Background: Mycosis fungoides (MF) usually has an indolent course. However, some patients develop more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis.
Objectives: To describe the clinicopathological characteristics and prognostic value of pMF.
Methods: We retrospectively collected data from all patients with MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinicopathological characteristics of pMF at diagnosis (pMFD) were compared with those of a cohort of patients with nonpustular MF (NpMF).
Results: Thirty-three patients with pMF (including 22 with pMFD) and 86 with NpMF were included. Median age at diagnosis of pMF was 61 years [interquartile range (IQR) 50-75]. The median duration of follow-up for patients with pMFD was 32 months (IQR 14-49). Clinically, 33% of patients with pMF had pustules. Large cell transformation (LCT) occurred in 17 patients. Patients with pMFD had significantly more advanced-stage disease and showed more LCT at diagnosis than those with NpMF [50% vs. 7% (P < 0.001) and 23% vs. 0% (P < 0.001), respectively]. On multivariate Cox analysis, the presence of histological pustules at diagnosis was associated with shorter overall survival (OS) in all patients [hazard ratio (HR) 13.90, 95% confidence interval (CI) 2.40-79.00); P = 0.003] and in patients with early-stage disease (HR 11.09, 95% CI 1.56-78.82; P = 0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (subdistribution HR 13.90, 95% CI 2.43-79.00; P = 0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up in all patients with pMF was 37 months, with a 5-year OS rate of 25% (95% CI 0.06-0.50).
Conclusions: pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for patients with early-stage disease. Histological pustules at diagnosis of MF might represent an independent poor prognostic factor, to be confirmed by further studies. As pustules are not always identified clinically, pustules found on histology should be mentioned in MF pathology reports and should prompt discussion of closer follow-up.
Mycosis fungoides (or ‘MF’ for short) is a type of cancer of white blood cells in the skin. It is usually slow to progress, but some cases can be more aggressive. Pustules in skin samples taken from people with MF are rare, but they seem to be associated with a worse outcome. We investigated the features and outcomes of pustular MF to understand it better. We looked at people with pustular MF diagnosed between 2009 and 2020 at a number of French and European centres. We compared 33 people with pustular MF to 86 with MF without pustules. We found that people with pustular MF often had more advanced disease. This suggests that pMF might be more aggressive. We also found that having pustules was linked to shorter overall survival, even in patients with early-stage disease. This was also associated with a higher risk of the disease transforming into a more aggressive cancer. Our study findings suggest that finding pustules in skin samples from people with MF could be a sign of a poor prognosis. Patients could also have a shorter survival, even those with early-stage disease. These results could help inform clinicians and pathologists, and lead to closer monitoring and discussion of treatment options with patients.
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