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Special Feature |

Pathological Case of the Month FREE

José A. Jiménez-Heffernan, MD; Pilar López-Ferrer, MD; Emilio Burgos, MD; José M. Viguer, MD
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Arch Pediatr Adolesc Med. 1998;152(5):509-510. doi:10.1001/archpedi.152.5.509.
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A 7-YEAR-OLD white girl was admitted to the hospital with vomiting and anorexia of 3 weeks' duration. During this time she had also had a weight loss of 3 kg. A physical examination revealed diffuse hepatomegaly. A laboratory study disclosed the following values: aspartate aminotransferase, 889 U/L (reference range, 5-40 U/L); alanine aminotransferase, 503 U/L (reference range, 5-42 U/L); alkaline phosphatase, 613 U/L (reference range, 90-380 U/L); γ-glutamyl transpeptidase, 408 U/L (reference range, 10-50 U/L); lactate dehydrogenase, 971 U/L (reference range, 200-270 U/L); serum uric acid, 0.54 µmol/L (reference range, 0.15-0.45 µmol/L); and calcium, 3.37 mmol/L(13.4 mg/dL) (reference range, 2-2.63 mmol/L[8-10.5 mg/dL]). The remaining values, including serum α1-fetoprotein, immunoreactive parathyroid hormone, and calcitriol and vitamin D were within normal limits. A computed tomographic scan confirmed the presence of a large lobulated intrahepatic mass. The tumor was hypodense and showed areas of necrosis and calcification (Figure 1). Small, peripheral nodular lesions in the lower pulmonary lobes were interpreted as metastasis. No brain, renal, or skeletal pathologic condition was present.

Exploratory laparotomy with tumor biopsy was performed (Figure 2 and Figure 3). Neoplastic cells also expressed epithelial membrane antigen. An ultrastructural study was also performed (Figure 4).

Chemotherapy was started with a combination of carboplatin, vincristine sulfate, and epirubicin hydrochloride. Despite treatment, progressive neurologic deterioration ensued and the patient died 22 days after her admission to the hospital.




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