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Mycosis Fungoides in Children and AdolescentsA Systematic Review

Educational Objective
To describe the clinical features, immunophenotypes, various treatment options, and prognosis of mycosis fungoides in children and adolescents.
1 Credit CME
Key Points

Question  What are the clinical features of childhood mycosis fungoides (MF)?

Findings  In this systematic review of 571 children and adolescents with MF, the most common subtype was the hypopigmented form, followed by classic MF. Most patients with MF presented with early-stage disease, and the prognosis of MF seems to be more favorable than in the general population, although a significant delay before the establishment of a correct diagnosis of MF in childhood was associated with a poor prognosis.

Meaning  These findings suggest that although the prognosis of childhood MF is not unfavorable, delayed diagnosis may have an adverse effect.

Abstract

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, 1.99-75.26), 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 Relevance  The 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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Article Information

Accepted for Publication: January 14, 2021.

Published Online: March 3, 2021. doi:10.1001/jamadermatol.2021.0083

Corresponding Author: Woo Jin Lee, MD, PhD, Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea (uucm79@hanmail.net).

Author Contributions: Drs Jung and W. J. Lee had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Concept and design: Jung, Chang, M. W. Lee, W. J. Lee.

Acquisition, analysis, or interpretation of data: Jung, Lim, Won, W. J. Lee.

Drafting of the manuscript: Jung, Lim, W. J. Lee.

Critical revision of the manuscript for important intellectual content: Jung, Won, Chang, M. W. Lee, W. J. Lee.

Statistical analysis: Jung, Lim, W. J. Lee.

Administrative, technical, or material support: Won, W. J. Lee.

Supervision: Won, Chang, M. W. Lee, W. J. Lee.

Conflict of Interest Disclosures: None reported.

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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