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2007-05-01-14 Answer to the case of the week #201 © Manson www.TheFetus.net


Answer to the case of the week #201

September 7-20, 2007

Francois Manson, MD.

Fécamp, France

 

 

This is a 16 year-old G1P0 scanned at 36th week of pregnancy. We discovered a liver tumor of the fetus which showed to be liver hemangioma. There was no associated malformation and fetal growth was normal.


36 weeks of pregnancy

Image 1: Bulky mass of the right upper fetal abdomen. The lesion appears quite well delimited, surrounding the gallbladder, and suggesting an right lobe hepatic tumor.

Image 2 and 3: The lesion appears heterogenous with several hypoechoic areas and hyperechoic foci suggesting liver calcifications (arrow).

 

Image 4 and 5: Parasagittal sections of the tumor (T). Image 5 shows downward displacement of the right kidney (K). The right adrenal gland couldn’t be visualized.

 

Image 6: Power Doppler image showing the vascularization of the lesion.

Image 7: Four-chamber view of the heart showing dilatation of the right atrium without any other signs of cardiac failure or malformation. Fetal growth was normal.

Prenatal MRI


Image 8: Coronal section showing well delimited tumor originates from right liver lobe. (L = lungs, S = stomach, B = bladder, T = tumor). Note the presence of T2 signal peripheral crescent shaped collection (arrow) suggesting a capsular rupture of the tumor.

Image 9 and 10:  The lesion appears heterogenous with essentially increased T2 signal aspect with several decrease T2 signal areas notably in its anterior part (*).

 

Image 11: Parasagittal section: note the respect of the right part of the diaphragm and the downward displacement of the kidney.

These prenatal aspects lead to suggest 3 possible diagnosis: hemangioma which is the most frequent hepatic tumor in fetuses and neonates, hepatoblastoma and hepatic metastasis of a choriocarcinoma. Potential risk of tumor rupture during labor led to a cesarean delivery at 39 weeks of pregnancy. The baby (3290 g) had a firm irregular palpable abdominal mass with discreet venous collateral circulation.

Postnatal sonography

Image 12: 2D ultrasound image shows quite homogenous tumorous mass within the liver with an hypoechoic anterior area.

Image 13: Postnatal color Doppler ultrasound: the tumor appears very vascularized with centripetal vascularization and peripheral displacement of the surrounding abdominal vessels leading to the diagnosis of hemangioma as corroborated by post natal MRI (data not available).

After birth, the tumor markers (Human Chorionic Gonadotrophin, Homo Vanillic Acid, Vanilmandelic Acid and Neuron Specific Enolase) were normal except for Alpha Fetoprotein which was lightly increased (123892 ng/ml at day one) but decreased to 71170 ng/ml at day three.

On the other hand, the baby developed several biochemical abnormalities.

• Anemia: 9.9 g/dl
• Thrombocytopenia: 41000/µl (N = 150000-400000)
• Hypofibrinogenemia: 0.82 g/l (N = 2-4)
• Fibrin split products: positive ++
• Fibrin soluble complexes: positive ++++

These features lead to the diagnosis of Kasabach Merritt syndrome. The baby received plasma and platelets infusions correcting the laboratory parameters. Embolization of the tumor have been considered but not realized.

 

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