Undifferentiated Sarcoma of the Liver in an Adult: A Case Report

Hiroshi Itoh, Ryouhei Izumi, Hisashi Hirosawa, Naotaka Kadoya, Wataru Fukushima, Keiichi Muraoka, Itsurou Terada, Tohru Yamazaki, Teisuke Hirono and Katsuhiko Saitoh^*

Department of Surgery and Pathology^*, Toyama City Hospital

A 24-year-old man who complained of right upperquadrant pain was admitted to our hospital. Biochemical studies showed elevations of carcinoembryonic antigen and alpha fetoprotein. Abdominal ultrasonography showed a well defined mass in the right lobe of the liver with multicystic areas. Contrast-enhanced computed tomography (CT) revealed a well-circumscribed enhancing tumor approximately 15 cm in diameter with focal low attenuation areas. Selective arteriography of the celiac axis demonstrated a hypervascular tumor in the right lobe of the liver and an arteriovenous shunt. CT during arterial portography (CT-AP) revealed a defect in portal flow from the superior mesenteric artery and some revealed entrapped branches of the portal vein in the area. With a preoperative diagnosis of hemangiosarcoma of the liver, right hemihepatectomy with resection of adherent diaphragm was performed. Macroscopic examination of the resected specimen showed a solid tumor with sharp demarcation between the tumor margin and adjacent normal liver. The cut surface of the resected tumor was variegated, predominantly tan-yellowish, and exhibited cystic areas of variable size that contained hemorrhagic fluid and gelatinous material with focal necrosis. Histologically, the major component of the tumor was sarcoma, dilated epithelium-lined structures were present, there were no differentiated components, and the tumor contained cells with intracytoplasmic hyaline globules that were periodic acid-Schiff positive, findings which were consistent with undifferentiated sarcoma of the liver. Immunohistochemically, strong CD34 immunoreactivity was observed in the cytoplasm of the tumor cells, but no markers of histiocytes, neurons, muscle-specific actin, or vessels were detected. CT-AP and immunohistochemical studies may be new diagnostic methods for this rare tumor.

Key words
undifferentiated sarcoma of the liver, CT during arterial portography, CD34

Reprint requests
Hiroshi Itoh Department of Surgery, Toyama City Hospital, 2-1 Imaizumihokubu, Toyama, 939-8511 JAPAN

Accepted
December 22, 1999

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