Biliary tract embryonal rhabdomyosarcoma
Updates to Case Attributes
The most common cause of benignbenign obstructive jaundice in childhood isarecholedochal cystcysts, while the most common malignant cause of obstructive jaundice in such agechildhood is Rhabdomyosarcomarhabdomyosarcoma. However, in this case, the secondother differential diagnosis is Lymphoma (Homogeneouslymphoma (homogeneous mass enhancement and associated with lymphadenopathy), while RMSrhabdomyosarcoma mainly shows heterogeneous enhancement.
Certain reports that RMS may arising from the preexisting choledochal cyst.
The provisional diagnosis was Embryonal RMS.
-<p>The most common cause of benign obstructive jaundice in childhood is<strong> </strong>choledochal cyst, while the most common malignant cause of obstructive jaundice in such age is Rhabdomyosarcoma. However, in this case the second differential diagnosis is Lymphoma (Homogeneous mass enhancement and associated with lymphadenopathy), while RMS mainly shows heterogeneous enhancement.</p><p>Certain reports that RMS may arising from the preexisting choledochal cyst.</p><p>The provisional diagnosis was Embryonal RMS.</p>- +<p>The most common cause of benign obstructive jaundice in childhood are<strong> </strong>choledochal cysts, while the most common malignant cause of obstructive jaundice in childhood is rhabdomyosarcoma. However in this case, the other differential diagnosis is lymphoma (homogeneous mass enhancement and associated with lymphadenopathy), while rhabdomyosarcoma mainly shows heterogeneous enhancement.</p>
Systems changed:
- Oncology
- Hepatobiliary
Updates to Study Attributes
TISSUE ORIGIN: 1- Omentum. 2- PortahepatisPorta hepatis lymph node. 3- PortahepatisPorta hepatis mass.
DIAGNOSIS: Omentum, portahepatisporta hepatis lymph node and portahepatisporta hepatis mass; biopsies: Consistent with embryonal rhabdomyosarcoma. The tumor cells are positive for Desmin, Myogenin (Focal) and Glypican-3. They are negative for Pan-CK, SALL4 and Heppar1 immunostains.
Image Pathology (Desmin) (update)
Image Pathology (Myogenin) (update)
Image Pathology (Glypican-3) (update)
Image Pathology (Pan-CK, SALL4 & Heppar1) (update)
Updates to Study Attributes
There is an ill defined-defined mass lesion in the portahepatisporta hepatis encasing the adjacent structures (hepatic artery, portal vein and common bile duct) causing moderate dilatation of the intra hepaticintrahepatic biliary tree. Associated with multiple lymphadenopathy.
Image CT (C+ portal venous phase) (update)
Image CT (C+ portal venous phase) (update)
