of all patients with MF at our center. Skin manifestations were similar to classic MF having an atypical lichenoid CD4(+) CD8(-) lymphocytic infiltrate with interstitial histiocytes and/or perivascular granulomas with giant cells. Fewer patients with GMF achieved a partial response or complete response with topical (57% vs 83%; P = .002) or ultraviolet light (62% vs 90%; P = .006) therapy. The 5- and 10-year progression-free survival rates were significantly lower in patients with GMF (59% and 33%) compared with patients with classic MF (84% and 56%; P = .02), but overall survival was similar between groups (86% and 72% vs 85% and 85%; P = .54). LIMITATIONS: The retrospective methodology may underestimate the frequency of GMF. The median follow-up time may be too short to detect possible differences in overall survival. CONCLUSION: More frequent disease progression and poorer response to skin-directed therapies were observed in patients with GMF. Our findings may be helpful in selecting the most appropriate treatment for these patients.
Authors: Daniel J Lewis; Ashley E Turkeltaub; Julia Dai; Priyadharsini Nagarajan; Kerri E Rieger; Cesar A Nunez; Youn H Kim; Madeleine Duvic Journal: JAAD Case Rep Date: 2017-06-15
Authors: Maria Isabel Beatriz Lim Puno; Ma Teresa Ela Dimagiba; Ma Jasmin Jacinto Jamora; Filomena Legarda Montinola; Patricia Ty Tinio Journal: JAAD Case Rep Date: 2017-07-16