Mycosis fungoides in children and adolescents

Nanda, Arti; Al-Ajmi, Hejab

doi:10.1586/edm.13.29

Cited by 7 publications

(6 citation statements)

References 71 publications

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“…However, dozens of atypical variants of MF have been described . The proportion of patients who present with atypical variants appears to be higher in children than in adults . The clinical behavior and prognosis of each phenotypic variant also seems different; however, data on this subject are not available for childhood MF.…”

Section: Introductionmentioning

confidence: 99%

Mycosis Fungoides in Children and Adolescents

et al. 2021

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IMPORTANCE Comprehensive data on childhood mycosis fungoides (MF) is scarce. OBJECTIVE To describe clinical features, immunophenotypes, various treatment options, and prognosis of MF in children and adolescents. EVIDENCE REVIEW This systematic review searched MEDLINE via PubMed, Embase, Cochrane, and Scopus databases in October 2019. The search terms included mycosis fungoides, infant, children, and adolescent.No filter for the publication period was used, but studies written in a language other than English were excluded. Reference lists of the relevant articles were also searched manually. Case series and case reports were included if data on childhood MF were extractable. The Asan Medical Center database for cases of childhood MF was also searched. Patients were treated from January 1, 1990, to July 31, 2019, and were younger than 20 years at the time of diagnosis. The methodologic quality of the included studies was assessed with items from the Newcastle-Ottawa scale. Data were analyzed from December 9, 2019, to September 4, 2020.FINDINGS A total of 571 unique patients were included. The mean (SD) age at diagnosis was 12.2 (4.2) years; at onset, 8.6 (4.2) years. The female-to-male ratio was 1:1.6 (350 male patients [61.3%]). Among 522 patients with data available at diagnosis, stage 1 disease constituted 478 cases (91.6%), followed by stage 2 (39 [7.5%]) and stage 4 (5 [1.0%]). Among the 567 patients with data available, the most common variant of MF was the hypopigmented form (309 [54.5%]), followed by classic MF (187 [33.0%]). The MF lesions were predominantly the CD4 + and CD8 + immunophenotype in 99 (49.5%) and 79 (39.5%) of 200 patients, respectively. Among the treatments, narrowband UV-B was the most frequently used (150 of 426 [35.2%]). Most patients were alive with the disease (185 of 279 [66.3%]); 83 of 279 (29.8%) were in complete remission; and 11 of 279 (3.9%) had died by the last follow-up. A longer time from onset to diagnosis (hazard ratio [HR], 1.24; 95% CI, 1.06-1.45), granulomatous slack skin (HR, 12.25; 95% CI,, granulomatous MF (HR, 14.59; 95% CI, 1.31-162.00), a history of organ transplant (HR, 10.15; 95% CI, 0.98-105.37), and stage 2 disease at the time of diagnosis (HR, 10.22; 95% CI, 2.94-35.50) were associated with worse outcomes. CONCLUSIONS AND RELEVANCEThe findings of this review suggest that there is often a significant delay until the establishment of a correct diagnosis of childhood MF, which may be detrimental to the prognosis.

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Section: Introductionmentioning

confidence: 99%

Mycosis Fungoides in Children and Adolescents

et al. 2021

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“…13 Hence, MF was aptly called the "great imitator" by Zackheim et al 14 Sex Slight male preponderance is seen in childhood MF, however, many studies reported a ratio between males and females close to 1 : 1 15 as compared to 2 : 1 seen in adults. 4,11,16,17 Only few studies showed an increased incidence in female children with one of them reporting a ratio as high as 1 : 9 (M : F). 18,19 Genetics Several human leukocyte antigen (HLA) alleles have been associated with MF in adult population, namely HLA-DQB1 and HLA-DRB1, however, no statistically significant association was found between pediatric MF and any of these HLA alleles except HLA-B*73, the biological significance of which remains questionable due to its low frequency.…”

Section: Incidencementioning

confidence: 99%

“…Slight male preponderance is seen in childhood MF, however, many studies reported a ratio between males and females close to 1 : 1 15 as compared to 2 : 1 seen in adults 4,11,16,17 . Only few studies showed an increased incidence in female children with one of them reporting a ratio as high as 1 : 9 (M : F) 18,19 …”

Section: Epidemiologymentioning

confidence: 99%

Mycosis fungoides in pediatric population: comprehensive review on epidemiology, clinical presentation, and management

et al. 2022

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Mycosis fungoides (MF) is the most common type of cutaneous T‐cell lymphoma. However, it is rare in pediatric population. Most of the cases of pediatric MF present with hypopigmented patches and/or various other forms, which may often mimic common childhood dermatoses, thereby causing a delay in the diagnosis. There are no established treatment guidelines for pediatric MF. As the progression of childhood MF is extremely rare and it has an indolent course, it is usually diagnosed at an early stage (IA, IB, IIA), and hence phototherapy with a response rate of >80% is a well‐established effective treatment in children. However, as recurrences are frequently seen on stopping the therapies, a maintenance regimen and long‐term follow‐up is equally important. This article reviews the epidemiological factors, clinical presentations, diagnosis, and various treatment modalities used in pediatric MF. We analyzed and compared the data of almost 616 childhood MF cases from various studies undertaken from 1988 to 2021.

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“…However, in children and adolescents MF is very rare with an incidence of 0.05 new cases per year per 100,000 [ 3 ]. While in adults the female to male ratio was reported to be 1:2, Nanda et al described a 1:1 ratio in children and adolescents [ 4 ]. The difficulties in diagnosing early stage MF in children arise from the multitude of differential diagnoses with similar clinical morphology but much higher incidences in this specific age group.…”

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confidence: 99%

“…Skin lesions of the patient presented as erythematous macules and plaques. In contrast, other authors described a high frequency of hypopigmented MF lesions in children often associated with a predominance of CD8-positive atypical T-cells [ 8 ] and a non-Caucasian skin phenotype [ 4 ]. The literature is somewhat discordant in terms of TCR clonality when assessed in skin biopsies.…”

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confidence: 99%