Acute Budd-Chiari Syndrome as First Manifestation of Adrenocortical Carcinoma
Franck Carbonnel, M.D., Dominique Valla, M.D., Yves Menu, M.D., Yves Lecompte, M.D., Jacques Belghiti, M.D., Bernard Rueff, M.D., and Jean-Pierre Benhamou, M.D.
We report the case of a young woman in whom investigations for acute Budd-Chiari syndrome disclosed an hormone-se- creting but clinically nonfunctioning adrenocortical carci- noma. We supply a very brief review of the literature.
Key Words: Budd-Chiari syndrome-Adrenocortical carci- noma.
Invasion of inferior vena cava with obstruction of the hepatic veins by an adrenocortical carcinoma has been reported only six times, as far as we can tell (1- 6). The condition is usually recognized at autopsy. We report the case of a young woman in whom investi- gations for acute Budd-Chiari syndrome disclosed a secreting adenocortical carcinoma.
CASE REPORT
A 28-year-old woman was admitted for ascites. One month before she complained of abdominal distention, right lumbar pain, and leg edema.
On admission, ascites, hepatomegaly, dilated epigastric and lumbar veins and pitting edema were noted. There was no sign of virilization or Cushing syndrome. Liver tests gave the following results: ALT 1,380 IU (10-40 IU), AST 680 IU (10-40 IU), and prothrombin time 18 s (normal: 12 s). Chest roentgenogram was normal. Ultrasonography disclosed a tu- mor of the right adrenal gland (Fig. 1) and obstruction of the hepatic portion of the inferior vena cava up to the level of right atrium (Fig. 2). Serum levels of adrenocortical hormones were as follows: cortisol at 8:00 a.m. >1,000 u.M (normal 580 ± 80 AM), androstenedione 23.3 mg/ml (normal 0.7- 3.3 mg/ml), dehydroepiandrosterone sulfate 3,400 mg/ml (normal 600-2,500 mg/ml), testosterone 5.4 mg/ml (normal 0.15-0.75 mg/ml); dehydroepiandrosterone, estrone, and es- tradiol were within normal range. Three days after admission, exploration confirmed the ultrasonographic findings. Inferior vena cava (IVC) was clamped 3 cm above its bifurcation, and clots in the iliac veins were removed. Venous cannulae for cardiopulmonary bypass were inserted into superior vena cava, right femoral vein, and portal vein. Arterial cannula was inserted into the ascending aorta. The right atrium was incised, and a small patent foramen ovale was immediately closed. The hepatic portion of the IVC was opened, and an adherent thrombus extending to the eustachian valve was easily cleaved (Fig. 3). A recent clot in the right hepatic vein was removed; left and median hepatic veins were patent. The right adrenal gland tumor was easily excised. In the first post-
From the Service d’Hépatologie and the Unité de Recherches de Physiopathologie Hépatique, Hôpital Beaujon, Clichy; and the Centre Medico-chirurgical de la Porte de Choisy, Paris, France.
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operative hours, anesthesia was followed by stage IV coma. The patient died 2 days later. Necropsy disclosed cerebral edema and massive infiltration of the lungs by tumor mi- croemboli. Histologic examination of the adrenal tumor demonstrated an encapsulated adrenocortical carcinoma. The material removed from the IVC had the same histologic ap- pearance as that of the adrenal tumor. The inferior vena cava was patent, but a clot was still present in the right hepatic vein (Fig. 4). Examination of liver specimens showed massive centrilobular congestion and hemorrhagic necrosis with nor- mal portal tracts and periportal areas.
DISCUSSION
Obstruction of the hepatic veins by an adrenal car- cinoma is uncommon since only six cases (1-6) have hitherto been reported. Leiomyosarcoma of the IVC, hepatocellular carcinoma, and renal carcinoma have been the most common malignancies causing hepatic vein obstruction (7). For instance, in a series of 53 cases of Budd-Chiari syndrome consecutively admitted to our unit, 6 were due to hepatocellular carcinoma, 1 was due to renal cell carcinoma, 1 was due to leio-
myosarcoma of IVC, and 1 was due to adrenocortical carcinoma.
The main features of the six reported cases of Budd- Chiari syndrome complicating adrenocortical carci- noma are summarized in Table 1. A few points deserve emphasis. First, it is noteworthy that six of seven pa- tients were young women aged 34 years on average (range 24-53 years). In our experience, at this age, the most common cause of hepatic vein obstruction is thrombosis due to a latent myeloproliferative disorder (8). Thus, although tumorous Budd-Chiari syndrome occurs mainly in older people, adrenocortical carci- noma should be considered as a cause of Budd-Chiari syndrome in young patients. Second, although serum levels of corticosteroids were high, our patient had no sign or symptom of Cushing syndrome or virilization. Similarly, Budd-Chiari syndrome was the only mani- festation of adrenocortical carcinoma in two other re- ported cases. Third, our patient presented with signs of acute and severe liver disease with high serum trans- aminases and markedly prolonged prothrombin time. Such a presentation is unusual in patients with Budd-
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Chiari syndrome (7,8). In our case, laparotomy showed that the adrenocortical adrenal tumor had extended into the lumen of the inferior cava up to the hepatic vein ostiae. The fact that all three main hepatic veins were simultaneously obstructed probably accounts for massive hepatic necrosis and severe hepatic failure.
Several reports have shown that Budd-Chiari syn-
drome can be demonstrated with a noninvasive im- aging procedure (9): in our case, ultrasonography proved extremely accurate in recognizing and delin- eating adrenocortical carcinoma, tumorous invasion of IVC, and obstruction of the hepatic veins as later verified at laparotomy. In contrast, most previously
| Reference | Age | Sex | Manifestations | Diagnosis | Tumor extension | Outcome (duration of follow-up after the first symptoms of BCS) | Cause of death |
|---|---|---|---|---|---|---|---|
| 1 | 52 | M | Right lumbar pain, hepatomegaly, ascites | Autopsy | Left ACC with hepatic vein thrombosis, generalized thromboembolism | Died (2 mo) | Hepatic failure |
| 2 | 25 | F | Dyspnea, right upper quadrant pain, ascites, hepato- megaly, hirsutism, moon face | Autopsy | Right ACC, tumor thromboses of the IVC with extension into the right atrium and both hepatic veins, tumor, pulmonary embolism, massive liver metastases | Died (6 mo) | Intraperitoneal bleeding |
| 3 | 53 | F | Epigastric pain, hepatomegaly, ascites | Cavography, autopsy | Left ACC, tumorous invasion of IVC up to right atrium, obstruction of hepatic vein ostiae | Died (10 mo) | Intraperitoneal bleeding |
| 4 | 32 | F | Secondary amenorrhea, hirsutism; removal of a right ACC; two months later, ascites and hepatomegaly | Cavography, autopsy | Tumorous invasion of IVC and right atrium, hepatic vein thrombosis | Died (3 wk) | Shock |
| 5 | 30 | F | Dyspnea, left hypochondrial pain, hepatomegaly, ascites, hirsutism | Surgical exploration | Left ACC, IVC invasion with tumor and thrombus, tumor pulmonary embolism | Died (1 mo) | Not specified |
| 6 | 38 | F | Incomplete resection of a right ACC causing hypercorticism; three months later, ascites and hepatic decompensation | Ultrasonography, cavography, surgical exploration | Hepatic veins and IVC thrombosis from L1 to right atrium | Alive at 9 mo with tumorous recurrence |
ACC, adrenocortical carcinoma; BCS, Budd-Chiari syndrome; IVC, inferior vena cava.
reported cases of Budd-Chiari syndrome complicating adrenocortical carcinoma were recognized only at au- topsy. Surgical treatment of hepatic venous outflow block was attempted in two patients, including our pa- tient (see Table 1). In both cases, tumorous material was easily cleaved from IVC and the adrenal mass could be excised. Unfortunately, our patient died early after operation, presumably of brain damage related to the cardiopulmonary bypass. Autopsy disclosed tumorous microemboli in the lungs. In the other case, the tumor recurred (6), which is usual even after radical surgery (10,11). Therefore definitive cure probably cannot be expected from surgery alone. Improvement in survival, however, could result from the association of prompt recognition through noninvasive procedure, early sur- gical treatment to relieve hepatic venous outflow block, and specific chemotherapy with OP’DDD to control residual disease (12,13).
Acknowledgment: We gratefully acknowledge the help of Dr. Jacqueline André, who performed the autopsy and the histologic studies.
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