Management challenges of sinonasal alveolar rhabdomyosarcoma in infanthood: case report and literature review

Abstract

<p class="abstract">Alveolar rhabdomyosarcoma (aRMS) is an aggressive soft tissue malignant neoplasm. It’s not known to occur often in the sinonasal region of an infant when compared with other histological types. And due to its aggressive nature in deep-seated locations, it’s often difficult to manage efficiently, thus associated with worse prognosis. In this article, a rare case of an infant with right sided sinonasal aRMS was reported. Eight month old child with two month history of right sided nasal blockage, watery discharge and a growth in the right nose. There was no epistaxis, palatal or neck swelling. On examination, a polypoid mass was completely occupying the right nasal cavity. There were no palpable cervical lymph nodes. Diagnosis of allergic rhinosinusitis with nasal polyposis was made. The mass progressed with associated ipsilateral cheek swelling extending to the nasal bridge. A greyish white, friable mass was biopsied and was diagnosed histologically as aRMS. The child had a debulking surgery and adjuvant chemotherapy. She later developed tumour lysis syndrome and died. Management of infanthood sinonasal aRMS is challenging especially in low socioeconomic region like ours. A multi-disciplinary approach with intensified multimodality therapy is crucial to improving the management outcomes and quality of life. In all, it is advisable to take closer look at any swelling in the paediatric age group by carefully evaluating it with high degree of suspicion, early diagnosis and individualized treatment.</p><p> </p>