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Extrahepatic bile ducts

Tumors

Rhabdomyosarcoma of extrahepatic bile ducts


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 16 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● <1% of bile duct malignancies are sarcomas
● Most common sarcoma is embryonal rhabdomyosarcoma
● In children past infancy, #2 cause of obstructive jaundice (after choledochal cysts)
● Usually 3-4 years old with fever, weight loss, jaundice
● Usually embryonal or botyroid subtypes
● 40% have metastases, usually to regional lymph nodes, distal metastases less common
● Often extends into liver or gallbladder

Sites
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● Common bile duct (77%)

Case reports
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● 1 1/2 year old child with tumor mimicking a choledochal cyst (J Cancer Res Ther 2007;3:40)
● 3 year old boy with tumor mimicking a choledochal cyst (J Gastrointestin Liver Dis 2009;18:95)
● 3 year old with endoscopic retrograde cholangiopancreatography for diagnosis and treatment (World J Gastroenterol 2008;14:4823)

Treatment
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● Surgery, chemotherapy, radiation therapy may produce long term survival

Clinical images
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ERCP images

Gross description
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● Botyroid tumors have white-tan, edematous, polypoid grapelike features
● Tumor 3-14 cm

Micro description
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● Embryonal tumors have condensed (cambium) layer of primitive spindle cells just below surface epithelium
● Rhabdomyoblasts may be prominent with abundant eosinophilic cytoplasm
● May have relatively acellular layer with myxoid stroma

Micro images
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Some rhabdomyosarcoma cells have eosinophilic cytoplasm suggesting skeletal muscle differentiation but most are very undifferentiated in myxoid stroma; tumor cells are strongly myogenenin+

Positive stains
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● Desmin, muscle specific actin, MyoD1, variable myoglobin

Electron microscopy description
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● Thick and thin filaments

End of Extrahepatic bile ducts > Tumors > Rhabdomyosarcoma of extrahepatic bile ducts


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