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

Presented at the annual meeting of the American Society of Hematology in Atlanta, GA, December 8, 2012.
https://doi.org/10.1016/j.jaad.2013.03.036Get rights and content

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.

Section snippets

Patient selection

After the approval of the institutional review board, a retrospective chart review was performed, which identified 430 patients with confirmed diagnosis of MF seen between 1981 and 2012. Patients were classified according to revised criteria proposed by the International Society for Cutaneous Lymphomas (ISCL) and EORTC.23 Pathology reports of skin biopsy specimens were screened for the terms “granulomatous” and “histiocytic” infiltrate. We identified 101 patients as possible candidates.

Patient characteristics

In all, 27 patients with GMF were identified representing 6.3% of the total number of patients with MF at our center (Table I). Most patients with GMF were white, presented with a median age of 56 years (range 25-83), and had early-stage IA to IIA disease (70%). The interval from visible symptom onset to MF diagnosis was longer in the GMF group (mean 8.0 years) compared with the control group (mean 4.3 years, P = .04). Second malignancies developed in 44% of patients (12 of 27) with GMF

Discussion

To our knowledge, our retrospective case-case study encompasses the largest number of GMF cases to date and is the first study to analyze relative frequency, prognosis, and treatment responses. Previous studies have found an incidence of 1.6% to 1.8% of granulomatous infiltrates out of all cases of either primary or secondary cutaneous lymphoma,4, 5 but the relative frequency compared with classic MF has never been assessed to our knowledge. We observed a distinct granulomatous pattern in 6.3%

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    The first 2 authors contributed equally to this article.

    Funding sources: None.

    Disclosure: Dr Horwitz has received research grants from Celgene, Allos, Seattle Gen, Infinity Pharmaceuticals, and Kyowa Hakko Kirin. He has consulted for Celgene, Allos, Seattle Gen, Bristol-Myers Squibb, Genzyme, Kyowa Hakko Kirin Pharma, and Johnson & Johnson. Ms Li, and Drs Pulitzer, Myskowski, Dusza, Moskowitz, and Querfeld have no conflicts of interest to declare.

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