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Good, Bad, or Ugly?

 

Did you ever see Sergio Leone's Western called "The Good the Bad and the Ugly"? It is a true masterpiece. Apart from featuring one of the best soundtracks ever produced (Ennio Morricone), Eli Wallach, Clint Eastwood (my hero) and Lee Van Cleef are distinctly different personalities, also changing constantly during the film. One is always in doubt as to who is good, bad, or ugly.

In a way I was reminded of this movie when I encountered the following case:


The plot

Susi, 46 years old, was admitted to a hospital in the province of lower Austria, close to Vienna, in a state of cardiogenic shock and pulmonary edema. She had been feeling low for a few weeks, but had had no other remarkable medical problems in the past.

I think you will find it quite easy to spot the pathology when you look at the following parasternal long-axis view:

Parasternal long-axis view showing a large mass in the left atrium

What a huge mass: it occupies almost the entire left atrium. The mass is clearly related to the patient's medical condition.

Four-chamber view: the mass protrudes through the mitral valve orifice.

The mass actually protrudes through the mitral valve into the left ventricle. It causes inflow obstruction and also leaves no room for blood to fill the atrium. Here you can see how blood flow is trying to find a way around the mass.

Color Doppler study showing flow obstruction

Clearly, there is turbulent flow in the left atrium - a sign of obstruction. Such pathologies tend to mimic mitral stenosis, which also causes symptoms of pulmonary edema - with the difference that the large size of the mass causes low cardiac output as well. This must be the reason why the patient was in cardiogenic shock (her blood pressure was fairly low when we saw her).

Character analysis

The reason for the patient's symptoms is clear, but the question remains: what is the mass in the left atrium? Is it “good” – a benign tumor such as a myxoma, or malignant - such as a thrombus or even a malignancy?

Sure, echo provides no information about the histology of the mass. But it does provide several clues that may point in one direction or the other. To determine whether the mass is good, bad or ugly, we have to take a look at probabilities and focus on a few more details on the echo.

Features of the bad

Could it be a thrombus? Most likely not. The patient has no predisposing factors for a thrombus. Her left ventricular function is normal, her left atrium is of normal size, and she is in sinus rhythm. To see a patient with a favorable constellation, take a look at one of our recent newsletters entitled “Stuffed Peppers”.

Good or ugly?

This is where transesophageal echo comes into play.
The first thing that strikes us is that Susi has not one, but two distinct structures in the atrium. This is not a good sign.

Two masses occupying the left atrium

Besides, the mass is fairly inhomogeneous. It has areas of variable echogenicity. However, this alone is no definite proof of malignancy because myxomas may look quite similar. Several other features favor the thesis of “ugly”.

More evidence of the ugly

Insertion of one of the two masses on the interatrial septum

One of the two structures arises from the interatrial septum. One might consider this a typical feature of a myxoma. However, myxomas usually have a stalk, whereas this mass has a broad insertion. The attachment of the second mass was even more diffuse.

One of the most important additional findings was invasion of atrial wall.

Thickened left atrial wall, showing invasion

Can you see the small chunk of tumor on the free atrial wall? One can hardly delineate the tumor from the atrial wall. This is a clear sign of invasion. In some patients with a cardiac malignancy, one can even see the tumor extending between two or more chambers.

Finally, if you return to the transthoracic images, you will notice that the patient has a pericardial effusion, which is another bad sign.

Very ugly

After the TEE study I was quite convinced that something ugly and malignant had grown in Susi's heart. Inhomogeneous invasive growth, several masses, and a pericardial effusion. As it turned out, she also had slightly elevated liver enzyme levels and anemia.

Unfortunately the odds were against her.

Since she was in a critical condition, we decided to waste no further time looking for malignancies in other organs, but sent her right away for surgery and removal of the mass.

The truth is out

The surgeons confirmed our findings. The results of histology were sent to us: the mass was a rhabdomyosarcoma, which is a highly malignant tumor.
The only other suspicious findings on CT were a fracture of the sixth thoracic vertebra and a sclerotic lesion in the hip. We hope these are not metastatic manifestations of the rhabdomyosarcoma.

Susi is doing fine so far. Her symptoms improved in a most impressive way. We now have to get her prepared to face further sequelae, such as chemotherapy.


Thomas Binder