Compuferized Medical Imaging and Graphics, Vol. 16, No. 4. pp. X37-290, Printed in the U.S.A. All rights reserved.

1992 Copyright

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EPITHELfOID HEMANGIOENDOTHELIOMA OF SPLEEN WITH INTRASPLENIC METASTASIS: ULTRASOUND AND COMPUTED-TOMOGRAPHY APPEARANCE Chui-Mei Tiu*, Y&Hong Chou, Huay-Tsang Wang?, and Tsuen Chang Departments

of Radiology

and %urgery, Veterans General Hospital-Taipei, and National College, Taipei, Taiwan, Republic of China

Yang-Ming

Medical

(Received 10 September 1991: Revised 16 January 1992)

Abstract-The US and CT appearances of epitheloid hemangioendothelioma of the spleen with intrasplenic metastasis have not been previously reported. We described a 29-yr-old female with such a disease. Abdominal US study revealed a large mass in the upper pole and multiple small nodules in the rest of the spleen. CT scan also showed similar lesions of hypodensity which were not apparently enhanced by contrast medium. Key Words: Epitheloid hemangioendothelioma,

Spleen, Metastasis, Ultrasound, Computed tomography

injection, the hypodense areas were not apparently enhanced while the other parts were well enhanced (Fig. 2). Both the US and CT appearances were compatible with splenic tumors associated with necrosis. Exploratory laparotomy was therefore performed. At surgery, an enlarged spleen, measuring 23 X 15 X 12 cm was identified. On section, a dark brown and yellowish tumor with areas of hemorrhage and necrosis was present in superior aspect with some small nodules in the other parts (Fig. 3). Multiple tiny lesions were also noted (< 1 cm) in both lobes of liver that were not identified in US and CT scan. The histologic diagnosis of the splenic tumors was epitheloid hemangioendothelioma and biopsy of the hepatic nodules also showed the same histologic appearance. Secondary hepatic tumors were considered. The patient had no history of exposure to thorium dioxide, vinyl chloride, or arsenic.

Primary malignant vascular tumors of the spleen are very rare ( 1,2). Histological classification of this entity in the literature includes hemangiosarcoma, sarcomatous angioma, angiosarcoma, hemangioendothelial sarcoma, and hemangioendothelioma (1, 3). Articles describing their appearances in imaging studies are few but include angiography, computed tomography (CT), and ultrasonography (US) (4-6). However, US and CT appearances of epitheloid hemangioendothelioma with intrasplenic metastasis have not been reported before. We herewith describe such a case. CASE REPORT

A 29-yr-old woman presented with a l-month history of left shoulder and left abdominal pain. Physical examination revealed. an enlarged spleen with the lower tip about 4 cm below the left mid-Costa1 margin. Laboratory data were within normal limits except mild anemia (hemoglobin 11.5 gm%, hematocrit 35.4%). US examination was performed using a sector real-time scanner (Diasonics DRF4OOR, 3.5 MHz). The scanner has a lateral resolution of 2.0 mm and an axial resolution of 0.4 mm. US revealed an enlarged spleen with heterogeneous echogenicity. A large focal mass was located in the upper pole and multiple small hypoechoic nodular areas were evident in the rest of the spleen (Fig. 1). CT scan also showed an enlarged spleen with multiple low-attenuation areas inside. After i.v. contrast

DISCUSSION Primary angiosarcoma of the spleen has been reported in 55 cases by Chen et al (3). There were 32 male and 23 female patients in this review. Most of the patients were adults (52/55), with the age ranging from 20 to 84 years (mean: 5 1 yr). The most significant clinical manifestation was hepatomegaly, splenomegaly, or both, presented in 55% of the cases. Anemia has been reported in a majority of patients (70%) and is also present in our patient. The association of hematologic abnormalities with hemangioma has been noted for 50 years (7). Hepatic and relatively rarely splenic hemangioendothelioma have also been mentioned to be associated with microangiopathic hemolytic anemia (8, 9). Thrombocytopenia, red blood cell

* Correspondence should be addressed to Chui-Mei Tiu, M.D., Department of Radiology, Veterans General Hospital-Taipei, Taipei, Taiwan, Republic of China. 287

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hemangioma of the liver ( 14). Our case showed a totally different CT pattern which may represent necrosis and hemorrhage of the main tumor. The US appearance of our patient is also nonspecific, and is different from that reported by Nahman and Cunningham (6). The large heterogeneous hyperechoic lesion and small hypoechoic nodules in the spleen is highly suggestive of a splenic malignant tumor with small intrasplenic matastases. These findings, although not pathognomonic, should remind us to put primary malignant splenic vascular tumor into the list of differential diagnosis, especially in the presence of hepatic metastatic foci. Sometimes it is difficult to establish the site of the primary neoplasm if both liver and spleen are involved by the same pathology. However, by the knowledge of the large solitary splenic tumor, the tiny splenic tumors, and the undetectable hepatic nodules, it is of little doubt

(a)

(a)

Fig. 1. Sonograms of the spleen. (a) The enlarged spleen contains a large heterogeneous echogenic mass in the upper pole (arrow heads). (b) Multiple hypoechoic nodules are diffusely scattered in the other parts of the spleen.

fragmentation, erythrophagocytosis, and hepatic siderosis were frequently observed in hepatic hemangioendothelioma and occasionally in splenic hemangioendothelioma (S-lo). The blood picture of our patient, unfortunately, was not thoroughly studied before surgery to confirm the association with her splenic tumor. Imaging studies of primary splenic malignant vascular tumor are usually nonspecific. Angiography, which is relatively specific, may show multiple vascular lakes in the arterial through the venous phase, mimicking a benign cavernous hemangioma (11). However, in many cases, only pathological vessels suggesting the presence of a hypervascular tumor can be revealed ( 12). Several authors have suggested that i.v. contrast-enhanced CT scan may be diagnostic in the hepatic angiosarcoma

(4, 13). The CT appearance

in these case

reports closely resemble those described in cavernous

Fig. 2. CT scan after i.v. contrast injection. (a) A large heterogeneous hypodense mass (M) is noted in the higher level scan. (b) In addition to the large hypodense mass (M), hypodense nodules with minima1 rim enhancement (small ar-

rows) are shown in the lower-level scan.

Epitheloid hemangioendothelioma

Fig. 3. The cut surface of the representative

C.-M. Tru ef al.

??

289

specimen showed a large tumor in the cephalad portion with numerous small nodules in the other parts.

to the diagnosis of prima.ry splenic hemangioendothelioma in the present case. In addition, primary hepatic hemangiosarcoma is frequently associated with exposure to substances, such as thorium dioxide, vinyl chloride, and arsenic (52/55, or 95%) (3,15), which was not noticed in our patient. SUMMARY We described a 29-yr-old female with splenic epitheloid hemangioendothelioma. Her chief complaint was pain in the left upper abdomen and left shoulder. Splenomegaly was suspected in physical examination and only minimal anemia was noted in the laboratory examination. Ultrasound study revealed an enlarged spleen with heterogeneous echogenicity. A large mass in the upper pole and multiple small nodules in the rest of the spleen were evident. CT scan also showed similar lesions of hypode:nsity which were not apparently enhanced by contrast medium. The liver was not remarkable by both imaging methods. Exploratory laparotomy revealed a large necrotic splenic tumor, associated with small sple:nic nodules and tiny hepatic metastatic lesions. The histological study of the representative specimens showed an appearance of epitheloid hemangioendothelioma of the upper pole of the spleen with multiple metastatic foci in the spleen and liver. Acknowledgments-We paring the manuscript.

of spleen

thank Miss Cheng-Yi Chang for help in pre-

2. Wright, M. Hemangiosarcoma of the spleen. Arch. Pathol. 47: 180-190: 1949. 3. Chen, K.T.K.; Craig-Belles, J.; Gilbert, E.F. Angiosarcoma of the spleen. A report of two cases and review of the literature. Arch. Pathol. Lab. Med. 103:122-124; 1979. 4. Mahony, B.; Jeffrey, B.; Federle, M.P.. Spontaneous rupture of hepatic and spleen angiosarcoma demonstrated by CT. AJR 138: 965-966; 1982. 5. Arbona, G.L.; Leoyd, T.V.; Lucas, J.; et al. Computed tomographic demonstration of angiosarcoma of the spleen. South Africa Med. J. 75:348-350; 1982. 6. Nahman, B.; Cunningham, J. Sonography of splenic angiosarcoma. J. Clin. Ultrasound 13:354-356; 1985. 7. Kasabach, H.H.; Merritt, K.K. Capillary hemangiomas with extensive purpura: Report of a case. Am. J. Dis. Child 59:10631065; 1940. 8. Wright, M. Hemangiosarcoma of the spleen. Arch. Pathol. 47: 180-183: 1949. 9. Alpert, L.I.; Benisch, B. Hemangioendothelioma of the liver associated with microangiopathic hemolytic anemia. Am. J. Med. 48:624-628; 1970. 10. Donald, D.; Dawson, D. Microangiopathic hemolytic anemia associated with hemangioendothelioma. J. Clin. Pathol. 24:456459; 1971. 11. Kishikawa, T.; Numaguchi, Y.; Tokunaga, M.; et al. Hemangiosarcoma of the spleen with liver metastases: Angiographic manifestations. Radioloav 123:3l-35: 1977. 12. Edsman, G. Malignant tumor of the spleen diagnosed by lienal arteriography. Acta Radio]. 42:46 l-464; 1954. 13. Lucaya, J.; Enriquez, G.: Amat, L.; Gonzalez-Rivero, M.A. Computed tomography of infantile hepatic hemangioendotheIioma. AJR 144:821-826; 1985. 14. Itai, Y.; Furui, S.; Araki, T.; Yashiro, N.; Tasaka, A. Computed tomography of cavernous hemangioma of the liver. Radiology 137:149-155; 1980. 15. Ishak, K.G. Mesenchymal tumors of the liver. In: Okuda, K.; Peters, R.L., eds. Hepatocellular carcinoma. New York: John Wiley & Sons; 1976:247-307.

REFERENCES 1. Moriwaki, S.; Mik, H.; Shinohara. T.; et al. Hemangioendothelioma of the liver and spleen. Report of an autopsy case and review of literature. Jpn. J. Cancer Clin. 20:561-571; 1974.

About the Author-CHUJ-ME1 TIU, M.D., graduated from Medical College, National Taiwan University, Taiwan, in 1976. She then completed a radiology residency at the Veterans General Hospital-

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Taipei, 1980. Presently, Dr. Tiu is chief of the Division of Pediatric Radiology at the Veterans General Hospital-Taipei and Associate Professor of Radiology at National Yang-Ming Medical College, Taipei.

About the Author-HUAY-TSANG WANG, M.D., graduated from National Yang-Ming Medical College, Taipei, in 1982. He completed his surgical residency at the Veterans General Hospital-Taipei, where he is now attending. He is also an instructor of Surgery at National Yang-Ming Medical College, Taipei.

About the Author-Yl-HONG CHOU, M.D., graduated from Taipei Medical College in 1978. He completed his residency in Veterans General Hospital-Taipei, in 1981, and is now chief of the Division of Ultrasound at the Veterans General Hospital-Taipei and is Associate Professor of Radiology at National Yang-Ming Medical College, Taipei.

About the Author-TsuEN

CHANG, M.D., graduated from National Defense Medical College, Taipei, in 1952. He then completed a radiology residency at Army General Hospital, 1958. Currently, Dr. Chang is Chairman of the Department of Radiology, Veterans General Hospital-Taipei, and Professor of Radiology, National Yang-Ming Medical College, Taipei.

Epithelioid hemangioendothelioma of spleen with intrasplenic metastasis: ultrasound and computed-tomography appearance.

The US and CT appearances of epithelioid hemangioendothelioma of the spleen with intrasplenic metastasis have not been previously reported. We describ...
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