Squamous carcinoma of the larynx and hypopharynx in children: A distinct clinical entity?

Squamous carcinoma of the larynx and hypopharynx in children: An enigma and a dilemma

“…Its rarity is exemplified by the fact that a series of six children with SCC of the larynx (Lx) and hypopharynx (HP) in this issue [1] is the largest series ever reported from a single institution. Giving a detailed account of the clinical features and outcome, the authors have raised important questions regarding the etiology and management of this disease in children.…”

“…Even for the few adolescent tobacco users who develop cancer, it manifests only in adult life due to the long latency period of tumor initiation and promotion. Juvenile papillomatosis or what is now better termed recurrent respiratory papillomatosis (RRP) has been noted in 14 of the 43 children with glottic or laryngeal carcinomas, excluding supraglottic primaries [1,2]. Many of the remaining children may also have a preceding RRP considering the long history of dysphonia, no prior laryngeal examination or masking of a pre-existing RRP by the carcinoma [2].…”

“…Prior radiotherapy (RT), smoking and bleomycin have been reported as co-factors for malignant transformation of RRP [2,10]. RT likely hastens the malignant transformation as 8 of the 14 children with RRP and childhood Lx carcinoma had prior RT [1]. This, however, may not necessarily be a causal association as there is a strong selection bias with RT being given to children with more aggressive RRP, which may have an inherently higher risk of malignant transformation.…”

“…Tobacco, the main causative factor in adult Lx and HP cancer has never been reported as a sole risk factor in children [1,2]. Even for the few adolescent tobacco users who develop cancer, it manifests only in adult life due to the long latency period of tumor initiation and promotion.…”

“…It is notable that HPV, tobacco or other environmental risk factors have not been reported for hypopharynx, supraglottic or subglottic laryngeal carcinomas in children [1]. Instead, a characteristic cytogenetic abnormality of translocation 15;19 has been reported in a child with supraglottic cancer and in five other children or young adults with rapidly fatal midline upper respiratory tract carcinomas [13].…”