Echocardiography in Imaging an
Extremely Rare Cause of Extrinsic
Pulmonary Stenosis: Rapidly
Progressive Primary Mediastinal
Embryonal Carcinoma
Ertuğrul Zencirci, MD, Sabahattin Gündüz, MD,
Aycan Esen Zencirci, MD, Nesrin Gündüz, MD,
Gökhan Göl, MD, and Murat Uğurlucan, MD
Department of Cardiology, Ümraniye Training and Research
Hospital, Istanbul; Department of Cardiology, Siyami Ersek
Cardiovascular and Thoracic Surgery Training and Research
Hospital, Istanbul; Department of Radiology, İskenderun
State Hospital, Hatay; Department of Cardiology,
Sureyyapasa Chest Diseases and Thoracic Surgery Training
and Research Hospital, Istanbul; and Department of
Cardiovascular Surgery, Duzce Ataturk State Hospital,
Duzce, Turkey
We report a case of a young man with a very rare cause of
atypical chest pain in whom primary mediastinal embryonal carcinoma causing extrinsic pulmonary stenosis was
diagnosed with echocardiography and computed tomography. This patient illustrates an unusual presentation of
the very rapid progression of the tumor in as little as 6
months. The patient underwent surgical resection and
was successfully treated with adjuvant chemotherapy.
(Ann Thorac Surg 2012;94:e113– 4)
© 2012 by The Society of Thoracic Surgeons
E
xtrinsic pulmonary stenosis (EPS) is an uncommon
clinical diagnosis and predominantly results from
mediastinal tumors. Imaging modalities constitute the
cardinal diagnostic tools. We present a patient with a
unique cause of EPS—rapidly progressive primary mediastinal embryonal carcinoma— causing compression of
the main pulmonary artery, and emphasize the importance of transthoracic echocardiography (TTE) and computed tomography (CT) imaging in diagnosis.
Fig 1. The scenogram obtained immediately before chest computed
tomography imaging demonstrates a large, mass-like opacity (black
arrows) located at the left side of the upper mediastinum.
ranging between 4 and 84 Hounsfield units), and measuring 63 ⫻ 76 ⫻ 70 mm in the anteroposterior, transverse, and craniocaudal diameters, respectively (Fig 1
and Fig 2).
Results of laboratory examinations were normal, except for an elevated ␣-fetoprotein level. Results of gonadal examination and scrotal ultrasonography were
normal. Imaging features indicated that the mass was not
invading the surrounding tissues, so it was deemed as
A 27-year-old man presented with atypical chest pain
lasting for 6 months. He had been evaluated in another
center 6 months before, and the chest roentgenogram
obtained at that time was unremarkable. Physical examination was normal except for a midsystolic murmur of
grade 3/6 in the second left intercostal space. His electrocardiogram was normal.
TTE in the parasternal snort-axis view demonstrated a
giant mass located adjacent to the left lateral wall of the
main pulmonary artery. The encapsulated mass measured 6 ⫻ 7 cm and included anechoic cystic areas within
it. Continuous-wave Doppler examination of the main
pulmonary artery revealed external compression causing
stenosis, with a peak systolic velocity of 3.38 m/s and a
gradient of 45.6 mm Hg. A contrast-enhanced CT of the
chest confirmed the presence of a solid mass in the
anterosuperior mediastinum with a regular border, heterogenous contrast enhancement (tomographic density
Accepted for publication April 20, 2012.
Address correspondence to Dr Zencirci, Department of Cardiology,
Ümraniye Training and Research Hospital, Elmalıkent M. Adem Yavuz C.
No. 1, 34764 Ümraniye, İstanbul, Turkey; e-mail: ertuzencirci@gmail.
com.
© 2012 by The Society of Thoracic Surgeons
Published by Elsevier Inc
Fig 2. A transverse view thoracic contrast-enhanced computed tomography image shows the large, heterogenously enhanced, solid
mass (white arrows) located at the anterosuperior mediastinum and
compressing the junction of the main and left pulmonary arteries.
0003-4975/$36.00
http://dx.doi.org/10.1016/j.athoracsur.2012.04.126
e114
CASE REPORT
ZENCIRCI ET AL
TEE AND CT FOR EMBRYONAL CARCINOMA DIAGNOSIS
Fig 3. Embryonal carcinoma is characterized by a solid sheet of
large, pleomorphic cells with glassy nuclei and prominent nucleoli
and indistinct cellular borders (hematoxylin and eosin stain, original
magnification ⫻200).
removable. Surgical resection was through a midline
sternotomy, with complete resection of the mass. Pathologic examination revealed embryonal carcinoma (Fig 3).
Cisplatin-based adjuvant systemic chemotherapy was
administered postoperatively. A postoperative TTE examination showed no residual mass or gradient. The
patient remained well at the 6-month and 1-year follow-up assessments.
Comment
Rarely seen acquired pulmonary stenosis results from
intrinsic and extrinsic causes [1]. Most EPS is caused by
mediastinal tumors that create nondynamic obstruction
of the right ventricular outflow tract [2]. Among these
tumors, mediastinal germ cell tumors with compression
of major cardiovascular structures have been reported
rarely. When an anterior mediastinal mass is found in a
young man, mediastinal germ cell tumors should always
be kept in mind, among other diagnostic considerations.
The anterior mediastinum is the most common extragonadal location for germ cell tumors. Primary mediastinal
germ cell tumors account for approximately 10% to 20%
of all anterior mediastinal tumors in adults [3].
It is uncommon for mediastinal tumors to compress the
heart or pulmonary artery sufficiently to produce hemodynamically significant obstruction or murmurs because
of the tendency of mediastinal tumors to enlarge laterally
[4]. However, very rarely as in this patient, a primary
mediastinal embryonal cell carcinoma could present as a
cause of EPS [5]. Furthermore, mediastinal germ cell
Ann Thorac Surg
2012;94:e113– 4
tumors presenting with rapid growth have been reported
rarely in the literature [6].
Pulmonary (eg cough, dyspnea, and chest pain) and
extrapulmonary symptoms due to compression on adjacent structures predominate in EPS caused by mediastinal tumors. Symptoms and cardiac auscultation are often
the initial diagnostic clues in EPS. A triad of dyspnea,
chest pain, and pulmonary ejection murmur is typical [7].
Electrocardiography may show normal sinus rhythm,
and a right axis deviation or right ventricular hypertrophy may be present. The most common finding on the
chest roentgenogram is enlargement of the mediastinum,
followed by a normal chest roentgenogram and cardiomegaly [2]. Although a physical examination, electrocardiogram, and chest roentgenogram may be helpful in
diagnosis, more sophisticated imaging techniques (eg,
CT) and biopsy specimen analysis are often required for
certain diagnosis in patients with anterior mediastinal
masses.
As this case report demonstrates, TTE is a complementary diagnostic tool for imaging of anterior mediastinal
masses and the only noninvasive method for a dynamic
investigation of the severity of great vessel compression
and invasion. Along with other imaging modalities, TTE
is also very crucial in the subsequent assessment of
therapeutic efficacy.
In conclusion, this is an uncommon case of a rapidly
progressive primary mediastinal embryonal carcinoma
presenting as a cause of EPS managed with upfront
surgical resection and adjuvant chemotherapy. Furthermore, the versatility of TTE in imaging of an anterior
mediastinal mass and its potentially significant hemodynamic consequences is also stressed.
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