Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia

Abstract

Whilst rhabdomyosarcoma (RMS) is the third most common solid tumour in children, congenital RMS of the tongue is extremely rare and usually present as painless progressive mass since birth (Dagher and Helman in Oncologist 4:34-44, 1999; Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). In neonates, presentation with respiratory distress is unexpected as neonates are preferential nasal breathers and restricted oral breathing due to tumour usually poses no problem. We herein report a case of rhabdomyosarcoma of the tongue with co-existent unilateral choanal atresia, presenting with respiratory distress. The baby developed upper respiratory tract infection following which developed severe respiratory distress. Airway symptoms were precipitated as there was combined obstruction of both the nostrils due to infection or adenoid enlargement and unilateral chonal atresia. Treatment of respiratory distress in the presence of RMS and bilateral nasal pathology must first prioritise the security of the airway, before taking a multi-factorial approach to the therapy of the lingual mass (Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). This case illustrates the importance of vigilance with respect to coexistent nasal pathology, in order to avoid the occurrence of complete airway obstruction. We therefore feel that any diagnosis of lingual RMS should warrant a formal examination of both nasal cavities.

Epithelioid Type Tongue Rhabdomyosarcoma in a Pediatric Patient: A Case Report With Literature Review

“…We reviewed all the cases reported in the English language on tongue RMSs in pediatrics using the PubMed engine. Twenty cases were reported, including our case, summarized in Table 2 [7,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26]. Ten (50%) of the cases were female.…”

Loss of MST/Hippo Signaling in a Genetically Engineered Mouse Model of Fusion-Positive Rhabdomyosarcoma Accelerates Tumorigenesis

“…We note that MST loss led to a propensity for tumors in the tongue, suggesting a role for MST activity in the muscle of the tongue. There are many reports of RMS arising in the tongue [Ref (21) and references therein], and an increasing body of literature on the role of Hippo regulation in craniofacial development (22).…”