Document ZBxGxEvLZZVR6ro25d433r98Y

Vol. 58, n. 4, 1967 Clinique des maladies professionnelles Cluj, Roumania 261 ETUDE DES MALADIES DUES AU CHLORURE DE VINYLE Dr. 1. SUCIU*. Dr. I. DREJMAN, Dr. M. VALASKAI Le developpement rapide de Findustrie du polychlorure de vinyle dans notre pays souleve de nouveaux problemes pour l'hygiene du travail, pour la toxicologie et la pathologie professionnelles. Afin de les etudier un collectif dTiygienistes et de clinicians de Cluj ont precede a Fexamen des con ditions de travail et des maladies des ouvriers de deux entreprises oil Fon fabriquait du polychlorure de vinyle. Les manifestations cliniques que Fon y a rencontrees posent des problemes d'interpretation etiopathologique d'intcret pratique et theorique pour la connaissance du tableau clinique et des mesures de prophylaxie et de traitement a instituer. L'etude s'averait d'autant plus necessaire que les donnees de la litterature etaient limitees et lacunaires. surtout relativement aux manifestations cliniques de la maladie. C'est ainsi que Danisewski avait demontre que les monomeres qui sont produits au debut du processus de fabrication ont une action agressive du point de vue biologique. Ils sont capables de determiner des modifications destructives dans les teguments et les muqueuses. Certains d'entre eux sont allergisants et ont une action sur les organes parenchymateux oil ils determinent des modifications degeneratives et sur Fappareil hematoformateur etant la source d'anemies de type hemolitique. Hervieux et Tassier rapportent 17 cas de dermatite chimique chez 145 travailleurs occupes dans Findustrie des masses plastiques polyvinyliques. Plesitzer. Bondar et Smirnova, cites par Dani sewski, en etudiant Faction chronique des vapeurs du chlorure de vinyle, mettent en evidence Fapparition, chez Fhomme, d'angionevroses toxiques. On voit done que ces etudes cliniques disparates n'ont pas encore abouti a Findividualisation d'un tableau clinique de la maladie. Quant aux recherches experimentales, Mastromatteo, Fischer, Christie et Danzinger, en etudiant Faction toxique du chlorure de vinyle sur un lot de 5 souris et 5 cobayes, ont demontre, qu'a doses de 10-20-30 %, cette substance a un effet narcotique. Les expositions repetees determinent Fapparition de con gestions hepatiques et de congestions pulmonaires, qui ont ete mises en evidence chez les animaux sacrifies ou decedes au cours de la periode d'exposition; dans certains cas il y a eu aussi une degenerescence graisseuse du foie. En ce qui concerne la toxicite, Lazareev situe le chlorure de vinyle parmi les substances a faible action narcotique. En poursuivant la dynamique de la symptomatologie clinique et de certaines modifications de laboratoire chez les ouvriers exposes au chlorure de vinyle et aux monomeres de celui-ci nous avons constate que les donnees obtenues permettent une individualisation du tableau clinique qui aidera les medecins practiciens a * Maitre de conferences. .TS!@49G6Z O 8 CP O'} & wi / n> 0=S,S-?r) K m o ?v> c' v* ?. o p *n o -M==* =*o a3 S2 ,S i"-S^.= S* '-*2 p o 2S T 52 =* ;; s: 0, ., ^3 = TS"-' S-" =2 g >*' "?=>- 52??-r y .,-a p, v-` -4;' 2.3 s*:?*- o. `TJ' o _j^o 2*< n J; - 22' "E *"3 2 =,5-> < ""2-J S-o " *2o"3o. m''!,2J*-y a? ? 5**a? ^*?- 9 XZO 2 2 --' o . . 0.3 m a - *< o^^coS5 z-53 ^g-o "=2.w" SS' Saw3 IoS..?^2"E.8"wC Sia52:-r-'5?_m85;g2_>_._*masOa_ o s.ps^:o| -- * * % r +>=*,," o 3-= t.>---r ^--w" l/Soo ^ A nrr H x?pa -<5.** "-- "T> . * P- o Oa -2 53 5*o o> 5 3 * -- >5 5 2. ><a. 3ssLn CL 5 *2a ? ia 3?"4- o2 22.2r~ M <t O c. --j * > -- A J-JlA* o r, oO-2-; s 52 co * ca ; S" = Q-i z <*< . *< A p !*- r*w a H*"*"" ^ 5n ao O'-- nT3> "-"'SS-.-S f9frCU<L-fotx3"fg3*S3o 5 -oSsa S' 5i2" o -- -- *. -o sr C-< A A or- A CL * 3 Oo> O . 3 3a.M ?*0|* "-o -5 3*3 CO A a ?r<:O* 5 'v S<^0J03 cnSJ >3 2.3 3 a N =r 7; 3 a a -2r ,,* O.A m: gS-i n-g" O. 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A .z!c"5? ^ 2 -1 i- Or g3 m3 ?_ a A S3 ' --o --" =--< 3. a cl -- X c. ?S a S3 -a a -- " 3 X< 2" c.^ *ao cnr ^ - S' 5P SSr h Oji ft on "a. =r a on s^s 5-p; Is- w* aa A-- 2.S - ^ 3 5t- JLlo e Si: 2 t'- C T030 3 O r? 3o -fg - O 2. t 3S z c --- 5' 2 cr a Qa ft H= O A"'5*2 Ac nP-ia- s-i. *9 p; nA A-- >^03 a r--8>.: 7CT- . - Hemangioendothelial Sarcoma of the Liver and Hemochromatosis Edward B. Sussman, MD; Irwin Nydick, MD; George P. Gray, MD, New York i/ r A patient who had Idiopathic hemochro history included a 37-year-old brother with present but greatly decreased in compari i matosis with cirrhosis, that was treated by multiple phlebotomies, developed heman adult onset diabetes mellitus and obesity who was said to have hemochromatosis, son to the original specimen. The amount of iron removed by phlebotomy was esti gioendothelial sarcoma (Kuptfer cell sar diagnosed after this was established in our mated to be 16 mg; however, serum iron o coma) of the liver with widespread bony patient. was 200pg/100 ml and total iron-binding i metastases. The possible relationship be A physical examination showed blood capacity was 200pg/100 ml. tween hemochromatosis, cirrhosis and pressure, 110/60 mm Hg; fair complexion Over the next three months the patient *55 this rare tumor is discussed. without pigmentation; pretibial edema; lost 5 kg, had a low grade fever, and pain splenomegaly; and testicular atrophy. Per in the left upper quadrant, back, and left tinent laboratory studies disclosed the fol shoulder. In September 1970, he was read lowing values: hemoglobin, 13.6 gm/100 ml; mitted because of multiple pathologic rib An exceptional instance of heman- hematocrit reading, 42.5%; white blood fractures. He had developed scleral icterus, . gioendothelial sarcoma occurred cell count, averaging 3,400 cu mm; platelet lymphadenopathy, and hyperpigmentation >1, in a patient with long-standing idio count, 40,000 cu mm; serum iron, 228pg/10Q of pretibial skin. The serum iron was now od pathic hemochromatosis. Less than ml; total iron-binding capacity, 228pg/100 174pg/100 ml and total iron-binding capac U- 100 cases of this rare tumor have been ml; total serum bilirubin, 1.8 mg/100 ml; ity, 164pg/100 ml. Abnormal liver function reported in adults.114 While the cause serum glutamic oxaloacetic transaminase (SGOT), 52 milli-international units/ml was now indicated by the following values: SGOT, 103 m-IU/ml; SGPT, 25 units; alka of this tumor is unknown, many cases (normal, 10 to 50 m-IU/ml); serum glu line phosphatase, 164 m-IU/ml (normal, 30 have been associated with cirrhosis or tamic pyruvic transaminase (SGPT), 40 units to 85); and total bilirubin 2.7 mg/100 mL A the administration of thorium dioxide (normal, 5 to 40 units); serum alkaline biopsy specimen of a soft tissue mass sur suspension (Thorotrast). phosphatase, 5.3 Bodansky units (normal, rounding a pathologic fracture of the sixth Report of a Case 1.5 to 5.0); fasting blood glucose, 276 rib on the left side of the chest showed mg/100 ml; and +4 glycosuria. There was metastatic anaplastic malignant tumor. A 46-year-old white man (NYH 77-40-15) was admitted to the New York HospitalCornel] Medical Center in July 1968 be cause of diabetes, weakness, impotence, pancytopenia, edema, and hepatosplenomegaly. Past medical history included se no evidence of hemolysis. Electrocardio grams showed nonspecific S-T segment and T wave abnormalities. The red blood cell (RBC) agglutination test for anti thyroid bodies was positive l:2Ji00. Radio active iodine uptake in 24 hours was 23%. The only clinical evidence of a primary site was a large defect in the right lobe of the liver demonstrated by a liver scan, but a definite diagnosis was not established. The patient was treated with medroxypro gesterone acetate (Depo-provera), cyclo vere "typhoidal" infectious mononucleosis in 1957, adult onset diabetes mellitus that was diagnosed in 1964, treated initially with oral hypoglycemic and then insulin, and onset of pancytopenia in 1966. Family Percutaneous liver biopsy specimen showed a marked increase in iron deposi tion, especially in periportal hepatocytes (Fig 1), periportal fibrosis, and glycogen vacuolization of hepatocyte nuclei. The diagnosis of idiopathic hemochromatosis phosphamide (Cytoxan), prednisone, and radiation therapy to the fracture site. Three months later the patient was ad mitted because of severe generalized bone pain. He had tenderness over multiple ribs, neck vein distention and decreased higher complicated by diabetes mellitus and a integrative functions. Laboratory data in Accepted for publication July 30,1973. From the departments of pathology (Drs. Suss- diagnosis of hypothyroidism were made. Biweekly phlebotomies were begun. cluded: serum iron, 122pg/100 ml; total iron-binding capacity, 130pg/100 ml; total man and Gray) and medicine (Dr. Nydick), New York Hospital-Cornell Medical Center, New York. Reprint requests to 525 E 68th St, New York 10021 (Dr. Gray). Two years later, in June 1970, after 63 units of blood (31,500 ml) had been re moved by phlebotomy, a liver biopsy speci men showed that iron stores were still bilirubin, 21 mg/100 ml; direct bilirubin, 0.6 mg/100 ml; alkaline phosphatase, 269 m-IU/ml; and negative a-feto globulin. Multiple osteolytic metastases were seen et al Arch Pathol/Vol 97, Jan 1974 Hemangioendothelial Sarcoma/Sussman et al 39 j9898^2 m Rg 2.--HemangioendotMelial sarcoma in liver. Hemorrhagic and necrotic mass replac ing a large portion of right lobe of liver. Multiple smaller tumor nodules are scattered throughout remainder of the cirrhotic liver. fn roentgenograms of ribs, cranial vault, long bones, clavicles, scapula, and pelvis. Liver scan showed hepatosplenomegaly with a grossly irregular distribution of ra dioactivity in the liver. A regimen of fluorouracil was added to his therapeutic pro gram but he developed hepatic failure and hypotension and died. The final clinical diagnoses were meta static malignant tumor of unknown pri mary site and hemochromatosis with dia betes mellitus and mild hypothyroidism. Autopsy Findings The liver weighed 1,600 gm and was chocolate-brown with diffuse, firm, 0.3- to. 1.0-cm nodules separated by thin bands of connective tissue. Microscopically, there was a pigmen tary cirrhosis with hemosiderin de posits at approximately the same level as seen on the last antemortem liver biopsy specimen. Glycogen vacu olization of hepatocyte nuclei, bile stasis, and scattered foci of necrosis in regenerating nodules were seen. The pancreas, submandibular glands, and lymph nodes were also dark brown and the testes and thyroid were atrophic. Iron deposits were most marked in the pancreas, sub mandibular salivary glands, lymph nodes, zona glomerulosa of the adre nals, choroid plexus of the brain, testes, thyroid, and the kidneys. A largely necrotic and hemorrhagic 11 x 10 x 8-cm tumor was found in the posterior portion of the right lobe of the liver (Fig 2). Multiple 0.5to 2.0-cxn hemorrhagic nodules were scattered throughout the remainder of the right and left lobes of the liver, primarily in and around portal veins. Microscopically, the tumor consisted of large, plump, spindle-shaped cells with large, vesicular nuclei with coarse, clumped chromatin (Fig 3). Mitoses and tumor giant cells were numerous. Reticulin stains showed that the tumor cells lined anastomos ing vascular channels containing RBC. The tumor diffusely infiltrated between hepatocytes and, in some areas, bore a striking resemblance to hyperplastic Kupffer cells. A few tumor cells contained hemosiderin granules and some also showed erythrophagocytosis. Extramedullary hem atopoiesis within the tumor or re maining liver was not seen. Identical 40 Arch Pathol/Vol 97. Jan 1974 Hemangioendothelial Sarcoma/Sussman et al tuznoi and ; and t inter] thelia Th* sion and b He: their st&nc at tb and t prims liver and t ing a cases coma in ad cases lifera uncei ignat endo> ligna Kupf thelic sarco loenc prim, tic sa sarco oma. He arise liver, tane< is sc becai mult theli ficult vasci nom; ularl ney diffe Kap Tv tribe $1hem; iver ent i C*ai C5 ^yrch tumor tissue was found in many ribs and adjacent soft tissue, vertebra, and the cranial vault. This lesion was interpreted as being hemangioendo thelial sarcoma. There was also cytomegalic inclu sion virus pneumonia of the middle and lower lobes of the right lung. Hemangioendothelial sarcoma of the liver is a rare tumor. Only one in stance was found in 52,000 autopsies at the Los Angeles County Hospital* and this lesion constituted only 3% of primary malignant tumors of the liver at Memorial Hospital for Cancer and Allied Diseases in New York dur ing a 25-year period.1 Less than 100 cases of hemangioendothelial sar coma of the liver have been reported in adults,114 but the exact number of cases is uncertain because of the pro liferation of names resulting from uncertainty of the cell of origin. Des Fig 3.--Hemangioendothelial sarcoma consists of multiple anastomosing vascular channels lined by plump endothelial-like cells with pleomorphic nuclei and bizarre mitot ic figures (hematoxylin-eosin, original magnification x 250). ff =' ignations have included hemangio endothelial sarcoma of the liver, ma lignant vascular tumor of the liver, and prognosis.13'11 In adults, males cluding hepatocellular carcinoma and 4 Kupffer cell sarcoma, hemangioendo are affected approximately three cholangiocarcinoma.14 The latent pe thelioma, hemangioblastoma, angio times as frequently as females. riod between the administration of sarcoma, endothelioblastoma, reticu- Hemangioendothelial sarcoma of the thorium dioxide suspension and loendothelioma, angioplastic sarcoma, the liver is a rapidly progressive fatal the clinical onset of hemangioendo primary hepatic sarcoma, angioblas- disease in adults. Clinical findings thelial sarcoma was often as much as tic sarcoma, endothelioma, hemangio- frequently include rapid hepatic en 20 years. Further support for the role 1C sarcoma, and malignant hemangi largement, hemorrhagic ascites and of thorium dioxide suspensions came n oma. jaundice. Occasionally, a vascular with the experimental induction of Hemangioendothelial sarcomas may bruit is heard over the hepatic re this tumor in animals injected with y- arise in a variety of. sites including gion.3 Microangiopathic hemolytic colloidal solutions of thorium diox e liver, bone, spleen, breast, and subcu anemia* and hypercalcemia1 have also ide.30 Another implicated iatrogenic >r taneous tissue. The exact primary site been reported in association with this agent is arsenic, according to a report r, is sometimes difficult to determine tumor. Distant metastases are pres by Regelson et al31 concerning heman s. because of rapid spread and possible ent in approximately 50% of cases at gioendothelial sarcoma of the liver in d multicentric origin. Hemangioendo autopsy, most commonly in portal a patient who had received Fowler so Is thelial sarcomas are occasionally dif lymph nodes, lungs, bones, and lution. There was no history of or h ficult to distinguish from extremely spleen. Death may follow intraperito- morphologic evidence of exposure to ') vascular spindly hepatocellular carci neal hemorrhage secondary to rup either thorium dioxide suspension or :e nomas or vascular metastases, partic ture of the tumor or from hepatic arsenic in our patient. 1. id ularly of adenocarcinoma of the kid coma. Cirrhosis was present in approxi 8- ney and leiomyosarcoma. The Several associations of possible eti- mately one third of the reported adult . differentia] diagnosis also includes ologic importance have been noted cases of hemangioendothelial sar id Kaposi sarcoma and choriocarcinoma. with these tumors. MacMahon et al1* coma of the liver.* The types of cir le Two distinct age peaks in the dis reported the development of this tu rhosis included alcoholic, postnecrotic, bo tribution of the reported cases of mor after the administration of a tho and pigmentary. Ansari and Wei- w hemangioendothelial sarcoma of the rium dioxide suspension, and Da Silva gent4 have explained the association in ba- liver have been noted by Videbeck,3 one at 8 months and the other at 49 years. The tumor in infants is differ Horta et al1* found 22 cases of heman gioendothelioma in 1,107 patients exposed to a thorium dioxide suspen of cirrhosis and hemangioendothelial sarcoma of the liver in two ways: one, that cirrhosis plays a role in the gen CO O) CO oent from that seen in adults in clini sion. This suspension is also associ esis of this tumor as it does in hepato cal and morphologic presentation. ated with other malignant tumors in cellular carcinoma; two, that the tu Ci Arch Pathol/Vol 97. Jan 1974 Hemangioendothelial Sarcoma/Sussman et al 41 i mor itself may initiate fibrosis and nodular regeneration. The latter ex planation seems less likely, since in cases like the one reported here, dif fuse cirrhosis was demonstrated prior to any evidence of existence of the tu mor. Only two cases of hemangioendothelial sarcoma in hemochromatosis have been previously reported.*-* In one instance,* hemochromatosis was suspected only at autopsy because of pigmentary cirrhosis and iron deposi tion in pancreatic acini; however, other causes of hemosiderosis were not ruled out clinically and iron was not identified within tumor cells. In the second instance,' the antemortem diagnosis of pigmentary cirrhosis was made only a few weeks before death and was not treated. Iron was found in many of the tumor cells. In three of the four cases of hemangioendothelio ma of the liver reported by Alpert and Benisch,* hepatic siderosis was noted and the authors suspected that this was a consequence of micro angiopathic hemolytic anemia due to the tumor. 1. Edmondson HA: Tumor* of tho Liver and Intrahepatic Bile Duets. Atlas of Tumor Pathol ogy series, Washington, DC, Armed Forces Insti tute of Pathology, 1958, section 7, pt 25, p 139. 2. Adam YG, Huvoe AG, Hajdu SI: Malignant vascular tumors of the liver. Ana Surg 175375383,1972. 3. Videbeck A: Hemangioendothelioma of the liver. Acta Pacdiatr Stand 33:129-143,1946. 4. Ansari A, Weigent CE: Hemangioendotbelial sarcoma of the liver. Am J Gastroenterol 56:420-427,1971. 5. Hastings JR: Malignant hsemsagioendotbelioma (haemangioblastoma) of the liver. J Pathol 61:49-53,1949. 6. Alpert LI, Benisch B: Hemangioendothelio ma of liver associated with microangiopathic he molytic anemia: Report of four eases. Am J Med 48:624-628, 1970. 7. Case records of the Massachusetts General Hospital (Case 12, 1967). N Engl J Med 276.-629634,1967. 8. Baker H deC, Paget GE, Davison J: Haemangioendothelioma (Kupffer cell sarcoma) of the In the case reported here, the pres ence of diabetes 1% years before he mochromatosis was diagnosed, sug gests that the latter condition may have been present at least four years antemortem. The patient was treated with multiple phlebotomies that re sulted in decreasing serum iron levels and diminution of liver iron load as seen on the biopsy specimen. Never theless, he went on to develop a hemangioendothelial sarcoma of the liver, a situation analogous to an in stance of hepatoma arising in treated hemochromatosis as reported by Hines et al." It would, therefore, ap pear that the removal of iron does not prevent the development of either hepatoma or hemangioendothelial sarcoma once cirrhosis has developed. Kupffer cell origin of these tumors is suggested by the tendency to form anastomosing vascular elements lined by prominent endothelial-like cells. The presence of hyperplastic Kupffer cells in areas of the liver re mote from frank tumor as described by Baker etal* also supports a Kupf fer cell origin of this tumor. Phagocy References liver. J Pathol 72:173-182,1966. 9. Kwitten J, Tartow LR: Haemochromatosis and Kupffer-cell sarcoma with ontuoal localixation of iron. J Pathol 92371-673, 1966. 10. Burston J: Kupffer cell sarcoma. Cancer 11.798-802, 1968. 1L Galup LN, Hawkins RA, Msnalo-Estrella P: Kupffer cell sarcoma of the liver. Aenep Med 36.-988-989,1965. 12. Miller EA, Richard WG, Reed WH: Haemangioendothelial sarcoma of the liver (Kupffer-cell sarcoma). Wit Med J 63:471-475, 1964. 13. Blackwell JB, Joake RA Kupffer cell sar coma. Am J Dig Dio 15:133-138,1970. 14. Swarm RL (ed): Distribution, retention and late effects of thorium dioxide. Ann NY Acad Sei 145:525-858,1967. 15. McGahon JJ, et ah Solitary infantile hemangioendothelioma of the liver: Report of one case. Rocky Mt Med / 6138-39,1964. 16. Blumfeld TA, Flemming ID, Johnson WW: Hemangioendothelioma of the liver. Report of a case and review of the literature. Cancer 24353- tosis of a thorium dioxide suspension and hemosiderin by tumor cells mimics the activity of Kupffer cells, but does not prove Kupffer cell origin, since other tumors may demonstrate phagocytic activity including erythrophagocytosis. The significance of extramedullary hematopoiesis within the tumor is unknown. Many of the clinical features seen in patients with hemangioendothelial sarcoma of the liver are shared with patients having hepatocellular carci noma. Because of the extremely vas cular nature of this tumor, aspiration or needle biopsy is contraindicated and may result in fatal hemorrhage. Early diagnosis and partial hepatectomy might offer some hope of in creased survival but unfortunately nearly all lesions in adults are diagnosed at an advanced stage and char acterized by a rapidly progressive course leading to death within six months. Nonproprietary Name and Trademark of Drug F]uorouaa\--Efudex, Fluoroplex. 857, 1969. 17. Edmondson HA Differential diagnosis oi tumors and tumor-like lesions of liver in infancy and childhood. Am J Die Child 91:168-186,1956. 18. MacMahon HE, Murphy AS, Bates MI: En dothelial-cell sarcoma of the liver following Thorotrast injections. Am J Pathol 23:585-611,1947. 19. Da Silva Horta J, et ah Malignancy and other late effects following administration of Thorotrast Lancet 2301-205, 1966. 20. Swarm RL, Miller E, Michelitch HJ: Malig nant vascular tumors in rabbits injected intrave nously with colloidal thorium dioxide. Pathol Mi crobial (Basel) 2537-44, 1962. 21 Regehon W, et al: Hemangioendothelial sarcoma of the liver from chronic arsenic intoxicstion by Fowler's solution. Cancer 21:514-522, 1968. 22. Hines C, Davis D, Ferrant* WA Hepa toma developing in hemochromatosis in spite of sdequate treatment by multiple phlebotomies. Am J Dig Dio 16349-355, 1971. > I !> K * Cy . He I } 1 Willart Leonar ! ' Cyt tocytes of alco cssm cases light changi varlabl Cry* cytoptr limitin' measu Their i appeal The t at the proteir year nr extrerr man h- Accep From fch Burns i CO (hepato trstion cn Medical ton, DC oo voir, V: o withCe m Repri Hoepita Bums). 42 Arch Pathol/Vol 97. Jan 1974 Hemangioendothelial Sarcoma/Sussman et al Arch F