B U D D C H I A R I S Y N D R O M E F R O M M YOS AR C OM A O F RIGHT ATRIUM F. E. DISCHE, 9. S. PRYOR A. THEODOSSI, A. A. AL-ASHBAL, AND ROGERWILLIAMS Liver Unit and Department of Pathology, King’s College Hospital & Medical School, London, SES; Royal Devon and Exeter Hospital, Barrack Road, Exeter, Devon; Norwich and Norfolk Hospital, St Stephen’s Road, Norwich, Norfolk

PLATES LXXXV-LXXXVZII MA L I G N ANprimary T tumours of the heart are rare and are usually unpredicted findings at necropsy (Straus and Melos, 1945). However, in those instances where the diagnosis is made during life, the presenting features have been those of congestive cardiac failure, arrhythmias or cardiac obstruction (Goodwin, 1968; Cheitlin et al., 1975). The patient described here presented with an acute Budd Chiari syndrome which, to our knowledge, has not been previously reported as a manifestation. CASEREPORT A 50-yr-old senior pathology technican was admitted to Norfolk and Norwich Hospital on 14 August 1977 with a 10-day history of abdominal pain, backache, and a painful left leg. He had been in good health until that time. On examination his abdomen was distended by ascites and was generally tender to palpation. There were signs of a left popliteal vein thrombosis. Laboratory investigations revealed a high blood urea (14.5 mmol/l, normal 3.3-6-7) and a raised white cell count (12.6 x 106/pl, normal 4-10 x 106/pl). The following day he was noted to be clinically jaundiced. Liver function tests showed a bilirubin of 40 pmolll (normal 3-20), alkaline phosphatase 350 IUjl (20-SS), and aspartate transaminase 212 IUjl (10-50). Liver scan appearances were not diagnostic but showed hepatomegaly with some impairment of uptake in the right lobe (fig. 1). Because of his deteriorating clinical state, with continuing abdominal pain and a fall in haemoglobin by 4 g/dl, laparotomy was carried out on 25 August. The inferior vena cava was found to be thrombosed and the liver enlarged and tense. However, liver biopsy histology did not show any specific abnormality. Renal failure became progressively more marked with the serum creatinine rising to 400 pmol/l(45-105) and the development of heavy albuminuria. The clinical course was also complicated by the appearance of hepatic encephalopathy, and the development of a right femoral artery thrombosis requiring a disobliteration procedure and large pleural effusions requiring drainage. After two periods of haernodialysis the patient was transferred on 27 September t o the Liver Unit at King’s College Hospital. On examination he was drowsy and had a flapping tremor. There was ascites and gross leg and sacral oedema with signs of bilateral pleural effusions. Examination of the cardiovascular system was normal, with a regular pulse, normal heart sounds, and no elevation of the jugular venous pressure. A chest X-ray showed normal cardiac size and outline. A clinical diagnosis of Budd Chiari syndrome secondary to inferior vena caval thrombosis, possibly as a result of intra-abdominal -

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malignancy, was made. The associated renal failure was attributed to renal vein thrombosis. Liver and renal function continued to deteriorate. The EEG showed findings compatible with a metabolic encephalopathy and the prothrombin time remained elevated at 26 s (control 13 s), serum albumin 20 g/l (normal 35-50 g/l), bilirubin 128 pmol/l, alkaline phosphatase 199 IU/l, and aspartate transaminase 239 IUjl. The blood urea was 22-1 mnmol/l and there was persistently heavy albuminuria. On 28 September the patient became hypotensive and the central venous pressure rose to 15 cm water. A dopamine drip was started and, although this maintained his blood pressure at 95/60 mmHg, the patient suffered a cardiac arrest from which he could not be resuscitated.

NECROPSY FINDINGS Necropsy was carried out 23 hr after death. There was oedema of both lower limbs, pulmonary oedema, pleural effusions and ascites, the peritoneum containing 2200 ml of straw-coloured fluid. An oval polypoid tumour mass ( 8 ~ 6 x cm) 4 was present in the right atrium (fig. 2), with a narrow site of attachment to the lateral atrial wall. Ante-mortem thrombus around the tumour extended the entire length of the inferior vena cava and into the iliac and femoral veins. Both hepatic veins contained thrombus, but only the right hepatic vein showed total occlusion, the left being much less severely affected. The renal veins were also occluded by thrombus. The splenic and portal veins were patent. Extensive oesphageal varices were present. The liver was moderately enlarged, weighing 1725 grams. The cut surface (fig. 3) showed intense centrilobular congestion of most of the right lobe. The left lobe was less severely affected. The kidneys were swollen and congested and weighed 195 grams (right) and 190 grams (left). There was no evidence of neoplasm elsewhere other than in the heart. HISTOLOGICAL FINDINGS The histological appearances of the right lobe of the liver were consistent with an acute Budd Chiari syndrome. Some areas were converted into blood lakes with only narrow rims of surviving periportal liver cells (fig. 4). The tumour of the right atrium was composed of elongated cells with tapered or blunt ends (fig. 5). The nuclei included occasional large bizarre forms and there were moderate numbers of mitoses. The cytoplasm was eosinophilic, containing localised refractile foci. Rarely there were ill-defined cross striations (fig. 5 inset). Section through the site of attachment to the atrial wall failed to reveal invasion. Electron microscopy of’ atrial tumour. This was carried out on post-mortem tissue fixed in formol saline, post-osmicated and embedded in Araldite. Tumour cells were elongated or of irregular shape and contained numerous longitudinallyarranged parallel masses of intracytoplasmic microfibrils (figs. 6 and 7) lacking periodicity or dense plaques. At high magnification there was a suggestion of alternating thick (5nm) and thinner filaments. Each cell contained one or more dense areas of fibrils, perhaps representing abnormal Z bands; these foci incorporated particulate material, possibly glycogen. The lesion was considered to be a myosarcoma and in view of the alternating thick and thin

THEODOSSI, AL-ASHBAL, DISCHE,PRYORA N D

WILLIAMS

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FIG. 1.-Liver scan. Hepatomegaly with impaired uptake over right lobe.

FIG.

2.--Polypoid

turnour of right atrium.

THEODOSSI, AL-ASHBAL, DISCHE, PRYORAND

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FIG. 3.-Liver

showing congested dark-coloured right lobe, with less severe congestion of left lobe.

FIG.4.-Liver

histology. Intense centrilobular congestion of the right lobe with blood lakes. Haematoxylin and eosin (H&E). x 80.

THEODOSSI, AL-ASHBAL, DISCHE,PRYOR

AND

BUDD

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tumour: elongated and pleomorphic cells. H&E. x 360. Inset: rarely, the elongated cells have ill-defined cross-striations (arrow). H&E. x 1270.

FIG.5.-Cardiac

FIG.6.-Electron micrograph of cardiac tumour. Elongated and irregular shaped cells containing bundles of parallel microfibrils; also felted masses of fibrils often in perinuc!ear position. A few collagen fibrils in interstitial matrix. Uranyl acetate and lead citrate. Y 5500.

THEODOSSI, AL-ASHEAL, DISCHE, PRYOK A N D WILLIAMS

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FIG.7.-Cardiac turnour cell containing parallel intracytoplasmic microfibrils. A central obliquely orientated felted mass of fibrils: suggestive of an abnormal Z band; dense particulate material, possibly glycogen, in relation to thk. EM. y 26,000.

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filaments (Morales et al., 1972) the tumour was thought more likely to be a rhabdomyosarcoma rather than leiomyosarcoma. DISCUSSION Although there has been one previous report of Budd Chiari syndrome due to a secondary tumour in the right atrium, the primary growth being in the testis (Feingold et al., 1971), its occurrence due to a primary myosarcoma has not previously been reported. Although it is not possible to be certain, because of the suboptimal preservation of the post-mortem material, the electron microscopical findings in our patient were more suggestive of a rhabdomyosarcoma than of a leiomyosarcoma. There were, however, no clinical features to indicate the presence of a cardiac lesion. The initial clinical diagnosis of Budd Chiari syndrome was difficult to sustain : the presenting features with hepatomegaly, ascites and abdominal pain were consistent with it, but the liver scan appearances, with a filling defect in the right lobe, were more suggestive of a tumour deposit. In the Budd Chiari syndrome the appearances are usually diagnostic, with prominent central uptake of the isotope due to preserved function of the caudate lobe which has a separate venous drainage (Meindok and Langer, 1976). Uptake into the left and right lobes is usually impaired or absent with prominent spinal and rib localisation of isotope. Again, although at laparotomy the liver was enlarged and tense and the thrombosis of the inferior vena cava was evident, characteristic histological appearances (Scheuer, 1973) of the Budd Chiari syndrome were not seen in the biopsy specimen. This was taken from the inferior surface of the left lobe of the liver. The explanation for this was found at necropsy which showed the liver to be more severely affected in its right Iobe with only minor changes in the left lobe. The aetiology in cases of Budd Chiari syndrome is often obscure (Tavill et al., 1975). One group of causes includes hypercoagulable states as in polycythaemia rubra Vera (Thomas and Caroli, 1971), paroxysmal nocturnal haemoglobinuria (Peytreniann et nl., 1972), or ingestion of oral contraceptives of the oestrogen-progesterone type (Hoyumpa et al., 1971). Hepatic venous obstruction following thrombosis of the inferior vena cava is usually secondary to malignant disease such as renal carcinoma spreading along the renal vein. Rare tumours that may cause it include leiomyosarcoma of the inferior vena cava (Cardell et al., 19’71) or of the hepatic veins (MacMahon and Ball, 1971). It would seem likely that the primary cardiac tumour in our patient had been present for some time, and it was the thrombosis of the inferior vena cava which precipitated the clinical illness. However, even though surgery is becoming increasingIy more effective for cardiac tumours (Gerbode, 1972), once significant inferior vena caval thrombosis has occurred it is very unlikely to be successful, even if it had been possible to show the presence of the lesion. SUMMARY A 50-year-old man presented with abdominal pain followed by marked liver and renal dysfunction. Although liver scan appearances were not

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diagnostic, at laparotomy a tense, enlarged liver was found with thrombosis of the inferior vena cava. Despite full supportive measures the patient died within a month of laparotomy. Subsequent necropsy confirmed the diagnosis of Budd Chiari syndrome and revealed the primary cause to be a myosarcoma of the right atrium, an occurrence hitherto unreported. We are grateful to Professor Colin Berry for advice on electron microscopy, to Mr R. Senkus for photographic material and to Dr B. S. Cardell for help and advice. Linda Rimmer provided editorial assistance. REFERENCES CARDELL, B. S., MCGILL,D. A. F., AND WILLIAMS, R. 1971. Leiomyosarcoma of inferior vena cava producing Budd Chiari syndrome. J. Path., 104, 283. CHEITLIN, M. D., DE CASTRO, C. M., KNOWLES, D. M., FANUGLIO, J. J., AND MCALLISTER, H. A. 1975. Clinical pathology conference. Am. Heurt J., 90, 248. FEINGOLD, M. L., LITWAK, R. L., GELLER, S. S., AND BARON,M. M. 1971. Budd-Chiari syndrome caused by a right atrial tumour. Arch. Intern. Med., 127, 292. GERBODE, F. 1972. Tumors of the heart. Cardiovasc. Clin., 3, 59. GOODWIN, J. F. 1968. The spectrum of cardiac tumors. Am. J. Cardiol., 21, 307. HOYUMPA, A. M., SCHIFF, L., AND HELFMAN, E. L. 1971. Budd Chiari syndrome in women taking oral contraceptives. Am. J. Med., 50, 137. MACMAHON, H. E., AND BALL,H. G. 1971. Leiomyosarcoma of hepatic vein and the Budd Chiari syndrome. Gastroenterology, 61, 239. MEINDOK, H., AND LANGER, B. 1976. Liver scan in Budd Chiari syndrome. J. Nucl. Med., 17, 365. MORALES, A. R., FINE,G., AND HORN,R. C. 1972. Rhabdomyosarcoma: an ultrastructural appraisal. Path. Annu., 7 , 81. PEYTREMANN, R., RHODES, R. S., AND HARMANN, R. C. 1972. Thrombosis in paroxysmal nocturnal haemoglobinuria with particular reference to progressive diffuse hepatic venous thrombosis. Ser. Haemafol.,5 , 115. P. J. 1973. Liver Biopsy Interpretation. Baillibre, Tindall & Cassell, London. SCWEUER, STRAUS, R., AND MELOS,R. 1945. Primary tumors of the heart. Arch. Pathol., 39,75. TAVILL, A. S., WOOD,E. J., KREEL, L., JONES, E. A., GREGORY, M., AND SHERLOCK, s. 1975. The Budd Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology, 68, 509. THOMAS, M., AND CAROLI, J. 1971. Polycythemie et syndrome de Budd Chiari: a propos de 17 observations. Ann. Med. Irzicrne, 122, 1175.

Budd Chiari syndrome from myosarcoma of right atrium.

B U D D C H I A R I S Y N D R O M E F R O M M YOS AR C OM A O F RIGHT ATRIUM F. E. DISCHE, 9. S. PRYOR A. THEODOSSI, A. A. AL-ASHBAL, AND ROGERWILLIAM...
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