Grand Rounds

Infantile Hemangioendothelioma - How to treat?

Ira Shah, Amit Dey
Medical Sciences Department, Pediatric Oncall, Mumbai, India

Clinical Problem:
A 1 months old boy presented with progressive abdominal distension since 1 month of age and breathlessness for a day. There was no jaundice or pallor. On examination, weight was 3.86 kg. His vital parameters were normal and systemic examination revealed an approximately 4 x 3.5cms sized lump palpable in epigastric region which was not separate from the liver. There were dilated veins were present over abdomen. On investigations, ultrasound (USG) of abdomen showed multiple mass lesions in liver suspected to be hemangioendothelioma with a hypoechoic lesion seen in left liver lobe. Baseline bilirubin was 2.8 mg/dl (direct =1.2 mg/dl), SGOT was 46 IU/L, SGPT was 42 IU/L, total proteins were 7.9 gm/dl, albumin was 3.0 gm/dl, alkaline phosphatase was 479 IU/L. Hemogram showed hemoglobin of 12.5gm%, white cell count of 18,000 cells/cumm and platelets 427000 cells/cumm. Serum alpha feto protein was normal. MRI abdomen showed an enlarged liver with smooth margins and multiple lobulated lesions in right and left lobes, the largest being in left lobe measuring 4.1x3.5x5cm and right lobe being 2.8x2.3x2.9cm. The lesions on post contrast showed peripheral enhancement with multiple internal enhancing septa within. It also showed prominent vessels in anterior abdominal wall with increased caliber of celiac axis and marginally reduced caliber of infrarenal aorta confirming multiple lobe liver epitheloid hemangioendothelioma.

How to treat this child?

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