Embryonal rhabdomyosarcoma (ERMS) of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040 cGy in 28 fractions.
[Show abstract][Hide abstract] ABSTRACT: Malignant primary hepatic tumors in children include lesions unique to the pediatric age group and others that are more common in adults. Important considerations when evaluating a child with a liver tumor are the age of the patient, laboratory findings, and specific imaging features. The most common primary malignant hepatic tumor in the pediatric population, hepatoblastoma occurs almost exclusively in patients younger than 5 years with no history of liver disease. Hepatoblastoma is associated with elevated serum α-fetoprotein (AFP) level and appears predominantly solid. Hepatocellular carcinoma (HCC) is the most common malignant liver tumor in older children, often with a history of liver disease. HCC is associated with elevated serum AFP level and also appears as a solid mass. Fibrolamellar carcinoma occurs in adolescents without elevated AFP level and contains a T2-hypointense fibrous scar that usually does not enhance. Undifferentiated (embryonal) sarcoma occurs in young children, contains cystic and mucoid components, and mimics a cyst at magnetic resonance imaging and computed tomography but appears solid at ultrasonography. Epithelioid hemangioendothelioma is a multifocal vascular tumor in older children with a distinctive imaging appearance of confluent peripheral nodules with adjacent capsular retraction. Angiosarcoma rarely occurs in young children and frequently shows evidence of hemorrhage. Embryonal rhabdomyosarcoma of the biliary tree is unique to children, usually under 5 years of age, and frequently demonstrates an intraductal growth pattern. Knowledge of the pathologic features of these tumors and their imaging appearances can help radiologists offer an appropriate differential diagnosis and management plan.
[Show abstract][Hide abstract] ABSTRACT: Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor. It is a rare tumor in children but the most common cause of malignant obstructive jaundice in them. A 4-year-old child presented to us with obstructive jaundice and palpable liver. He was misdiagnosed as choledochal cyst on imaging studies; however, intraoperative and histopathological features confirmed the diagnosis of rhabdomyosarcoma. Mass excision and Roux en Y portoenterostomy was performed. Post operatively; the patient was put on multimodal chemotherapy. This, being an uncommon entity, is being reported with a review of the available literature.
Indian Journal of Surgical Oncology 12/2012; 3(4). DOI:10.1007/s13193-012-0186-7
[Show abstract][Hide abstract] ABSTRACT: We present a girl 21 months old with recurrent jaundice. Initially she presented fever of unknown origin but jaundice, white coloured stools and pruritus were observed 10 days later. She underwent endoscopic retrograde cholangiopancreatography with sphincterotomy; symptoms dissapeared. One month later, symptoms came back and, suspecting choledochal cyst the patient underwent endoscopic retrograde cholangiopancreatography for diagnostic confrmation and for placement of a biliary stent. The material obtained was sent for histopathology study and embryonal rhabdomyosarcoma of the biliary tree was diagnosed. The patient started chemotherapy following EpSSGRMS 2005 protocol. There was no evidence of metastasis. She completed treatment and to the day of this report she is free of illness.
Archivos argentinos de pediatría 08/2013; 111(4):e94-6. DOI:10.1590/S0325-00752013000400017 · 0.37 Impact Factor
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