Article

Biliary Rhabdomyoscarcoma Mimicking Choledochal Cyst

University of Texas, Southwestern Dallas, USA.
Journal of gastrointestinal and liver diseases: JGLD (Impact Factor: 2.2). 04/2009; 18(1):95-7.
Source: PubMed

ABSTRACT

A 3-year old male presented with complaints of pruritus, abdominal pain for 3 weeks and jaundice. Stools were acholic. There was jaundice, liver palpable 3 cm below right costal margin, no ascites or palpable masses. Serology revealed albumin 2.9 g/dl; ammonia of 31 mmol/l; elevated conjugated bilirubin, GGT, ALT, AST and alkaline phosphatase; alpha fetoprotein 1.3 ngm/ml; BhCG 9.1 IU/; PT 12.3 secs, INR 0.9; negative hepatitis A,B,C serology. CT scan showed a non-calcified heterogeneously enhancing mass centered at the liver hilum. MRCP showed a large heterogeneously enhancing, partially solid mass in the region of the porta hepatic. Liver biopsy revealed patternless proliferation of polymorphic oval to spindled shaped neoplastic cells. There was bile ducts distortion. Immunohistochemistry revealed positivity for vimentin, desmin.These findings were diagnostic for biliary rhabdomyosarcoma.There was no evidence of metastasis. Chemotherapy was initiated. Repeat imaging 6 months after initiation of treatment showed improvement in the degree of intrahepatic ductal dilatation and decrease in tumor bulk size. Rhabdomyosarcoma is the most common malignant tumor of the biliary tree in childhood. It is difficult to diagnose and delayed diagnosis influences the prognosis.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Accompanying rapid developments in hepatic surgery, the number of surgeries and identifications of histological types of primary hepatic space-occupying lesions (PHSOLs) have increased dramatically. This has led to many changes in the surgicopathological spectrum of PHSOLs, and has contributed to a theoretical basis for modern hepatic surgery and oncological pathology. Between 1982 and 2009 at the Eastern Hepatobiliary Surgery Hospital (EHBH) in Shanghai, 31 901 patients underwent surgery and were diagnosed as having a PHSOL. In this paper, we present an analysis of the PHSOL cases at the EHBH for this time period, along with results from a systematic literature review. We describe a surgicopathological spectrum comprising more than 100 types of PHSOLs that can be stratified into three types: tumor-like, benign, and malignant. We also stratified the PHSOLs into six subtypes derived from hepatocytes; cholangiocytes; vascular, lymphoid and hemopoietic tissues; muscular, fibrous and adipose tissues; neural and neuroendocrine tissues; and miscellaneous tissues. The present study provides a new classification system that can be used as a current reference for clinicians and pathologists to make correct diagnoses and differential diagnoses among various PHSOLs.
    Full-text · Article · May 2011 · World Journal of Gastroenterology

  • No preview · Article · Jan 2012
  • [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.
    No preview · Article · Dec 2012 · Indian Journal of Surgical Oncology
Show more