Muhammad Fasih Mansuri, Marya Hameed, Kanwal Laique, Ashraf Amir Ali, Syed Shariq Ullah


Rhabdomyosarcoma of childhood is a rare malignant soft tissue tumor of mesenchymal origin with an incidence of 4.5 cases per 1 million children. Obstructive jaundice is the most common presentation due to this neoplastic biliary obstruction. Among its histological subtypes, the embryonal subtype remains the most common with an incidence of around 54%. It commonly occurs in the genitourinary tract and head and neck region with a rare occurrence in the retroperitoneum and biliary tract. A 5-year-old child presented to us with obstructive jaundice and itching. On imaging, the peri-ampullary mass was misdiagnosed as biliary ascariasis/hydatid cyst; however, operative excision and histopathological correlation of the mass steered us towards the diagnosis of embryonal rhabdomyosarcoma. The patient was referred to the pediatric oncology department for chemotherapy and further follow-up. Despite being a rare entity, embryonal rhabdomyosarcoma of the biliary tract must be suspected in children presenting with obstructive jaundice and should get evaluation through multiple diagnostic tools in order to get an accurate diagnosis and avoid a possible misdiagnosis.

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