2026-05-21 | Primary cutaneous lymphomas in children: a single-center retrospective analysis and narrative review of the literature.
Primary cutaneous lymphomas (PCLs) are uncommon in children, with mycosis fungoides (MF), lymphomatoid papulosis (LyP), and primary cutaneous small/medium CD4+ T-cell lymphoproliferative disorder (PCSM-TCLPD) representing the most frequent entities. Due to their rarity, available data derive mainly from case reports and small series, and diagnostic delays are frequent. This study aimed to describe the clinical, histopathological, and therapeutic features of pediatric PCLs in a single Italian referral centre and compare them with the literature evidence. We conducted a single-center, retrospective observational study including patients aged ≤16 years with a histologically confirmed diagnosis of PCLs, followed for at least six months at the Dermatology Unit of the University Hospital of Bologna between 2002 and 2024. Clinical, histological, therapeutic, and follow-up data were collected and analyzed. Among 354 PCL patients, 10 pediatric cases were identified: six MF, three LyP, and one PCSM-TCLPD. The mean age at diagnosis was 9.1 years. MF cases were mostly early-stage (IA, 5/6), with folliculotropic (67%), hypopigmented (50%), and classic (50%) variants; all were treated with topical corticosteroids and/or PUVA, with good long-term outcomes (67% complete remission). LyP patients presented at younger ages (mean 5.7 years) with type A (N.=2) and D (N.=1) subtypes; all achieved complete remission despite recurrences, one requiring methotrexate. The single PCSM-TCLPD case was cured with surgical excision. After a mean follow-up of 8.2 years, all patients were alive, most disease-free. Pediatric PCLs are rare but generally indolent, with an excellent prognosis. However, their clinical heterogeneity and diagnostic delay highlight the importance of early biopsy, integrated clinicopathological assessment, and long-term follow-up. Multicenter studies are needed to establish tailored treatment algorithms for children.
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2026-02-06 | A historical review of mycosis fungoides: from Alibert to mogamulizumab.
In 1806, French physician Baron Jean-Louis Alibert saw a man with a desquamating rash and skin tumours. Alibert considered this to be a variant of yaws. In 1829 Alibert named the condition mycosis fungoides (MF), meaning 'mushroom-like fungal disease'. Over 100 years later, French dermatologist Albert Sézary published papers from 1938 to 1949 detailing a mysterious disease containing 'cellules monstrueuses', describing cutaneous 'monster cells'. In 1961, these clinical findings were collated together into 'Sézary syndrome'. In the 1870s English dermatologist William Tilbury Fox published a dermatology atlas detailing cases similar to what we know now as MF, with the name 'fibroma fungoides'. The atlas described MF as a type of fungus, before giving a description of yaws and painting a clinical picture that differed from that of a lymphoma. Over the twentieth century, our understandings of the origins of MF were changing and by 1975 the classification system and term we now recognize as cutaneous T-cell lymphoma (CTCL) was developed. Neoplastic cells have been thought to arise from chronic activation of T cells via antigen-presenting cells due to inappropriate cytokines and C-C chemokine receptors. In 2018, the World Health Organization and European Organisation for Research and Treatment of Cancer officially recognized four variants of MF. These are the classic Alibert-Bazin variant and its three variants: folliculotropic MF, pagetoid reticulosis and granulomatous slack skin. Developments in immunohistochemistry for the T-cell receptor gene in the 1990s improved the diagnosis of CTCL; however, diagnosis is still challenging. Advanced MF therapies have evolved from cytotoxic chemotherapy to novel monoclonal antibodies such as mogamulizumab, targeting proteins on T-cell lymphoma cells.
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2025-12-04 | Spatial transcriptomics uncovers unique tumor microenvironments in folliculotropic versus classic mycosis fungoides.
The most common subtype of cutaneous T-cell lymphoma, mycosis fungoides (MF), is characterized by proliferation of malignant T cells in the skin. In the more clinically aggressive folliculotropic MF (FMF), malignant T cells localize to follicular epithelium, whereas in classic MF, they infiltrate the dermis and epidermis. How the localization of neoplastic T cells to the follicular niche contributes to the clinical aggression in FMF is unclear. To uncover the tumor microenvironmental differences between perifollicular FMF and dermal classic MF regions, we analyzed patient samples using spatial transcriptomics. Transcripts were collected from specific cell subsets within follicular (FMF) and dermal (classic MF) regions of interest to compare gene expression, spatial deconvolution, and pathway activity. Our work revealed that the neoplastic CD4 T cells around the hair follicles in FMF had a highly inflammatory phenotype, better adaptation to cellular starvation, higher metabolic activity, and enhanced antigen presentation. In contrast, cutaneous T-cell lymphoma-associated macrophages in FMF exhibited an immunosuppressive phenotype with decreased IL-2 and IFN signaling. These findings suggest that the follicular microenvironment may provide survival advantages to malignant T cells while promoting a dysregulated antitumor immune response. These observations provide insight into the mechanisms underlying the more aggressive clinical features of FMF versus classic MF.
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