DOI:10.2214/AJR.04.1548
AJR 2006; 186:365-367
© American Roentgen Ray Society
Myxoid Fibrosarcoma of a Pulmonary Vein with Extension into the Left Atrium
Patrick Hoffstetter1,
Behrus Djavidani1,
Stefan Feuerbach1,
Ferdinand Hofstädter2 and
Johannes Seitz1
1 Department of Diagnostic Radiology, University of Regensburg,
Franz-Josef-Strauss-Allee 11, Regensburg, Germany 93047.
2 Department of Pathology, University of Regensburg, Regensburg, Germany.
Received October 14, 2004;
accepted after revision January 24, 2005.
Address correspondence to Patrick Hoffstetter
(patrick.hoffstetter{at}gmx.de).
Keywords: cardiac imaging • MDCT • MRI • oncologic imaging
Introduction
Primary malignancies of the heart and especially of the great vessels are
extremely rare. These tumors make clinical diagnosis and therapy difficult
because in most cases symptoms are unspecific and occur more in the advanced
stages of the disease. Modern imaging technology such as sonography, CT, and
MRI make the findings of these lesions more frequent and enable discovery of
these malignancies in their earlier stages. The probability of successful
resection is increased, but the prognosis is still very poor. Presented here
is a rare case of a myxoid fibrosarcoma involving the pulmonary vein with
extension into the left atrium.
Case Report
Clinical History
A 44-year-old woman was sent to the emergency department by her general
practitioner because of tachycardia and visual disturbances. She complained of
a cough for 1 week, and had low blood pressure and occasional nausea for about
1 year. She had had 2 syncopal episodes so far. Both her neurologic and
cardiac evaluations in the past were negative. Due to a tick bite 2 months
prior, her general practitioner administered an FSME (tick-borne encephalitis)
vaccination. At that time, she was taking no medications and denied illicit
drug use. On physical examination, the patient appeared well, with blood
pressure of 120/90 and heart rate of 90. Cardiac and pulmonary examinations
were normal. The ECG showed a regular rhythm.
Imaging
The chest radiograph revealed moderate pulmonary edema. After a normal CT
of the brain, the patient underwent a lumbar puncture to exclude an FSME
infection, with a negative result. Pneumonia was suspected and the patient was
started on antibiotics. Three days later, a chest film showed increasing edema
and pleural effusions. The patient underwent both a bronchoscopy and a CT of
the chest. The bronchoscopy was negative. The CT scan confirmed the edema and
pleural effusions and a mass of 2.6 x 5 cm in the left atrium with
extension into one of the right pulmonary veins
(Fig. 1A). Echocardiography
confirmed this tumor. MRI with IV contrast was performed and revealed an
extended mass of the left atrium. It seemed that the right lower pulmonary
vein was the origin of the mass. The signal intensity was high on T2 and
intermediate on T1-weighted sequences (Figs.
1B and
1C). There was slight
contrast enhancement of the tumor. Cine images revealed diastolic prolapse of
the tumor into the left ventricle causing a functional severe mitral stenosis
(Figs. 1D and
1E). The most probable
diagnosis was a neoplasm with its origin in the right lower pulmonary vein. A
myxoma seemed unlikely because usually myxomas do not grow against the
direction of the blood flow. Because an echocardiography a year ago showed no
evidence of pathology, its dynamic of growth was another argument against its
being a benign lesion. A thrombus could have been included in the differential
diagnosis, but the signal was not characteristic of a thrombus. Additional
cardiac catheterization was performed, resulting in an increased pulmonary
capillary wedge pressure. There were no abnormal vessels detected, which might
support the hypothesis of a primary cardiac tumor.
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Fig. 1D —44-year-old woman with myxoid fibrosarcoma of a pulmonary vein. Cine
MR images obtained on 1.5-T scanner with true fast imaging with steady-state
free precession (FISP) (four-chamber view). On systolic image (D), mass
fills nearly whole left atrium. Diastolic image (E) shows mass
prolapsing into left ventricle and causing severe functional mitral
stenosis.
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View larger version (106K):
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[in a new window]
[as a PowerPoint slide]
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Fig. 1E —44-year-old woman with myxoid fibrosarcoma of a pulmonary vein. Cine
MR images obtained on 1.5-T scanner with true fast imaging with steady-state
free precession (FISP) (four-chamber view). On systolic image (D), mass
fills nearly whole left atrium. Diastolic image (E) shows mass
prolapsing into left ventricle and causing severe functional mitral
stenosis.
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|
Pathology/Therapy
The patient underwent a thoracotomy the next day that confirmed our
diagnosis of a tumor of the right lower pulmonary vein with extension into the
left atrium. A complete radical resection was not possible because of the
tumor's wide extension into the pulmonary vein. The patient received a
pulmonary graft. Histologic examination showed a malignant tumor composed of
spindle cells in a mostly myxoid matrix, which were immunohistochemically
negative for actin, desmin, and CD34. The tumor was classified as a myxoid
fibrosarcoma. The patient tolerated the operation well. After 3 days on the
ICU and another week of recovery, she was sent to the oncology department for
adjuvant chemotherapy with doxorubicin and ifosfamide. She underwent 4 cycles
of chemotherapy, and 9 months after the diagnosis she is doing well. No
recurrence or distant metastasis has been detected so far by CT control
scans.
Discussion
Patients with sarcoma of the pulmonary vein seem to be in the smallest
subgroup of rare cases of primary sarcomas of the great vessels
[1,
2]. To our knowledge, 23 cases
of primary sarcoma of the pulmonary vein, most of them leiomyosarcomas, have
been reported [3,
4,
5]. The histologic
differentiation between leio- and fibrosarcoma is difficult and the criteria
for classification often change. An important criterion in classifying our
case as a fibrosarcoma is the negative reaction to actin and desmin, which is
typical for smooth muscle differentiation. There are no relevant differences
between the clinical features and prognosis of the histologic subgroups. It
seems that women are mostly affected; only 7 cases in men are known. The mean
age is 50 years, with a range of 23-74 years. Typical symptoms of pulmonary
vein lesions are exertional dyspnea, hemoptysis, and chest pain. Although
heart failure is reported, some patients are asymptomatic for a long period.
In our case, the symptoms were caused by a severe functional mitral stenosis.
All cases exhibit a relatively short course of clinical presentation, normally
3 months. Like all sarcomas of the great vessels and the heart, the prognosis
of pulmonary vein sarcoma is poor. Only two patients are known who have
survived 3 or more years. This represents a 3-year survival rate of about 5%
[4,
5]. Surgical resection with
wide margins seems to offer the only chance of cure, and the role of other
therapy options such as chemotherapy or radiation either adjuvant or
neoadjuvant are not clear at the moment. Such a poor prognosis would seemingly
demand adjuvant therapy, but so far, its efficacy has not been proved
[1].
Because of the low number of cases, not much is known about the radiologic
features of pulmonary vein sarcomas. A review of the literature shows that in
about 50% of the cases, CT and MRI were used to make the diagnosis. In nearly
all cases, the lesions were depicted as a left atrial mass and the origin of
the pulmonary vein was not obvious. The differential diagnosis for left atrial
masses includes thromboses, valvular vegetations, and primary or secondary
neoplasms. Today, transthoracic echocardiography is still the method of choice
for detecting cardiac tumors because of its noninvasive character. This method
allows successful differentiation between a tumor, the most common of which
are myxomas, and the other elements of the differential diagnosis.
Another diagnostic option is transesophageal echocardiography. This offers
higher spatial resolution, which is independent of an optimal window
[6]. A major advantage of
echocardiography is the real-time modus of the imaging. If results of the
echocardiography are not clear or if a sarcoma is suspected, a CT scan can
provide additional information. Cardiac-gated MDCT seems to have the potential
to become the method of choice in the future because, in contrast to cardiac
echo, all cardiac veins can be visualized and extramural growth or invasion of
other anatomic structures can be seen. Compared with echocardiography, CT
provides a better soft-tissue contrast but lower spatial resolution. Another
advantage is that CT provides much clearer depiction of fat and calcifications
[7]. MRI combines many of the
advantages of sonography and CT. It is noninvasive, achieves the best
soft-tissue contrast, and cine MRI allows functional analysis. Thus MRI is a
powerful tool in the detection and differential diagnosis of cardiac and great
vessels tumors.
References
- Burke AP, Virmani R. Sarcomas of the great vessels: a
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- Burke AP, Virmani R. Primary cardiac sarcomas in tumors of the
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lesions: case 3, pulmonary vein leiomyosarcoma presenting as a left atrial
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Introduction
Case Report
