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"to Epidemiology of mgiosarcoma of Liver In lew York State CHOLAS J. VIANNA, M.D., M.S.P.H. Albany, New York IDITH A. BRADY, B.S. Albany, New York INEMaRIE tobia cardamone, B.A. Albany, New York m the New York State Department of Health, Bureau of ironmenta! Epidemiology and Occupational Health giosarcoma of the liver (ASL) is a highly maiant tumor1 which can be caused by exposure to enic in pesticides2-3 and Fowler's solution (posium arsenite),4- thorium dioxide (ThC>2--a ditost^^adiopaque medium also called Thoro*t),^id vinyl chloride monomer (VCM), a coliss gas which can be polymerized to make polyyl chloride (PVC) in the production of plastics.9-15 s disorder has also been associated with inorganic per16 (vineyard sprayers), diethylstilbestrol,17 and nochromatosis.18-19 Recently it has been sugted that more subtle types of exposure such as ;e residential proximity to VCM/PVC industrial iplexes20 might be important. This report exnes the geographical distribution of ASL in New k State with particular emphasis on occupation other epidemiologic characteristics which might f importance in the etiology of this disorder. 'hods icidence study. This study included all resi-. ts of New York State, excluding New York City, .nosed with histologically confirmed ASL from ) through 1978, who were reported to the Tumor istry. Cancer Control Bureau, New York State artment of Health. The New York State Tumor istry is the largest population-based registry in world. State law requires that all diagnoses of gnancy be reported by physicians and/or hosl institutions. This nine-year study period was ted because during this time the registry esshet^ynechanism to improve reporting of cases Jgh^wxlic examinations of the percent of cases isior^Tinto the State Cancer Registry by death certificates only and by an ongoing survey of hospital records. Patients from New York City were ex cluded because cancer reporting from this area was not instituted until 1973, and at present findings are incomplete. An average annual incidence rate was calculated for the nine-year period using the 1974 population estimate (Office of Biostatistics, New York State Department of Health). Age-specific incidence rates (calculated by 10-year age groups) and an overall sex ratio (male rate to female rate) were also determined using the above population estimates. The 57 counties of New York State, ex cluding New York City, were divided into the seven standard health service areas (HSA), and a regional rate was calculated for each area. Descriptive study. All residents of New York State diagnosed with histologically confirmed ASL from 1958 through 1979 and reported to the New York State Tumor Registry (including those reported from New York City from 1973 through 1979) were included in this investigation. Trained field inves tigators used a prepared questionnaire to obtain in formation from all cases (or in the event of death, their closest living relative) regarding past and present occupation, medical, familial, travel, and social histories. Particular attention was given to histories of exposure to known or suspected causative agents. In addition, specific questions were asked about residential histories in an effort to determine whether patients ever lived in close proximity (one mile or less) to industrial complexes, farms, or known dumpsites prior to being diagnosed with ASL.21 Using information obtained from the New York State Departments of Commerce and Environmental Conservation22 to identify known VCM and/or PVC factories and dumpsites, we plotted all places of residence of patients with ASL prior to their diag nostic dates. The urban-rural distribution of all patients at the time of diagnosis was also determined. (Urban counties are counted as those with a popu lation of 100,000 or greater.) Medical records of all patients were reviewed to confirm 1) the diagnosis of ASL; 2) past medical histories (diagnostic procedures involving TI1O2 and drug histories); 3) place of resi dence, and 4) occupation at the time of diagnosis. Results Twenty-five cases (14 males, 11 females) of ASL were included in the incidence study. The average annual incidence rate for New York State, excluding New York City, was 0.26 per million. Annual rates ranged from 0.0 to 0.46 per million population per year during the nine-year study period, and no tem poral peaks were evident. Age at diagnosis ranged from one month to 83 years, with an overall mean age of 50.5 years (males 54.1 years, females 49.6 years). Analysis of age-specific incidence rates indicated that the highest rate, 1.08 per million population, occurred in the 60-through-69-year age group. The overall male to female ratio was 1.3 to 1. HSA I had the highest average annual incidence rate of 0.40 per May 1981/New York State Journal of Medicine 895 1 I I 30 1; co 1. million population, followed by area VIII with a rate of 0.33 per million. Area V had a comparable rate to that for the state and the other four HSAs had lower rates (Fig. 1). A total of 43 patients (male 24, female 19) diag nosed with ASL in New York State (Table I) and reported to the Registry from 1958 through 1979 were included in the descriptive study. Forty-two pa tients were deceased at the time of the investigation. A first degree relative (spouse, sibling, parent, or child) was interviewed regarding 37 of the 43 (86 percent) patients. Information on three patients was obtained from other close relatives and no interview was obtained for three cases (including two infants). Their ages ranged from one month to 83 years with an overall mean age of 52.7 years (males 65.5 years, females 49.1 years). There were 39 white patients, 3 blacks, and 1 Oriental. Four male patients (num bers 4,8,10, and 39; Table I) from Niagara and Erie counties worked in the same VC polymerization plant for a period ranging from 16 to 35 years (median 23.5 years) prior to being diagnosed with ASL. In addi The geographic distribution of ASL (angiosarcoma of the liver) among residents of New York was ex amined with special emphasis on the epidemiologic characteristics which might be of etiologic importance in this malignancy. The average annual incidence rate for the state, excluding New York City, was 0.26 per million population. Of the chemicals associated with ASL, potential exposure to vinyl chloride ap peared to be the most important factor in certain urban industrialized counties, whereas probable ar senic exposure was the major factor in rural areas. Prior thorium dioxide injections accounted for only 7 percent of the 43 patients studied. We suggest that certain chemicals structurally similar to vinyl chlo ride might be of etiologic importance among patients who have no documented exposure to any of the es tablished causes of ASL. tion, five patients, all females (numbers 5,11,17,21, and 25; Table I), resided within one mile of plastic- FIGURE 1. Average annual Incidence rates (per 100,000 population) in the seven health service areas of New York State, excluding New York City, from 1970 through 1978. 896 New York State Journal ol Medlclne/May 1981 demographic data and exposure history of 43 residents of New York State with angiosarcoma of liver (1958 through 1979) It Age at nr Diagnosis % 32 49 f 67 52 54 60 60 45 45 18 68 55 2 mos. 31 77 64 47 62 47 63 59 31 45 18 62 65 71 73 57 no. 3 7 5 7 Race* W W w w w w w w w w w w B w w w 0 w w w w B w w w w w w \v B W W w w w w w w w w w w w Year of County of Sex Diagnosis Residence Occupational History Probable Possible Residential Exposure Exposure History M 1958 Greene* gardener-caretaker As** F 1959 Westchester secretary/housewife F 1960 Monroe salesclerk M 1961 Niagara chemical operator VC F 1965 Nassau clerk/liousewife VC lived 4500' from factory F 1967 Erie aircraft worker/ verifax operator M 1968 Albany machinist/appliance ThOi repair M 1968 Niagara chemical operator VC M 1969 Nassau secret service/ attorney M 1970 Niagara chemical operator VC F 1970 Erie housewife , VC lived 4500' from M 1970 Nassau attorney F 1970 Westchester clerk factory M 1971 Nassau school principal/ chemistry teacher F 1971 Fulton* battery filler/glove As, Cu** operator F 1971 Westchester infant F 1972 Nassau bankteller VC lived 1500* from factory M 1972 Erie laborer--aerospace industry & City of Buffalo F 1973 Lewis* sewing machine As lived on farm operator M 1973 Nassau real estate broker Th02 F 1973 Niagara clerk--chemical VC lived 1700' from company/ factory manager--candy store M 1973 Queens bookkeeper- As lived on banana farm M 1973 Nassau carpenter, cleaning and maintenance M 1973 Seneca* minister F 1974 Erie medical secretary/ VC housewife M 1974 Rockland office worker--plastic co./executive F 1974 Brooklyn student F 1974 Greene* owner--clothing store M 1975 Jefferson* report analyst--brake lived 500' from factory CO. M 1975 Queens janitor--subways M 1975 Nassau carpenter/painter F 1975 Manhattan film lab./contract administrator for TV M 1975 Greene* infant M 1976 Wayne* research chemist-- As photo co. M 1976 Brooklyn maintenance/cleaning M 1976 Manhattan executive--soda co. F 1977 Richmond housewife F 1977 Cattaraugus* chromium chair coJ cafeteria worker M 1977 Erie chemical operator VC F 1977 Bronx domestic/housewife F 1978 Suffolk sales--radio shop M 1978 Suffolk radio operator ThOi M 1979 Dutchess dairy & fruit farmer/ As truck driver lived on farm Slack, O Oriental. nuinin; than 100,000 population. W-cop] May 1981/New York State Journal of Medicine 897 ler in the mannfaLture of polyvinyl chloride, J. Occup, Mc-d. 1G: *) (1974>. 12. Lee. F, I- and Harry, D. S.: Angiosarcoma of the liver in \ inyl-ch!ride worker, Lancet 1: 1316 (1974). 13. H.. and Waxweiler. R.: Epidemiological studies of nyl ^^Bde health effects in the United States, Proc. R. Soc. led. bP'3 (1976). 14. Block. J. B.: Angiosarcoma of the liver following vinyl iloride exposure, J.A.M.A. 229: 53 (1974). 15. Heath, C. W., .Jr.. Falk, H., and Creech, J. L., Jr.: Char:teristics of cases of angiosarcoma of the liver among vinyl iloride workers in the United States, Ann. N. V. Acad. Sci. 246: 51 (1975). 16. Pimentel J. C., and Menezes, P.: Liver disease in vineyard irayers. Gastroenterology 72:275 (1977). 17. Hoch-Ligeti, C.: Angiosarcoma of the liver associated with lethylstilbestrol, J.A.M.A. 240: 1510 (1978). 18. Kwittken, J., and Tartow, L. R.: Haemochromatosis and upffer-cell sarcoma with unusual localization of iron, J. Path, act. 92:571 (1966), 19. Sussman, E. B., Nvdick. I., and Gray, G. F.: Hemangmendothelial sarcoma of the liver and hemochromatosis, Arch. Pathol. 97:39(1974). 20. Landrigan, P. J., and Heath, C. W,, Jr., 1976, unpub lished. 21. Department of Environmental Conservation and county departments of Commerce and Industry. 22. Hazardous Waste Disposal Sites in New York State, pre pared by Division of Solid Waste, N'YS Dept, of Environmental Conservation with the cooperation of the NYS Dept, of Health, DEC Pub. June, I960, vols. 1-3. 23. Nicholson, W. J.: Cancer following occupational exposure to asbestos and vinyl chloride. Cancer (suppl. 4) 39: 1792 (1977). 24. Falk, H., et al: Epidemiology of Thorotrast-induced hepatic angiosarcoma in the United States, Environ. Res. 18:65 (Feb.) 1979. 25. Falk, H., Thomas, L. B., Popper, H., and Ishak, K. G.: Hepatic angiosarcoma associated with androgenic-anabolic ste roids, lancet II: 1120 (24 Nov.) 1979. jrvival rate improving r systemic lupus erythematosus Medical science is learning more about a particularly jbborn and uncomfortable disease--systemic lupus erhematosus--and the survival rate of sufferers has imoved markedly in recent years. SLE (systemic lupus erythematosus) is a chronic, inmmatory disease of unknown cause affecting skin, joints, ineys, nervous system, and often other organs of the dy. The most obvious sign is a persistent skin rash with lassie butterfly pattern. The disease strikes about one person in each 1,000 and five to 10 times more common among females than lies. In a report in the March 6 Journal of the American -dical Association, a California research group relate perience in treating 609 patients with SLE, with a folv-up period of up to ten years. The overall ten-year survival was 79 percent, said Edmd I^Dubois, M.D., of the University of California at s An^A School of Medicine, Male patients did worse m fe^Hfe patients. There was no basic difference in "vival of blacks and whites. One of the most serious results of SLE is kidney damage, and this was a factor in many of the fatal cases. Dr. Dubois found. The survival rate of 79 percent was much improved over earlier results, and the California group credits improve ment to closer follow-up, better nutritional status, and treatment begun at earlier stages of the disease. "Our understanding of SLE has improved substantially over the last 30 years. While SLE initially was reported to have a 90 percent two-year mortality, more recent re ports have shown much longer survival. This article rep resents the most cohesive group published to date. It represents the largest number ever reported by a private physician, with a follow-up report twice that of any other study," he said. The disease progresses slowly. There is an average time lapse between first signs of the ailment and obtaining a diagnosis from the physician of around four years. "We believe that the socioeconomic problems of patients who attend the larger city or county hospital clinics affect survival significantly. Less optimal living conditions, poor nutrition, and inferior education lead to less reliable medication administration, missed clinic appointments, greater risk of infection, and presentation with more ad vanced disease," Dr. Dubois said. May 1981/New York State Journal of Medicine 899 R&s 000816