Ann Thorac Surg 2008;85:1092-1094. doi:10.1016/j.athoracsur.2007.08.072
© 2008 The Society of Thoracic Surgeons
Case Reports
Presentation of Pulmonary Artery Intimal Sarcoma in an Infant With a History of Neonatal Valvular Pulmonic Stenosis
Tresa Chappell, MDa,
C. Buddy Creech, MD, MPHb,
David Parra, MDc,
Arnold Strauss, MDc,
Frank Scholl, MDe,
Gina Whitney, MDd,*
a Department of Pediatrics, Vanderbilt Children’s Hospital, Nashville, Tennessee
b Division of Infectious Diseases, Vanderbilt Children’s Hospital, Nashville, Tennessee
c Division of Cardiology, Vanderbilt Children’s Hospital, Nashville, Tennessee
d Division of Critical Care Medicine, Vanderbilt Children’s Hospital, Nashville, Tennessee
e Department of Cardiothoracic Surgery, Vanderbilt Children’s Hospital, Nashville, Tennessee
Accepted for publication August 21, 2007.
* Address correspondence to Dr Whitney, 2200 Children’s Way, 5121DOT, Nashville, TN 37232 (Email: gina.whitney{at}vanderbilt.edu).
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Abstract
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Intimal sarcoma of the pulmonary artery is rare in the adult population. It is usually diagnosed postmortem in patients thought to have pulmonary emboli. We present a case of intimal sarcoma of the pulmonary artery in an infant with a history of neonatal pulmonic stenosis.
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Introduction
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Pulmonary artery sarcoma is a rare tumor described in the adult literature that often presents with pulmonary hypertension and right ventricular dysfunction. Patients are often mistakenly thought to have pulmonary thromboemboli, although advances in cardiac magnetic resonance imaging (MRI) have resulted in improved diagnostic accuracy. The diagnosis of pulmonary artery sarcoma carried a poor prognosis, although combined surgical and chemotherapuetic management offers the best hope of survival.
A 2-month-old male presented with tachypnea, poor feeding, and a heart murmur. He was found to have moderate supravalvar and valvar pulmonic stenosis with a peak systolic gradient of 55 mm Hg. He underwent balloon valvuloplasty without relief of the right ventricular outflow tract (RVOT) obstruction. He then underwent pulmonary patch arterioplasty and partial valvotomy, during which the valve was found to be thickened. Owing to residual RVOT obstruction, he underwent resection of the left anterior leaflet. Intraoperative transesophageal echocardiograhy showed no residual RVOT obstruction and moderate pulmonary insufficiency. He did well and was discharged home 4 days postoperatively. Over the next 2 months, the patient continued to have failure to thrive. At 4 months of age, the patient presented with increased work of breathing and poor feeding. A chest radiograph showed cardiomegaly, bilateral pulmonary infiltrates, and a small right-side pleural effusion. Echocardiogram revealed severe pulmonary stenosis with a peak gradient of 100 mm Hg and a 17 x 10 mm mass extending from the pulmonary valve to the pulmonary artery bifurcation (Fig 1). He underwent emergent resection of the pulmonary valve and pulmonary artery mass. He was treated with vancomycin and gentamicin for presumed endocarditis. On postoperative day 28, after multiple failed attempts at ventilator weaning and continued fevers despite broad spectrum antibiotics, chest computed tomography scan showed unchanged pulmonary nodules and new mass lesions in the ribs and thoracic spine. The etiology of these lesions was thought to be infectious in nature, despite an extensive negative work-up to that point. A biopsy of a chest wall lesion was obtained.
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Fig 1. Echocardiogram in the parasternal short axis view. An echo-bright mass was noted in the main pulmonary artery (MPA), extending into the left pulmonary artery (LPA). The right pulmonary artery (RPA) appears widely patent.
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The tissue obtained showed unclassified pleomorphic spindle cell sarcoma with features of intimal sarcoma. Review of the resected pulmonary artery "thrombus" showed spindle cells and other similar histologic features. Repeat echocardiogram at that time showed regrowth of the main pulmonary artery mass. Chemotherapy with cytoxan, doxycycline, and vincristine alternating with etoposide and ifosfamide was initiated. A cardiac MRI scan was obtained at the beginning of therapy and showed the mass in the RVOT blocking the branching of the left pulmonary artery (Fig 2). After several months of chemotherapy, the patient’s ventilatory status slowly improved, and he was able to transition to a home ventilator, allowing him to be discharged home between treatments. A repeat cardiac MRI scan 3 months into chemotherapy showed significant decrease in the size of the RVOT mass (Fig 3), and subsequent computed tomography scans have shown a decrease in tumor mass in his thoracic spine and lungs.
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Fig 2. Cardiac magnetic resonance imaging axial double inversion recovery fast spin echo image showing the mass in the main pulmonary artery (MPA), extending into the left pulmonary artery (LPA). The right pulmonary artery (RPA) appears widely patent.
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Fig 3. Cardiac magnetic resonance imaging axial double inversion recovery fast spin echo image obtained 3 months into chemotherapy shows significant decrease in the size of the mass with no obstruction in the main pulmonary artery (MPA), left pulmonary artery (LPA), or right pulmonary artery (RPA).
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Comment
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Pulmonary arterial sarcomas are exceedingly rare and most often diagnosed in adult patients. Since the initial description in 1923 by Mandelstamm, approximately 200 cases in patients aged 13 to 81 years, with an average age at presentation of 49.3 years [1], have been reported. Farooki and coworkers [2] reported 2 cases of pulmonary artery sarcoma, both in teenagers, but both cases were consistent with hemangiopericytoma upon pathology review. Therefore, this patient is the first report of pulmonary artery sarcoma in an infant and of intimal sarcoma in a child.
Patients with pulmonary arterial tumors usually present with evidence of pulmonary arterial hypertension and right ventricular failure. As would be expected, patients are often misdiagnosed with pulmonary thromboemboli, as was the case with this infant. On review of 100 cases by Nonomura and coworkers [3], only 7 patients were diagnosed preoperatively or antemortem. At the time of excision, the tumors may be mistaken for mucoid-appearing thrombi within the lumen of the pulmonary artery. Pathology examination reveals tissue of intimal origin, with growth into the lumen of the vessel. Most are poorly differentiated or undifferentiated.
The true incidence of pulmonary arterial tumors is likely underestimated because of the misdiagnosis of thromboembolic disease. Findings such as fever, weight loss, and anemia may help distinguish pulmonary arterial malignancy from obstruction due to congenital or thromboembolic disease [1]. Echocardiography may allow visualization of the tumor within the right ventricular outflow tract. Advances in cardiac MRI imaging have improved the rate of preoperative diagnosis substantially in recent years. Factors that favor diagnosis of pulmonary artery sarcoma are heterogeneous soft tissue density, vascular distension, and enhancement of the gadolinium contrast [1].
Although pulmonary artery sarcoma carries a poor prognosis because of difficulty in diagnosis and high metastatic potential, surgical resection offers the best chance of survival. In a review of 93 cases of pulmonary artery sarcoma, Kruger and associates [4] reported a median survival of 1.5 months after diagnosis. However, the 27 patients who underwent surgical resection at the time of diagnosis survived an average of 10 months. The role of chemotherapy in the treatment of pulmonary artery sarcoma is unclear. A recent review reported about a 50% response rate to palliative chemotherapy with anthracyclines and ifosfamide in patients with advanced pulmonary artery sarcoma [5].
There is little information to direct treatment or predict outcome in this patient, given the rarity of this tumor in children. His tumor burden has steadily decreased with surgery and chemotherapy. Given the experience in adults, his long-term prognosis is poor. It is likely that his original pulmonic stenosis at 2 months of age was due to tumor growth at the valve. Therefore, pulmonary artery sarcoma should be considered in the differential diagnosis of pulmonary stenosis presenting in infancy.
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References
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- Yi ES. Tumors of the pulmonary vasculature Cardiol Clin 2004;22:431-440.[Medline]
- Farooki Z, Chang C, Jackson WL, et al. Primary pulmonary artery sarcoma in two children Pediatr Cardiol 1988;9:243-251.[Medline]
- Nonomura A, Kurumaya H, Kono N, et al. Report of two autopsy cases studied by immunohistochemistry and electron microscopy and review of 110 cases reported in the literature Acta Pathol Jpn 1988;38:883-896.[Medline]
- Kruger I, Borowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of pulmonary sarcomas of the pulmonary artery Thorac Cardiovasc Surg 1990;38:91-95.[Medline]
- Manso L, Alvarez E, Quintela M, Cortes-Funes H, Hitt R. Primary pulmonary artery sarcoma: report of three cases and review of the literature Clin Lung Cancer 2007;8:277-281.[Medline]
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Abstract
Introduction
