A case-control study of clinicopathologic features, prognosis, and therapeutic responses in patients with granulomatous mycosis fungoides

Abstract

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.

Keywords: CR; EORTC; European Organization for Research and Treatment of Cancer; GMF; ISCL; International Society for Cutaneous Lymphomas; LCT; MF; OS; PFS; PR; T-cell receptor; T-helper; TCR; Th; complete response; cutaneous T-cell lymphoma; disease progression; granulomatous mycosis fungoides; histopathology; incidence; large cell transformation; mycosis fungoides; outcome; overall survival; partial response; progression-free survival; treatment response.