Case of the Week # 39

Daniel Margulies, MD and Nestor Matarasso, MD

November 24 - December 8, 2000

Buenos Aires, Argentina

This fetus has 3 examinations spanning the late second and third trimester (note the positions of the stomach). There are two distinct pathologies in these images.

First examination.

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Second examination (2 weeks later). 

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Third examination (4 weeks after the first exam).

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Answer

This fetus has 3 examinations spanning the late second and third trimester (note the positions of the stomach). There are two distinct pathologies in these images.

First examination.

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case0039-3
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Second examination (2 weeks later). 

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Third examination (4 weeks after the first exam).

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Findings

  1. There is clearly a placental mass and it is vascular. Little differential diagnoses here: this is a typical chorioangioma
  2. The other finding is of a vascular mass inferior to the heart and anterior to the stomach. This is highly characteristic of a liver hemangioendothelioma.

Hemangioendothelioma may present with the following: 

  • elevated maternal serum alpha-fetoprotein level 
  • cardiomegaly from high-output cardiac failure 
  • hepatomegaly 
  • a hepatic mass 
  • dilated intrahepatic vessels

The association with chorioangioma 1,2 or other placental lesions has been reported4.

Other primary liver tumors that could have been differential diagnosis include:

  • hepatoblastoma, 
  • infantile hemangioendothelioma, 
  • mesenchymal hamartoma, 
  • undifferentiated (embryonal) sarcoma, 
  • hepatocellular carcinoma,

Treatment of the hemangioendothelioma may involve3:

  • medical management (steroids, furosemide and alpha-interferon) or 
  • interventional modalities (hepatic artery ligation or embolization, resectional surgery, or orthotopic liver transplantation).

So the final diagnoses were chorioangioma and hemangioendothelioma

References:

1) Meirowitz NB, Guzman ER, Underberg-Davis SJ, Pellegrino JE, Vintzileos AMHepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion.J Clin Ultrasound 2000 Jun;28(5):258-63 

2) Marton T, Silhavy M, Csapo Z, Szende B, Papp Z Multifocal hemangioendothelioma of the fetus and placenta. Hum Pathol 1997 Jul;28(7):866-9 

3) Daller JA, Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS, Mazariegos G, Reyes J Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg 1999 Jan;34(1):98-105

4) Kanai N, Saito K, Homma Y, Makino S Infantile hemangioendothelioma of the liver associated with anomalous dilated and tortuous vessels on the placental surface. Pediatr Surg Int 1998 Mar;13(2-3):175-6

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