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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 co-existent 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.
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PMID:Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia. 2740 48

Rhabdomyosarcoma (RMS) is a malignant form of neoplasm that originates from skeletal muscle. RMSs can exist anywhere in the human body but are more commonly detected in the neck region and extremities. The alveolar type is one of the subtypes of RMS that has a poor prognosis. Because the clinical manifestation of a tumour can be a painless mass, symptoms might be non-contributary to the diagnosis. Herein, a four-month-old girl was admitted to the emergency department with complaints of respiratory distress without a runny nose, cough, and fever. Recurrent effusions subsided with subsequent tube thoracostomy. Video-assisted thoracoscopic surgery (VATS) was performed to determine the aetiology of the recurrent effusion. The Tru-Cut biopsy obtained during VATS resulted in the diagnosis of alveolar rhabdomyosarcoma. Pleural effusion decreased, and the tube drainage was stopped rapidly after first vincristine, actinomycin-D, and cyclophosphamide chemotherapy cycle. Persistent and recurrent pleural effusions should alert physicians to rule out unusual diagnoses like that of our case.
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PMID:Persistent pleural effusion in an infant with an unusual diagnosis: congenital alveolar rhabdomyosarcoma. 3277 39


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