1991, The British Journal of Radiology, 64, 1067-1069 Case reports MOHENSIFAR, Z., TASHKIN, D. P., CARSON, S. A. & BELLAMY,

P. E., 1982. Pulmonary function in patients with relapsing polychondritis. Chest, 81, 711-717. NEILLY, J. B.,

WISTER,

J. H.

&

STEVENSON,

R. D.,

SANE,

D. C ,

VIDAILIST,

H. H.

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BURTON,

C. S.,

1987.

Cutaneous signs of cardiopulmonary disease. Saddle nose, red ears and fatal airway collapse. Chest, 91, 268-270.

1985.

STEWART, S. S., ASHIZAWA, T., DUDLEY, A. W., GOLDBERG,

Progressive tracheobronchial polychondritis: need for early diagnosis. Thorax, 40, 78-79.

J. W. & LIDSKY, M. D., 1988. Cerebral vasculitis in relapsing polychondritis. Neurology, 38, 150-152.

Thorium dioxide, forgotten but not gone By D. Kessel, K. F. Robbins, * M . L Wilkinson and t P . M. Smith Lewisham General Hospital, Lewisham, London SE13 6LH, *Guy's Hospital, St Thomas' Street, London SE1 9RT and tLlandough Hospital, Penarth, South Glamorgan CF6 1XX, UK

(Received March 1991) Keywords: Thorotrast, Liver disease, Radiology

The recent report edited by Taylor et al (1989) reminds us of the continuing risk of morbidity in patients exposed to thorium. We present three cases, with hepatic sequelae, in which the radiological diagnosis was missed on initial presentation. They illustrate the need to be alert to the possibility of Thorotrast toxicity. Thorotrast, a 25% colloidal solution of thorium dioxide, was a widely used contrast medium from 1928 (Bluhbaum et al, 1928) until 1955, when its use was abandoned following a report (MacMahon et al, 1947) of hepatic angiosarcoma associated with exposure to Thorotrast and concern about the long term effects of internal irradiation. Case reports Case 1 A 69-year-old female was admitted in 1989 for investigation of malaise, right hypochondrial pain and anorexia. She had had a cholecystectomy 10 years previously at which abnormal liver texture was noted. A liver biopsy revealed cirrhosis. Examination was normal apart from tender, non-pulsatile hepatomegaly. Liver function revealed a markedly elevated alkaline phosphatase and gamma glutamyl transferase. The alpha-fetoprotein (AFP) was not raised. An abdominal X ray (Fig. 1) revealed metallic opacities in the spleen, periphery of the liver and coeliac axis lymph nodes. Abdominal ultrasound showed the liver to be enlarged with increased echogenicity. The spleen was noted to be very small. Further history revealed that the patient had received Thorotrast in 1938 during investigation of a "kidney problem". A repeat liver biopsy showed multiple deposits of refractile brown material consistent with Thorotrast. There was macronodular cirrhosis with marked periductal fibrosis. No malignancy was seen. Review of the previous biopsy also showed Thorotrast particles and cirrhosis. She was treated with predni-

Address correspondence to Dr D. Kessel, Department of Radiology, The Middlesex Hospital, Mortimer Street, London WIN 8AA, UK. Vol. 64, No. 767

solone on which her general condition subjectively improved with a corresponding improvement in her liver function tests. Case 2 A 62-year-old man presented as an outpatient in 1979 with a 4 month history of continuous "gnawing" epigastric pain associated with mild anorexia and 3.5 kg weight loss over the preceding 2 months. On examination he had an enlarged liver, visible as an epigastric mass. In 1934 he underwent repair of a right femoral artery aneurysm following a gunshot wound. An angiogram was performed as part of the investigation. An abdominal radiograph revealed a typical pattern of Thorotrast deposition. An isotope liver scan demonstrated a large area of decreased uptake in the right lobe of the liver. A liver biopsy revealed cholangiocarcinoma, marked fibrosis and deposits of Thorotrast. Whole body gamma spectroscopy demonstrated the typical emission pattern of thorium. Despite chemotherapy his condition steadily deteriorated and he died 3 months later. Case 3 A 69-year-old man was referred from his general practitioner, in 1989, with "flu-like" symptoms and a palpable liver.

Figure 1. Abdominal radiograph showing the typical distribution of Thorotrast in the liver capsule (large straight arrows), spleen (note punctate distribution) (curved arrow) and parapancreatic lymph nodes (small straight arrows). 1067

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Case reports

Figure 2. Abdominal CT scan showing a large area of low attenuation in the right lobe of the liver (large straight arrow). Metallic deposits of Thorotrast are seen in the spleen (curved arrow) and peripancreatic lymph nodes (small arrows).

A chest radiograph revealed Thorotrast deposition in the liver and spleen. In 1935 he had undergone carotid angiography during the investigation of migraine. Biochemical and haematological investigations were normal but the AFP was elevated massively. Ultrasound revealed a 10 cm echogenic mass in the right lobe of the liver and no other abnormality. Computed tomography (CT) (Fig. 2) revealed a large low attenuation lesion in the right lobe of the liver. Thorotrast deposition was noted in the periphery of the right lobe and in the coeliac axis lymph nodes. The spleen was shrunken and full of Thorotrast. He was offered partial hepatectomy for his presumed hepatocellular carcinoma but declined this and has remained well apart from some upper abdominal pain. Discussion

Thorium is radioactive and, owing to retention in the reticuloendothelial system, has a biological half-life of 200-400 years (Ito et al, 1988). 90% of the emitted radiation is high linear energy transfer (LET) a-particles (Kojiro et al, 1982). The typical dose rate to the liver and spleen is 0.3 Gy/year (Looney, 1960). Thorotrast is inhomogeneously distributed in the body, 60% is in the liver, 19% in the spleen. The remainder is found mainly in bone marrow (21%) and the para-pancreatic lymph nodes (Ishikawa et al, 1989). This results in the characteristic X ray appearance with diffusely scattered opacities in the liver, spleen and porta hepatis (Gondos, 1973). The abdominal X ray is almost pathognomonic (Velasquez et al, 1985). Where doubt exists the diagnosis is confirmed by total body gamma radiation counting (as in our second case) or by autoradiographic studies of a biopsy specimen. The formation of aggregates of Thorotrast in the liver in the subcapsular and peri-portal regions results in wide ranges in the local radiation dose which has been estimated to vary from 0-4 /iGy to 20 Gy/year (Kaul et 1068

al, 1979; Dalheimer & Kaul, 1989). The amount of thorium deposited in the liver can be estimated from the Hounsfield number on abdominal CT scanning (Miyajima et al, 1985). The presence ot Thorotrast induces local fibrosis and hepatocyte necrosis which develops into cirrhosis (Scully et al, 1981). The carcinogenicity is due to local radiation damage (Spiethoff et al, 1989). Exposure to Thorotrast is associated with decreased life expectancy. Several large studies (Janower et al, 1972; Da Silva Horta et al, 1974; Cayolla da Motta, 1979; Mays, 1979) have shown that there is excess mortality due to liver cirrhosis and malignant disease particularly hepatic cancers (occurring in up to 10% of cases of prolonged exposure) and leukaemias. Cholangiocarcinoma and hepatic angiosarcoma account for 40% each, with the remainder being hepatocellular carcinoma (16%) and unspecified or dual cell type (Kojiro & Ito, 1989). The latent period for the development of hepatic fibrosis is over 15 years and for liver tumours greater than 25 years (Da Silva Horta et al, 1965). Interestingly, the incidence of hepatocellular cancer and hepatic angiosarcoma is increasing whilst cholangiocarcinoma appears to be on the decline (Falk, et al, 1979; Mori et al, 1989). Thorotrast will continue to cause disease until the end of this century and beyond; however, the incidence of Thorotrast related disease will fall as the number of survivors diminishes. Thorotrast should be considered as a possible cause for liver disease in any patient with a history of angiography up to the mid 1950s. It is important for clinicians, radiologists and pathologists to remain alert to the diagnosis and to be aware of the long term complications of Thorotrast exposure. Any patient known to have been exposed to Thorotrast should be followed-up for life as only early detection of hepatic malignancy will allow effective treatment. All cases should be documented fully in order to increase our knowledge of the consequences of long term exposure to an internal radiation source. Acknowledgment The authors would like to thank Dr C. N. Mallinson for allowing us to report his patient. References BLUHBAUM, T., FRIK, K. & KALKBRENNER, H., 1928. Eine Neue

Anwendungsart der Kolloide in der Roentgendiagnostik. Fortschritte auf dem Gebiete der Rontgenstrahlen, 37, 18-29. CAYOLLA DA MOTTA, L., 1979. Epidemiological study of

Thorotrast-exposed patients in Portugal. Environmental Research, 18, 152-172. DALHEIMER, A. R. & KAUL, A., 1989. Calculation of local dose to tissues adjacent to Thorotrast conglomerates. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 108-111. DA SILVA HORTA, J., ABBAT, J. D., CAYOLLA DA MOTTA, L. &

RORIZ, M. L., 1965. Malignancy and other late effects following administration of Thorotrast. Lancet, 2, 201-205. DA

SILVA HORTA, J., CAYOLLA DA MOTTA, L. & TAVARES,

M. H.,

1974. Thorium

dioxide

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in man:

The British Journal of Radiology, November 1991

1991, The British Journal of Radiology, 64, 1069-1072 Case reports epidemiological, clinical and pathological studies (experience in Portugal). Environmental Research, 8, 131-159. FALK, H., TELLES, N. C ,

ISHAK, K. G., THOMAS, L. B. &

POPPER, H., 1979. Epidemiology of Thorotrast-induced hepatic angiosarcoma in the United States. Environmental Research, 18, 65-73. GONDOS, B., 1973. Late clinical and roentgen observations following Thorotrast administration. Clinical Radiology, 24, 195-203. ISHIKAWA, Y., KATO, Y. & HATEKEYAMA, S., 1989. Late effects

of a-particles on Thorotrast patients in Japan. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 129-131. ITO,

Y., KOJIRO, M., NAKASHIMA, T. & MORI, T., 1988.

Pathomorphological characteristics of 102 cases of Thorotrast related hepatocellular carcinoma, cholangiocarcinoma and hepatic angiosarcoma. Cancer, 62, 1153-1162. JANOWER, M. L., MIETTINEN, O. S., & FLYNN, M. J., 1972.

Effects of long-term Thorotrast exposure. Radiology, 103, 13-20.

LOONEY, W. B., 1960. Investigation of late clinical findings following Thorotrast (thorium dioxide) administration. American Journal of Roentgenology, 83, 163-185. MACMAHON, H. E., MURPHY, A. S. & BATES, M. I., 1947.

Endothelial cell sarcoma of the liver following Thorotrast injections. American Journal of Pathology, 23, 585-611. MAYS, C. W., 1979. Liver cancer risk. In International Atomic Energy Agency, Comp. Biological Implications of Radionuclides Released from Nuclear Industries. Vol. 2. (International Atomic Energy Agency, Vienna), pp. 146-165. MIYAJIMA, J., OKAJIMA, S., TAKAO, H., NAKASHIMA, A. &

HOMBO, Z., 1985. Estimation of thorium deposited in Thorotrast patients by CT scanner in comparison with whole body counter. Journal of Radiation Research, 26, 196-210. MORI, T., KUMATORI, T., HATAKEYAMA, S., IRIE, H., MORI, W., FUKUTOMI, K., BABA, K., MARUYAMA, T., UEDA, A., IWATA,

S., TAMAI, T. & AKITA, Y., 1989. Current (1986) status of the Japanese follow-up study of the Thorotrast patients, and its relationships to the statistical analysis of the autopsy series. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 119-124.

KAUL, A., FOLL, U., HASSE, V. A., PALME, G., RIEDEL, W. &

SCULLY, R. E., GALDABINI, J.J. & MCNEELY, B. U., 1981. Case

STOLPMANN, H.-J., 1979. Microdistribution of Thorotrast and dose to cellular structures. Environmental Research, 18, 13-22.

reports of the Massachusetts General Hospital. Case 151981. New England Journal of Medicine, 304, 893-899.

KOJIRO, M., KAWANO, Y., KAWASAKI, H., NAKASHIMURA, T. &

IKEZAKI, H., 1982. Thorotrast-induced hepatic angiosarcoma and combined hepatocellular and cholangiocarcinoma in a single patient. Cancer, 49, 2161-2164. KOJIRO, M. & ITO, Y., 1989. Pathomorphologic study of 106 autopsy cases of Thorotrast-related hepatic malignancies with comparison to non-Thorotrast-related cases. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 125-128.

SPIETHOFF, A., WESCH, H., WEGENER, K. & HOVER, K.-H.,

1989. Tumour induction in rat liver by fractionated irradiation with neutrons and a foreign body burden (Zirconotrast) in comparison to Thorotrast induced tumours. In BIR Report 21, Risks from Radium and Thorotrast, Ed by D. M. Taylor (BIR, London) 149-152. TAYLOR, D. M., MAYS, C. W., GERBER, G. B. & THOMAS,

R. G. (Eds) 1989. BIR Report 21, Risks from Radium and Thorotrast (British Institute of Radiology, London). VELASQUEZ, G., WARD, C. F. & BOHRER, S. P., 1985. Thorium

dioxide: still around. Southern Medical Journal, 78, 743-745.

Radiological manifestation of elastofibroma: a case report and review of the literature By Takashi Nakano, MD, Zenta Tsutsumi, MD, Toshikazu Hada, MD and Kazuya Higashino, MD The Third Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan {Received December 1990 and in revised form March 1991) Keywords: Elastofibroma, CT, Rib fracture, Subscapular, Chest wall tumour

Elastofibroma is a very rare benign tumour of connective tissue, whose features are distinguished by the presence of abundant elastinophilic polymorphic structures and characteristic location. The lesion usually appears in elderly women (50-80 years) characteristically arising from connective tissue in the subscapular region. There

are few reports of elastofibroma in the radiological literature (Berthoty et al, 1986; Marin et al, 1987). We report a case of elastofibroma dorsi presenting with chest radiographic abnormalities and its radiological manifestation is reviewed. Case report

Correspondence should be addressed to Takashi Nakano, The Third Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663 Japan.

Vol. 64, No. 767

An 84-year-old Japanese female with no significant medical history was referred to hospital for evaluation of an abnormal chest X ray and a feeling of fullness in the upper abdomen. 6 months earlier she began to experience an occasional feeling of fullness in the upper abdomen. Initial evaluation revealed a

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Thorium dioxide, forgotten but not gone.

1991, The British Journal of Radiology, 64, 1067-1069 Case reports MOHENSIFAR, Z., TASHKIN, D. P., CARSON, S. A. & BELLAMY, P. E., 1982. Pulmonary fu...
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