Case report
An 18-year-old young lady with mild dyspnea and a 15-day history of
treatment with a preliminary diagnosis of respiratory infection was
admitted to our hospital. The diagnosis of pneumonia was suspected. In
this context, a computerized tomographic (CT) scan revealed a 4 x 2,5 cm
mass, involving the left atrium and the right inferior pulmonary vein,
suggesting an intracardiac neoplasm. The patient was referred to us with
a suspected diagnosis of a left atrial tumor. This was confirmed by a
cardiac magnetic resonance imaging (MRI) that also showed a 4.4 x 3 x
2.2 cm mass in the left atrium extending to the right inferior pulmonary
vein with gadolinium enhancement. A transthoracic and transesophageal
echocardiogram showed a mass with little mobility in the left atrium
extending to the right inferior pulmonary vein (Figure 1). The tumor was
not obstructive, and both ventricular functions were preserved. We
performed positron emission tomography (PET) CT to exclude malignancy
and metastasis. PET CT revealed high fludeoxyglucose (FDG) uptake, and
surgery was planned.
Because of the possible necessity for lung resection, the right
submammarian thoracotomy approach was chosen. A double-lumen
endotracheal tube was placed for unilateral ventilation during surgery.
A right sub-mammary skin incision was performed to avoid damaging the
mammary gland, after that subcutaneous fat and mammary gland were
dissected from the fascia to expose the 4th rib. After
unilateral lung ventilation was initiated, the pericardium was opened
vertically 1.5-2 cm anterior to the right phrenic nerve and suspended to
ensure adequate exposure. After heparin was administered, aorta cannula
was placed in the ascending aorta, and bicaval venous cannulas were
inserted via the same thoracic incision. The cardioplegia needle was
placed into the aortic root from the thoracic incision, and
cardiopulmonary bypass (CPB) was started with mild hypothermia. A
Chitwood clamp was used through the 4th intercostal space to clamp the
ascending aorta, and cold blood cardioplegia was given. After the heart
was arrested, the right atriotomy incision was performed, the
interatrial septum was opened and the mass in the left atrium was
reached. The tumor had a stable consistency, there was no necrosis or
ulceration on its surface, and it was gently attached to the left atrial
wall with a thin capsule. The tumor extended to the bifurcation of the
right lower pulmonary vein branches, and it could be excised entirely
with its stalk (Figure 2). Cold saline was used to rinse the heart
chambers, and careful examination was performed to ensure complete
resection. After the de-airing procedure, the interatrial septum and the
right atriotomy was closed primarily. The aortic cross-clamp was
removed, and the heart was reperfused.
The tumor was evaluated with the frozen section examination but couldn’t
be classified as benign or malignant neoplasm during the operation.
Therefore, we didn’t perform lymph node dissection and right lower
lobectomy. Cessation of CPB was uneventful, and the thoracotomy was
closed. The patient recovered smoothly, she was transferred to ward on
postoperative day 1, and discharged from the hospital on postoperative
day 6. On the histopathologic evaluation, multiple sections
revealed pleomorphic spindle tumor cell fascicles with nuclear
hyperchromasia. Atypical mitosis was seen (14 mitotic activity/10 HPF)
with coagulation necrosis. Actin and Desmin were positive for reaction,
but CD34 and S100 were negative (Figure 3). A total of 30% positivity
was shown by tumor cells for Ki-67, and these morphological features
were consistent with a diagnosis of leiomyosarcoma.
After histopathologic confirmation of leiomyosarcoma, we planned right
lower lobectomy to be sure complete resection of the tumor and to
prevent a recurrence. The same incision was used. Right lower lobectomy
with right inferior pulmonary vein excision, mediastinal lymph node
dissection and partial left atrial resection was performed without CPB.
The samples of tissues were taken from the surgical margin. The
thoracotomy was closed, and the patient was transferred to the
cardiovascular intensive care unit. The postoperative period was
uneventful, and the chest tube was removed on postoperative day 5. The
patient discharged from the hospital on a postoperative day 7.
Histopathological examination revealed that the resected pulmonary vein
and left atrial specimen borders were histologically clear.
Thorax and abdomen MR scans revealed no metastases in the postoperative
early period. According to a multidisciplinary staff, adjuvant
chemotherapy consisting of four cycles of Doxorubicin and Ifosfamide was
prescribed by the medical oncology department. Moreover, she had
adjuvant cardiac radiotherapy (54 Gy in 27 fractions) at postoperative 5th month. The patient is regularly followed, no
metastasis was detected in PET CT and thoracic MR scans, after 4 and 6
months follow up, respectively. After a follow-up of 12 months, she
still has no clinical or radiological evidence of recurrence.