Mycosis Fungoides in Children and Adolescents: A Series of 23 Cases

Eltayeb

Dermatology Research and Practice

et al. 2023

The treatment options for mycosis fungoides (MF) have been expanding but unfortunately many of the currently used treatment modalities are unavailable in Egypt and other African/Arab countries. In addition, there is a lack of consensus on the treatment of hypopigmented MF (HMF), which is a frequently encountered variant in our population. We aimed to develop regional treatment guidelines based on the international guidelines but modified to encompass the restricted treatment availability and our institutional experience. Special attention was also given to studies conducted on patients with skin phototype (III-IV). Treatment algorithm was formulated at Ain-Shams cutaneous lymphoma clinic through the collaboration of dermatologists, haematologists, and oncologists. Level of evidence is specified for each treatment option. For HMF, phototherapy is recommended as a first line treatment, while low-dose methotrexate is considered a second line. For early classical MF, we recommend Psoralen-ultraviolet A (PUVA), which is a well-tolerated treatment option in dark phenotype. Addition of either retinoic acid receptor (RAR) agonist and/or methotrexate is recommended as a second line. Total skin electron beam (TSEB) is considered a third-line option. For advanced stage, PUVA plus RAR agonist and/or methotrexate is recommended as first line, TSEB or monochemotherapy is considered a second line option. Polychemotherapy is regarded as a final option. All patients with complete response (CR) enter a maintenance and follow-up schedule. We suggest a practical algorithm for the treatment of MF for patients with dark phenotype living in countries with limited resources.

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Suggested Guidelines for the Treatment of Mycosis Fungoides in Countries with Limited Resources

Eltayeb

Dermatology Research and Practice

et al. 2023

“…The most widely used treatment was Psoralen Ultra-Violet A (PUVA), reported in 8 studies, in 3 as monotherapy (1 case report with CR, 1 original article with CR 70% and PR 26%, 1 original article with ORR of 76%) 5 , 24 , 25 and in 5 studies in association with other drugs: in 2 case reports PUVA was combined with bexarotene, with CR in both cases and subsequent stem cell transplantation following progression in one of them, 27 , 28 whilst in another report it was associated with Interferon, with CR. 31 This treatment, among others, was mentioned in the Japanese case series by Kamijo et al, yet it was less commonly used compared to UVB therapy (ie, 10% vs 50% of the patients in the cohort). 29 In the Dutch experience, PUVA was administered as monotherapy to 31%, combined with retinoid or IFN-alpha to 9%, and with RT to 13% of the patients, respectively, with ORR of 76%.…”

Section: Resultsmentioning

confidence: 99%

Folliculotropic Mycosis Fungoides: Current Guidance and Experience from Clinical Practice

Roccuzzo¹,

Mastorino²,

Gallo³

et al. 2022

CCID

Introduction Folliculotropic mycosis fungoides (FMF) is the most frequent variant of mycosis fungoides (MF), with clinical features which differ from the classic form. As for therapeutic options, the latest guidelines on MF agree on a stage-driven strategy, in consideration of clinical presentation, symptom burden and patient’s comorbidities. Materials and Methods A search on MEDLINE, PubMed, Scopus and Cochrane Library was conducted to gather the latest evidence on FMF clinical management. Manuscripts published in the last five years (January 2017–April 2022) were included. Our single-center experience was also described. Results A total of 15 articles were analyzed, with a total of 432 patients (disease stage from IA to IVA2). The most widely-used treatment was psoralen ultra-violet A (PUVA) in monotherapy or in association with other drugs. Oral retinoid-based therapy was also described as a therapeutic option alone or in combination. Other therapy reported were based on Brentuximab Vedotin, Mogamulizumab, Carmustine, topical steroids, tazarotene and excimer laser, interferon, nitrogen mustard, imiquimod, systemic chemotherapy, extracorporeal photopheresis and stem cell transplantation. Discussion FMF is characterized by specific clinical-pathologic features. Advanced forms assume characteristics more similar to classic MF (infiltrated plaques and nodules), whilst early stages can present in a wide range of clinical forms (acneiform lesions, follicular-like keratoses, erythematous patches). As for therapeutic options, in absence of specific guidelines, a high number of treatments are described in clinical practice, with variable results. Phototherapy in all its forms, especially as PUVA, appears to have the greatest initial therapeutic success. Retinoids, although widely used, appear to be poorly effective in monotherapy, particularly acitretin. Combination treatment with phototherapy seems to be advisable. Ionizing treatments, such as radiotherapy and TSEBT, appear effective, at least in the short term. Overall, an integrated approach is mandatory for the inconstant course of the disease and its multidisciplinary nature.

“…19 -21,23 -29 Pediatric MF may show the classic presentation of erythematous patches and plaques with lymphocyte epidermotropism, yet various atypical clinical variants have been reported, including hypopigmented, psoriasiform, granulomatous slack skin, follicular, poikilodermic and purpuric. 19,21 -23,29,30 The hypopigmented variant seems to be overrepresented in childhood MF, 19,22,24,25,28 -32 and this clinical subtype is more often associated with a cytotoxic phenotype with expression of CD8 and TIA-1 reflecting the adequate antitumor immune response, which explains the better prognosis of that variant. 31,33 The association of MF with PLC has already been noted in other studies 33,34 and has been reported in a ratio as high as four out of five patients.…”

Section: Discussionmentioning

confidence: 99%

“…35 PLC has been described to be highly CD8 + 36 and might be a predisposing factor in MF, but MF can mimic PLC, and be initially misdiagnosed. 32 Most pediatric MF patients present with stage IA or IB disease and have an excellent prognosis. 19 -21,23 -28 Granulomatous slack skin (GSS) is a very rare MF subtype that has seldomly been reported in children.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Cutaneous Hematologic Disorders: Cutaneous Lymphoma and Leukemia Cutis—Experience of a Tertiary-Care Pediatric Institution and Review of the Literature

et al. 2022

Background Cutaneous hematologic malignancies are rare in children, and the literature about them is still sparse. Objective The purpose of our study was to report our experience with pediatric cases of cutaneous hematologic disorders and describe their clinical and histological features. Methods Data were retrospectively collected from the histopathologic database of the CHU Sainte-Justine, University of Montreal, Montreal, Canada. All patients up to 18 years of age with a diagnosis of a primary cutaneous lymphoma (including lymphomatoid papulosis), secondary cutaneous lymphoma or cutaneous manifestations of leukemia, followed from 1980 to 2019 at our center were reviewed. Results Thirty-six patients were included. Age at presentation ranged from birth to 18 years of age (mean 7.83 ± 5.16; median 7.0). Ten different hematologic disorders were identified according to the WHO-EORTC classifications: lymphomatoid papulosis (10 cases), mycosis fungoides (6 cases), anaplastic large cell lymphoma (4 cases), pre-B acute lymphoid leukemia (5 cases), primary cutaneous marginal zone B-cell lymphoma (4 cases), primary cutaneous CD4+medium T-cell lymphoproliferative disorder (1 case), extranodal NK/T-cell lymphoma (1 case), hydroa vacciniforme-like lymphoproliferative disorder (1 case), B-cell lymphoblastic lymphoma (1 case) and acute myeloid leukemia (3 cases). Conclusion The most common subtype of cutaneous hematologic disease in our single institution study was lymphomatoid papulosis (type A and type C), followed by mycosis fungoides. Recognition of this large clinical and histological spectrum by dermatologists is important because diagnosis is often established by biopsy of skin lesions, even in secondary cutaneous cases. Moreover, the clinicopathological correlation is of utmost importance for the final diagnosis of those pathologies.