Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides. Recent studies recognized indolent and aggressive subgroups of FMF, but there is controversy how patients presenting with plaques should be classified. The present study describes the histopathologic features of 40 FMF plaques. The aim of the study was to identify risk factors for disease progression and poor outcome in this group. METHODS: Clinical, histopathological, and immunophenotypical data from 40 patients with plaque stage FMF were reviewed and analysed for risk factors for disease progression and survival. RESULTS: After a median follow-up of 80 months, disease progression occurred in 20 of 40 patients. Percentage of atypical cells, cell size, percentage of Ki-67+ cells, and co-existent interfollicular epidermotropism, but not the extent of perifollicular infiltrates, were associated with disease progression and reduced survival, while extensive follicular mucinosis was associated with increased survival. CONCLUSIONS: This study underlines that FMF patients presenting with plaques represent a heterogeneous group and that a subgroup of these patients may have an indolent clinical course. It further shows that histological examination is a valuable tool to differentiate between indolent and aggressive disease.

Folliculotropic mycosis fungoides presenting with a solitary lesion: Clinicopathological features and long-term follow-up data in a series of 9 cases.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides, which in rare cases may present with a solitary lesion. Reported cases describe an excellent prognosis, but follow up was generally short. Herein, clinicopathologic characteristics, long-term follow-up data of 9 patients with solitary FMF are presented and differential diagnosis is discussed. METHODS: From a cohort of 203 patients with FMF, 9 cases with solitary FMF were selected. Clinical data and histological sections obtained at diagnosis and during follow up were reviewed. RESULTS: Skin lesions, in all patients located on the head, went into complete remission after treatment with radiotherapy (6 cases) or topical steroids (1 case) or regressed spontaneously (2 cases). After a median follow up of 89 months (range 51-203 months), 5 patients were still in complete remission, 2 patients had developed multiple skin relapses, while 2 patients had progressed to extracutaneous and fatal disease. Histologically, all patients showed marked folliculotropism, associated with syringotropism (4 cases) and/or follicular mucinosis (5 cases). Large-cell transformation was observed at presentation (2 cases) and during follow-up (3 cases). CONCLUSIONS: Long-term follow-up data indicate that patients with solitary FMF do not always have an indolent clinical course and therefore require long-term follow up.

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides.

IMPORTANCE: Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way. OBJECTIVE: To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF. DESIGN, SETTING, AND PARTICIPANTS: In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded. MAIN OUTCOMES AND MEASURES: Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques. RESULTS: In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival. CONCLUSIONS AND RELEVANCE: The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

[Childhood melanoma in the Netherlands]. / Melanomen bij kinderen in Nederland.

Childhood melanoma is a rare disorder which is often associated with a diagnostic delay. Worldwide, the incidence of cutaneous melanoma is rising in both adults and children. We describe three cases of childhood melanoma in the Netherlands which illustrate different aetiological aspects of the disease. The epidemiology of childhood melanoma in the Netherlands is discussed. In reviewing the literature, we wish to draw attention to important clinical aspects which may contribute to the early recognition of melanoma in children.