Journal de pathologie clinique et expérimentale

Abstrait

Congenital Spindle Cell Rhabdomyosarcoma of Tongue: Rare Presentation as an Acute Emergency

Balasundaram P, Kumaresan K, Sathyanarayana MV and Sateesh M

Rhabdomyosarcomas (RMS) comprise the largest category of soft tissue sarcomas in pediatric population. RMS is the third most common solid tumor in children and about 30-40% arises from the head and neck region. Nearly 30-40% of the head and neck RMS arise from intraoral and pharyngeal structures, but involvement of the tongue by this tumour is extremely rare. About 4% of embryonal rhabdomyosarcoma affect infants and few cases are congenital. Most RMS of the tongue are reported to be of embryonal histology, although there are cases diagnosed with alveolar or undifferentiated RMS. Spindle cell rhabdomyosarcoma is a rare subtype constituting 5-10% of all cases of rhabdomyosarcomas. Congenital spindle cell RMS of tongue presenting with an acute onset of bleeding is extremely rare. A new-born female was referred to our institute (SRM Institutes for Medical Science, Chennai, India) for management of a tongue lesion, for which basic hematological and biochemical investigations were done. The patient developed sudden onset of severe bleeding from the lesion and emergency excision of the lesion was done under anesthesia and a diagnosis of spindle cell rhabdomyosarcoma was made, following routine histopathology and immunohistochemistry. Post-operative period was uneventful.