AJR 2000; 174:531-533
© American Roentgen Ray Society
Pulmonary Infarction Resulting from Metastatic Osteogenic Sarcoma with Pulmonary Venous Tumor Thrombus
Erik Nelson1 and
Jeffrey S. Klein
1
Both authors: Department of Radiology, Fletcher Allen Health Care and the
University of Vermont College of Medicine, 111 Colchester Ave., Burlington, VT
05401.
Received May 3, 1999;
accepted after revision July 15, 1999.
Address correspondence to J. S. Klein.
Introduction
Pulmonary venous thrombosis and infarction can result from a variety of
clinical conditions and are difficult to distinguish clinically and
radiographically from infarction resulting from pulmonary embolism. Although
osteogenic sarcoma most frequently metastasizes to the lungs, to our knowledge
pulmonary venous (tumor) thrombosis with lung infarction has not been
previously described in this disease. We report a case of osteogenic sarcoma
with pulmonary metastases with secondary pulmonary venous (tumor) thrombosis
and infarction presenting 9 years after resection of the original tumor.
Case Report
A 29-year-old woman presented to her oncologist with a nonproductive cough,
progressive dyspnea, and pleuritic right chest pain. The patient had undergone
limb-sparing complete surgical excision and chemotherapy for a right tibial
osteogenic sarcoma 9 years earlier. The physical examination and laboratory
data were normal. Chest radiographs showed two well-defined right lung
nodules, the largest measuring 3 cm in the right upper lobe. Because of a
concern for pulmonary embolism, a ventilation-perfusion lung scan was obtained
using aerosolized 99mTc-diethylenetriamine pentaacetic acid for
ventilation and 99mTc-macroaggregated albumin. The
ventilation-perfusion scan showed generalized decreased ventilation and
perfusion to the right lung with a small matched defect in the right upper
lobe corresponding to the nodule seen radiographically. These findings were
interpreted as a low probability for pulmonary embolism. Sonography of the
deep veins of the lower extremities had normal findings.
CT of the chest revealed a 3-cm right upper lobe mass; several nodules in
the right lower lobe, the largest one densely calcified; and a single nodule
in the superior segment of the left lower lobe. The calcified right lower lobe
nodule was adjacent to a distended right inferior pulmonary vein that
contained an intraluminal soft-tissue filling defect measuring 78 H and
extending into the left atrium (Figs.
1A and
1B). Several pleural-based
wedge-shaped opacities in the periphery of the right lower lobe and a small
right pleural effusion were seen, findings consistent with pulmonary
infarction (Fig. 1C). CT-guided
biopsy of the right upper lobe mass revealed malignant plasmacytoid and
spindle cells interwoven in an osteoid matrix with benign and malignant
osteoclasts containing metachromatic granules. These findings were consistent
with a high-grade (3/3 on the histologic grading scale) osteogenic sarcoma,
small cell type, similar in appearance to the surgical specimen from the
original tibial lesion.
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Fig. 1A. —29-year-old woman with metastatic osteogenic sarcoma that caused
pulmonary venous thrombosis and infarction. CT scan shows calcified right
lower lobe lesion with nodular soft-tissue density extending toward heart.
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Fig. 1B. —29-year-old woman with metastatic osteogenic sarcoma that caused
pulmonary venous thrombosis and infarction. CT scan at level immediately above
A shows dense calcification in mass. Note soft tissue that fills and
expands right inferior pulmonary vein and extends into left atrium
(arrow).
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Fig. 1C. —29-year-old woman with metastatic osteogenic sarcoma that caused
pulmonary venous thrombosis and infarction. CT scan through lung bases
photographed at lung windows shows two areas of consolidation within periphery
of right costophrenic sulcus. Note associated pleural effusion representing
pulmonary infarcts.
|
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To better define the extent of left atrial involvement, a transesophageal
echocardiogram was attempted, but the patient was unable to tolerate the
procedure. A transthoracic echocardiogram could not adequately assess the left
atrium. Spin-echo cardiac MR imaging showed a mass in the right inferior
pulmonary vein extending into the left atrium
(Fig. 1D), with enhancement of
the mass after IV gadolinium administration
(Fig. 1E). During the course of
her examination for metastatic disease, the patient experienced an episode of
right-sided weakness lasting approximately 10 min, which we thought
represented a transient ischemic attack caused by cerebral embolism from the
left atrial lesion.
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Fig. 1D. —29-year-old woman with metastatic osteogenic sarcoma that caused
pulmonary venous thrombosis and infarction. Axial T1-weighted spin-echo MR
image reveals low-signal-intensity filling defect (arrow) in right
inferior pulmonary vein that extends into left atrium.
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Fig. 1E. —29-year-old woman with metastatic osteogenic sarcoma that caused
pulmonary venous thrombosis and infarction. Gadolinium-enhanced T1-weighted MR
image shows enhancement of intraluminal mass representing tumor thrombus.
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Three weeks after initial presentation, the patient underwent midline
sternotomy and open heart surgery; a 3-cm soft fleshy tumor was excised from
the right inferior pulmonary vein and left atrium. Surgical pathology revealed
small cell-type osteogenic sarcoma, high grade, similar to that seen on the
aspiration biopsy of the right upper lobe mass. Subsequently a right
pneumonectomy was performed with identification of the two largest nodules in
the right upper and lower lobes and several smaller lesions. The margin of the
right inferior pulmonary vein stump was negative for tumor. The patient
recovered uneventfully and awaits resection of the left lung nodule.
Discussion
Pulmonary venous thrombosis is an uncommon condition and a rare cause of
pulmonary infarction. This entity has been most often associated with
sclerosing mediastinitis; less common causes include congenital pulmonary
venous stenosis, single lobe resection or bilobectomy, left atrial myxoma, and
squamous cell carcinoma [1,
2,
3]. This condition presents in
one of two fashions: acutely as pulmonary infarction with cough, dyspnea, and
pleuritic chest pain, or in a more protracted manner as progressive or
recurrent interstitial pulmonary edema and fibrosis
[1,
2,
3].
Although several of the reported cases of pulmonary venous infarction have
been discovered incidentally at surgery, several authors describe the use of
transesophageal echocardiography and pulmonary angiography with venous phase
imaging in diagnosing this condition
[1,
4,
5]. Radiographic findings are
variable and nonspecific and include air-space consolidation, effusion, and
interstitial opacities [2,
3]. The widespread availability
of helical CT with rapid scanning of the chest during maximum pulmonary
arterial and venous opacification allows detection of intraluminal filling
defects representing arterial emboli or venous thrombi, respectively. MR
imaging, particularly that performed with intravascular gadolinium injection,
has likewise been shown to reveal arterial emboli and venous thrombosis
[6].
The treatment of pulmonary venous thrombosis depends on the signs and
symptoms at presentation and is directed at the underlying condition. Acute
thrombosis as seen after lobectomy is usually associated with lung infarction
[3]. Although some animal and
human studies have shown successful conservative management of postoperative
pulmonary venous thrombosis with the administration of broad-spectrum
antibiotics [2,
5], most patients require
resection to prevent the development of pulmonary gangrene and death. Patients
with sclerosing mediastinitis generally have a more indolent onset of edema
and fibrosis, and although resection is the treatment of choice, many patients
are not surgical candidates. In such patients, anecdotal regression of
mediastinal disease with antifungal therapy and corticosteroids has been
reported [1]. Although patients
with bland pulmonary venous thrombosis and systemic embolization can be
treated with anticoagulation and, in the case of peripheral embolization, with
thromboembolectomy [4,
7], tumor thrombus requires
surgical resection whenever possible.
The lung is the most common site of metastatic osteogenic sarcoma, with
pulmonary nodules seen in 95% of patients in autopsy series. The nodules often
have characteristic calcification or ossification that can be seen
radiographically but is more easily visualized on CT. Metastatic osteogenic
sarcoma may have a propensity for vascular invasion, as described in a recent
report of a patient with kidney metastasis with secondary invasion of the
inferior vena cava [8].
Similarly, our patient shows osteogenic sarcoma metastatic to the lung with
pulmonary venous invasion and thrombosis resulting in lung infarction.
In summary, pulmonary venous infarction may mimic pulmonary embolism and
should be considered in a patient with a predisposing condition that may
produce pulmonary venous thrombosis, particularly primary or metastatic
intrathoracic malignancy.
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Introduction
Case Report
