Paediatric cutaneous lymphomas: a review and comparison with adult counterparts

Abstract

Primary cutaneous lymphomas (CL) in children is rare. Only a few studies focused specifically on paediatric CL and therefore little is known whether primary CL in children are similar to or different from their adult counterparts with respect to the clinicopathological presentation, behaviour and prognosis. An extensive literature search using PubMed/MEDLINE from January 1995 through July 2014 was undertaken for articles reporting cases of paediatric CL. In addition, we identified 31 children with CL in our institutions. Mycosis fungoides and lymphomatoid papulosis are the two most prevalent lymphoma forms in children. A few entities of cutaneous lymphomas such as cutaneous diffuse large B-cell lymphoma leg type, and Sézary syndrome have not been reported so far in children. Other lymphoma entities such as hydroa vacciniforme-like lymphoma are mostly seen in certain geographic areas (Asia, Central and South America). In the paediatric population, low-malignant indolent forms such as primary cutaneous marginal zone lymphoma and primary cutaneous follicle centre lymphoma are very rare, whereas the more aggressive forms of B-cell lymphomas, precursor lymphoblastic lymphomas, and blastic plasmacytoid dendritic cell neoplasm are the most common forms in children, mostly involving the skin secondarily. Most paediatric lymphomas have similar clinicopathological features and course as their adults counterparts, particularly in the group of cutaneous T-cell lymphomas. The spectrum of cutaneous B-cell lymphomas in children significantly differs from the one in adults. Diagnostic work-up and treatment of paediatric patients with lymphomas are best achieved in close collaboration with paediatric haematopathologists and oncologists.

Folliculotropic mycosis fungoides

“…The majority of patients develop the disease in adulthood; the mean age of onset has been reported to vary from 46 to 59 years . However, FMF can also occur in childhood and is a common variant of juvenile mycosis fungoides, a generally rare disease . On average, the diagnosis is made 18–48 months after the onset of skin lesions .…”

“…By contrast, there is only mild pruritus in children with FMF . In children, FMF appears to be a rather common manifestation of juvenile MF, which in turn is a rare disease overall . Hodak et al reported that 36 % of their juvenile MF patients exhibited follicular involvement .…”

Papulose Linfomatóide em Idade Pediátrica: Desafios no Diagnóstico e Follow-up

“…2,4 Nesta faixa etária, a idade média de início da doença foi aos 8,5±5 anos, sendo um lactente de 8 meses o doente mais novo reportado. 4,5 À semelhança da apresentação na forma adulta, observa-se um ligeiro predomínio no sexo masculino (Tabela 1).…”

“…7 A doença pode persistir ao longo da vida, contudo, em idade pediátrica parece ter maior tendência para a resolução espontânea, na medida em que quase metade dos casos atinge a remissão completa. 2 Um estudo que incluiu uma série de 25 crianças identificou uma correlação da PL com infeção viral prévia em 7 casos (28%) e com dermatite atópica, igualmente em 7 casos. Neste sentido, os autores sugerem que na idade pediátrica a PL será, provavelmente, mais uma condição reacional do que uma doença maligna.…”

Die follikulotrope Mycosis fungoides

“…Zumeist sind erwachsene Patienten betroffen, wobei das mittlere Manifestationsalter, je nach Literatur, zwischen 46 und 59 Jahren liegt . Die FMF kann sich jedoch auch im Kindesalter manifestieren und ist dort eine häufige Manifestationsform der insgesamt seltenen juvenilen Mycosis fungoides . Der Zeitraum zwischen Erstmanifestation der Hautveränderungen und Diagnosestellung liegt bei durchschnittlich 18–48 Monaten .…”