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Malignant liver tumors in infants and children

Abstract

At the Children’s Hospital in Washington, D.C., 18 patients have been seen in the past 12 years with primary tumors of the liver. The tumors were divided as follows: 13 hepatoblastoma, 2 hepatocellular carcinoma, 2 rhabdomyosarcoma, and 1 mixed mesenchymal sarcoma. Of the patients with hepatoblastoma, all were seen by 4 years of age, and 8 were under 1 year when first seen. Three had pulmonary metastases when first seen. The 2 patients with hepatocellular carcinoma were 8 and 12 years old, respectively. The patients with embryonal rhabdomyosarcoma were 18 and 30 months of age when seen; 1 had a primary tumor arising in the liver, the other had a tumor in the bile ducts extending into the liver. Ten of the 13 children with hepatoblastoma underwent lobectomy; 5 of these 10 are alive, 1 with known metastases. The other 4 are presumed cured. One of the children with hepatic carcinoma had severe preexisting cirrhosis of the liver and has had 3 local limited excisions. One of the patients with rhabdomyosarcoma is alive and cured after hepatic lobectomy. As yet, there is no known effective chemotherapeutic regimen. Radiation therapy has been useful only in shrinking the tumor, but neither of these modalities has resulted in cure. At the present time, the only known cures are achieved by early detection and successful surgical removal.

Résumé

Au Children’s Hospital de Washington, D.C., 18 malades ont été traités au cours des 12 dernières années pour tumeur primitive du foie: 13 hépatoblastomes, 2 carcinomes hépatocellulaires, 2 rhabdomyosarcomes, 1 mésenchymome malin. Les patients atteints d’hépatoblastome avaient tous moins de 4 ans lors du premier examen et 8 avaient moins d’un an; 3 présentaient des métastases pulmonaires. Les deux malades atteints de carcinome hépatocellulaire avaient 8 et 12 ans. Les deux enfants porteurs de rhabdomyosarcome embryonnaire avaient 18 et 30 mois; l’un avait une tumeur primitive du parenchyme hépatique, l’autre une tumeur de la voie biliaire infiltrant le foie. Sur les 13 hépatoblastomes, 10 ont eu une lobectomie: 5 sont en vie, dont 1 avec métastases, les 4 autres étant considérés comme guéris. Un des enfants atteint de carcinome hépatocellulaire souffrait d’une cirrhose hépatique pré-existante et avait déjà subi 3 exérèses locales limitées. Un des malades atteints de rhabdomyosarcome est guéri après lobectomie. A l’heure actuelle, il n’existe pas de chimiothérapie efficace. La radiothérapie ne peut que réduire le volume de la tumeur. Aucune de ces 2 modalites thérapeutiques ne peut guérir le malade et, aujourd’hui, les seules guérisons ont été obtenues grâce au diagnostic précoce et à l’exérèse chirurgicale.

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Supported in part by National Institute Grant Number 03888.

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Randolph, J., Chandra, R. & Leiken, S. Malignant liver tumors in infants and children. World J. Surg. 4, 71–81 (1980). https://doi.org/10.1007/BF02393100

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Keywords

  • Sarcoma
  • Hepatocellular Carcinoma
  • Pulmonary Metastasis
  • Rhabdomyosarcoma
  • Chemotherapeutic Regimen