Lancet 1991 Oct 15;68(8):1737-41

Hepatocellular carcinoma in childhood. Clinical manifestations and prognosis

Ni YH, Chang MH, Hsu HY, Hsu HC, Chen CC, Chen WJ, Lee CY

Abstract
Department of Pediatrics, College of Medicine, National Taiwan University,
Taipei, Republic of China.

From December 1964 to November 1989, 71 children from 3 to 17 years of age with
the eventual diagnosis of hepatocellular carcinoma (HCC) presented at the
National Taiwan University Hospital (Taipei, Taiwan, Republic of China).
Forty-three of them had pathologic proof, whereas 28 were diagnosed on a
clinical and laboratory basis. A male predominance (M:F = 3.2:1) was noticed.
Most patients presented in a late, advanced stage. Abdominal pain and abdominal
mass were the major symptoms and signs, followed by anorexia, fever, and
internal bleeding. Hydrocele, purpura, and obstructive jaundice were rare
presenting symptoms. Hepatosplenomegaly, superficial venous engorgement, and
ascites were the main physical signs. The prognosis for such children with HCC
was very poor. Only 10% of the patients survived longer than 1 year after the
onset of the initial symptom. Among 49 patients who could be followed, only two
had long-term survival of over 5 years. One patient had a small HCC with
internal bleeding, whereas the other had a large HCC with abdominal distention.
Both received surgical resection, and a resection was repeated for tumor
recurrence in the patient with the large mass. The resectability of these 71
patients was low (9.8%). Resectability and nonicterus seemed to be the factors
indicating favourable prognosis. Observation indicated that the prognosis for
children with symptomatic HCC is grave but surgical resection, whenever
possible, should be carried out