*From the Department of Medicine, Division of Pulmonary and Critical Care Medicine (Dr. Wilson), Division of Cardiology (Dr. Guardino), and the Department of Cardiothoracic Surgery (Dr. Shapira), Boston University School of Medicine, Boston, MA.
Correspondence to: Oz M. Shapira, MD, FCCP, Department of Cardiothoracic Surgery, Boston Medical Center, 88 East Newton St, Boston, MA 02118; e-mail: email@example.com
Pulmonary tumor embolism as a presenting feature of hepatocellular
carcinoma is rare. It has been reported only once previously. In that
case, other signs and symptoms of liver disease were present. We report
the case of a patient with cavoatrial hepatocellular carcinoma
presenting with isolated pulmonary tumor embolism, without any clinical
or laboratory evidence of liver disease. Hepatocellular carcinoma
should be included in the differential diagnosis as a rare cause of
pulmonary tumor embolism.
presenting features of hepatocellular carcinoma include abdominal pain,
a right-upper-quadrant mass, weight loss, ascites, and abnormal liver
function tests. Winterbauer et al1 described the only case
reported in the literature of hepatocellular carcinoma presenting with
pulmonary tumor embolism. In that case, there were other symptoms and
laboratory findings that were suggestive of associated liver disease.
We report a case of a patient with hepatocellular carcinoma presenting
with pulmonary tumor emboli. In contrast to the prior case, there was
no evidence of acute or chronic abdominal pathology on presentation.
A 65-year-old man presented with recurrent episodes of dyspnea.
He denied orthopnea, hemoptysis, cough, peripheral edema, or chest
pain. His medical history was significant for coronary artery disease
with recent non-Q-wave myocardial infarction and obesity. There was no
history of hepatitis or alcohol intake. Admission physical examination
was unremarkable. A plain chest radiograph was clear, and the
laboratory profile was within normal limits.
Two-dimensional Doppler echocardiogram revealed moderate to severe
biventricular dysfunction and a 6-cm echogenic mass in the intrahepatic
inferior vena cava (IVC) extending into the right atrium (Fig 1
). A ventilation-perfusion lung scan revealed multiple unmatched
perfusion defects, consistent with pulmonary embolism. The mass was
assumed to be an organized thrombus, the patient was treated with
heparin, followed by warfarin, and discharged home.
Several weeks later, he was readmitted with recurrent dyspnea. A repeat
ventilation-perfusion scan showed multiple new abnormalities consistent
with new pulmonary emboli. The international normalized ratio was
within therapeutic range. Hematologic workup for a hypercoagulable
state was normal. During this admission, he developed ascites.
Contrast-enhanced CT of the abdomen demonstrated an IVC mass extending
into the hepatic veins. A discrete liver mass was not seen. Angiography
of the IVC confirmed the presence of a 6-cm mass extending from the
level of the hepatic veins to the right atrium, with evidence of
partial obstruction resulting in retrograde filling of a lumbar vein
). Cardiac catheterization showed moderately reduced left ventricular
ejection fraction, pulmonary hypertension (pulmonary artery pressure of
56/30 mm Hg), and coronary angiography revealed a 70% left anterior
descending coronary artery stenosis.
The patient underwent coronary artery bypass grafting using the left
internal mammary artery to the left anterior descending coronary
artery. Using deep hypothermia and circulatory arrest, the right atrium
and the distal end of the IVC were explored. A 6-cm red-brown mass
covered by a thin layer of fibrin was found that was almost totally
occluding the IVC. After excising the IVC mass, extension of this tumor
to the right hepatic vein was noted. The hepatic venous component could
only be partially excised. Histologic examination (Fig 3
) revealed hepatocellular carcinoma. The patient recovered uneventfully.
This case is the second reported case of tumor emboli as a
presenting feature of hepatocellular carcinoma and the only reported
case in which pulmonary embolism was the sole presentation without any
of the typical manifestations of hepatocellular carcinoma.
Hepatocellular carcinoma typically presents with some combination of
abdominal pain, right-upper-quadrant mass, weight loss, or ascites.
More than 70% of patients have cirrhosis, and > 80% have abnormal
alkaline phosphatase, 5′-nucleotidase, or serum glutamic
oxaloacetic transaminase (SGOT). None of these were observed in
the patient described.
Pulmonary manifestations of hepatocellular carcinoma vary. The most
common manifestation is the appearance of “cannonball” metastases.
Less common forms of pulmonary involvement include diffuse interstitial
infiltration, a solitary pulmonary nodule mimicking bronchogenic
carcinoma, and pleural effusion.2
Pulmonary embolization in patients with hepatocellular carcinoma may
result from either tumor embolization or thromboembolization. Tumor
embolization can range from small, asymptomatic microemboli detected
only at autopsy to massive saddle emboli complicated by cardiovascular
collapse and death. Microscopic tumor emboli are characterized by
perfusion abnormalities on ventilation-perfusion scan despite a normal
The association between venous thromboembolism and a subsequent
diagnosis of cancer has been known for over a century. Sorensen et
al4 confirmed this and showed that there were
strong associations with certain types of cancer—in particular, cancer
of the pancreas, ovary, liver, and brain. In light of the demonstrable
association between venous thromboembolism and primary hepatic cancer,
it is surprising that only one case of hepatocellular carcinoma
presenting as pulmonary thromboembolism has previously been reported.
This may be due to the low prevalence of hepatic cancer in the western
world or because the diagnosis of malignancy is typically established
prior to the thromboembolic event.
We believe that in this case the pulmonary emboli were tumor emboli
rather than thromboemboli, for several reasons. The IVC mass enlarged,
and the patient had recurrent embolic events despite therapeutic
anticoagulation. The tumor was covered with only a very fine fibrin
layer, with the cephalad end being free of thrombus. There was no
evidence of deep vein thrombosis on lower-extremity and pelvic venous
ultrasound. Finally, the tumor mass at the cavoatrial junction almost
completely occluded the IVC, preventing embolization from peripheral
The only clue to the presence of hepatocellular carcinoma in this
case was the IVC mass. The differential diagnosis of an IVC mass
includes an organized thrombus and primary or secondary tumors such as
renal cell carcinoma. However, other rare tumors, such as
hemangiosarcoma and IV leiomyomatosis, have also been
described.5–6 In our case, findings of a contrast-enhanced
CT scan specifically aimed at these diagnoses were negative.
The surgical approach to IVC masses is difficult. Deep
hypothermia and circulatory arrest have been increasingly used
to afford optimal exposure and to allow complete resection of the
In summary, hepatocellular carcinoma should be included in the
differential diagnosis of pulmonary tumor embolism, even in the absence
of clinical or laboratory data suggestive of abdominal pathology. The
diagnosis should be strongly considered in any patient with an IVC
Abbreviation: IVC = inferior vena cava
Become a CHEST member and receive a FREE subscription as a benefit of membership.
Individuals can purchase this article on ScienceDirect.
Individuals can purchase a subscription to the journal.
Individuals can purchase a subscription to the journal or buy individual articles.
Learn more about membership or Purchase a Full Subscription.
Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 8
Customize your page view by dragging & repositioning the boxes below.
Enter your username and email address. We'll send you a reminder to the email address on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.