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A male patient, 50 years old, was hospitalized in our department because of repeated vague pain under the right rib for more than 3 months. Two weeks prior to his hospitalization the pain became severe accompanied by fever. The right subcostal vague pain and discomfort occurred 3 months before admission, with no radiating pain elsewhere, no vomiting, no diarrhea or jaundice but with chills and pyrexia. In the afternoon the fever intensified, with a body temperature of about 38.5°C. There was the vague pain and discomfort under the right rib, which was paroxysmally intensified.
Physical Examination
The patient was conscious with neither yellowing of the skin nor of the sclera. Furthermore there was no bleeding points or ecchymosis and rash. No liver palm and spider telangiectasia were found and there were no palpable lymph nodes. The abdomen was flat and soft and there were no varicose veins in the abdominal wall, the liver was palpable, the upper margin of the liver lay under the sixth rib on the median line of the right collarbone and the inferior margin of the liver lay at 2 cm below the right flank. A hard substance and obtuse margin with slight haphalgesia were found at 4 cm below the appendix ensiformis, and it was not palpable below the left collarbone at the edge of the spleen. Murphy’s sign was negative, percussion pain in the hepatic region was positive, percussion pain in the double kidney region was negative, excursive dullness negative and bowel sounds normal. The color ultrasonic B of the liver showed there was a low-echo space occupying lesion at the right posterior lobe of the liver, with a scope of about 8.0 cm × 9.1 cm, a distinct boundary and an uncertain character.
Hospitalization and Diagnosis
The quality of the space occupying focus was examined. After hospitalization the result of routine blood examination was as follows: WBC 9.9×109/L, N 73.5%, Hb 109 g/L, PLT 546×109/L. Liver function results: albumen 26 g/L, TBil 21.0 μmol/L, ALT 51 IU/L, AST 48 IU/L. AFP quantitation: 2.16 μg/L. The result of a fine needle aspiration and pathological biopsy for the space occupying lesion of the liver, conducted under guidance of ultrasonic B (on May 6th, 2005): capillary angiogenesis was found among spindle cells, hemangioendothelioma of liver was considered first.
A tumorectomy at segment VII of the right posterior lobe of the liver was conducted with general anesthesia and during the operation on the tumor was found at segment VII of the right posterior lobe of the liver. The size of the tumor was about 8 cm × 9 cm × 10 cm, being hard with a distinct boundary. The tumor was adherent with a part of the diaphragmatic muscle, and no evident metastatic lesions were found in the liver or abdominal cavity. After postoperative treatment, such as fluid replacement, antibiotics, hemostasis, protection of the liver and nutritional support, etc. the patient recovered well. The pathological exam after the operation showed it was a hemangioendothelioma of the liver with low potential malignancy (Figs. 1,2). Immunohistochemical chromoscopy showed F8 (Factor VIII) and the actin appeared as a pulpy brown positive staining (Figs.3,4).
The grass specimen for the low-potential malignant hemangioendothelioma of the liver, yellow white color, ellipsoid-like, with the size of about 8 cm× 9cm× 10 cm and a distinct limit of the peripheral hepatic tissues.
The low-potential malignant hemangioendothelioma of the liver. Under the microscope the shape of the tumor is spindle-like or asterism-like, the core is big, with anochromasia, nucleolus is small, with acidophilous cytoplasm and indistinct boundary of the cells, and an angioid fissure is formed (H&E× 100).
Result of actin immunohistochemical staining: most of the tumor cells showed a pulpy brown positive staining (× 200).
Result of F8 immunohistochemistry staining: part of the tumor cells showed a pulpy brown positive staining (× 200).
Discussion
Malignant hemangioendothelioma of liver is rare, with other names such as hepatic angiosarcoma, vascular endotheliocyte sarcoma and Kupffer’s cell sarcoma. Hepatic angiosarcomas account for 2% of the total primary malignant tumors of the liver, and about 10 to 20 cases of the diseases have been diagnosed in the U.S. every year, with a incidence rate of 0.14~0.25/106. A study of hepatic biopsies from Chicago showed that there was 1 case of hepatic angiosarcoma in every 30 samples of malignant tumors.[1] The disease is frequently found in adults, with a sex ratio of 3:1 (male/ female). Most causes of the disease are unclear, but some relate to carcinogens such as thorium dioxide, vinyl chloride and arsenic compounds, etc. There is no obvious difference between the clinical manifestations and the type of hepatic cellular cancer, and the diagnosis mainly relies on the pathological examination. Most of the tumors occurr multicentrically, which show a hemorrhagic nodus with indistinct limit and various sizes, and sometimes a spongelike nodus. Microscopic examinations show the tumors are composed of malignant spindle endothelial cells, the cores are big, with anochromasia, a small nucleolus, with aci-dophilous cytoplasm and an indistinct boundary of cells. F8 fos-related antigen is found in tumor cells using immunohistochemistry.[2] The tumor lacks distinctive structure under the light microscope, and should be diagnosed in combination with immunohistochemical staining, to improve accuracy. It is mainly used in the differentiation of the following primary hepatic cancers, i.e. fibrosarcoma, malignant fibrohistiocytoma, hepatic spindle cell carcinoma and solitary fibro-mesothelioma. The main clinical situation includes pyrexia, hypodynamia and hepatomegaly, and there may be abdominal dropsy, jaundice and hepatic disfunction. Malignant hemangioendothelioma of the liver is not sensitive to radiotherapy or chemotherapy. Excision is currently one of the effective treatment methods.
- Received July 2, 2005.
- Accepted September 27, 2005.
- Copyright © 2005 by Tianjin Medical University Cancer Institute & Hospital and Springer
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