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Ann Thorac Surg 2009;87:977-984. doi:10.1016/j.athoracsur.2008.08.018
© 2009 The Society of Thoracic Surgeons
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Shanda H. Blackmon
David C. Rice
Reza Mehran
Jon-Cecil Walkes
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Michael Reardon
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Review

Management of Primary Pulmonary Artery Sarcomas

Shanda H. Blackmon, MD, MPHa,b,c,d,*, David C. Rice, MMB, BChc, Arlene M. Correa, PhDc, Reza Mehran, MDc, Joe B. Putnam, MDc, W. Roy Smythe, MDc, Jon-Cecil Walkes, MDb,d, Garrett L. Walsh, MDc, Cesar Moran, MDc, Harsh Singh, MDe, Ara A. Vaporciyan, MDc, Michael Reardon, MDb,d

a The Methodist Hospital, Department of Surgery, MD Anderson Cancer Center, Houston, Texas
b The Methodist DeBakey Heart Center, MD Anderson Cancer Center, Houston, Texas
c The University of Texas MD Anderson Cancer Center, Houston, Texas
e Christchurch, Canterbury, New Zealand
d Weill Medical College of Cornell University, New York, New York

* Address correspondence to Dr Blackmon, 6550 Fannin St, Smith Tower Ste 1661, Houston, TX 77030 (Email: shblackmon{at}tmhs.org).


   Abstract
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Introduction
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The objective of this review is to determine the outcome of patients with sarcomas involving the main pulmonary artery, pulmonic valve, or right ventricular outflow tract. Survival data were analyzed using an aggregate series derived from the published literature in conjunction with a current series. Median survival was 36.5 ± 20.2 months for patients undergoing an attempt at curative resection compared with 11 ± 3 months for those undergoing incomplete resection. Median survival was 24.7 ± 8.5 months for patients undergoing multimodality treatment compared with 8.0 ± 1.7 months for patients having single-modality therapy. A complete review of diagnosis, evaluation, treatment, and surveillance of primary pulmonary artery sarcomas follows.


   Introduction
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Primary pulmonary artery (PA) sarcomas are uncommon tumors. The first published case was reported in 1923 from an autopsy by Mandelstamm [1], and since then, fewer than 250 patients with primary PA sarcoma have been described. For a subclassification of PA sarcomas, please refer to Table 1 [2–4].


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  		Table 1 Subclassifications of Primary Pulmonary Artery Sarcomas a
 
Patients with PA sarcoma often present with dyspnea, cough, hemoptysis, or chest pain. Because these symptoms sometimes mimic pulmonary emboli, patients are often initially treated with anticoagulation therapy. Clinical data that aid in the differentiation between these two diagnoses include fever, elevated erythrocyte sedimentation rate, anemia, weight loss, absence of a procoagulant state, and a lack of history for deep venous thrombosis [5]. PA sarcomas can be evaluated by a variety of imaging modalities, but a contrast computed tomography (CT) scan is usually standard.

The results of chemotherapy management alone are suboptimal [6], and there is no established treatment regimen. Survival for this disease is often reported in weeks instead of months or years. Interventions reported for PA sarcomas range from palliative stenting, pneumonectomy, debulking, and endarterectomy to excision with or without pneumonectomy and with or without reconstruction of the PA. If complete resection is achieved, the entire right ventricular outflow tract or main PA trunk may require excision.

Owing to the extensive operation required to achieve a complete resection, the paucity of clinical data indicating benefit, and the frequent lack of preoperative tissue diagnoses, aggressive resections are infrequently performed or reported. Our institutional experience with extended resections for cardiac tumors has led us to adopt an aggressive approach to these tumors in selected patients [7–9]. We report a review of the literature, including our cumulative experience to date, along with treatment guidelines derived from the combined groups separated by treatment type.


   Methods
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A literature search of Ovid/Medline was performed using the terms "sarcoma," "pulmonary artery," "surgery," and/or "chemotherapy," and/or "radiation therapy." The search was limited to English articles about humans in the past 17 years. Each article was screened for central primary PA sarcomas as the diagnosis for each patient. Patients with lung parenchymal disease were included as long as their primary tumor was a central primary PA sarcoma. The analysis only included PA sarcomas undergoing treatment such as chemotherapy, anticoagulation therapy, stenting, or surgery. Autopsy reports or diagnostic reports with no discussion of patient treatment were excluded.

The article search identified 60 patients. The case reports of patients from these articles were tabulated in a standard form to allow comparison against the current study group. The last date of reported survival was considered the last date of contact. The patients were recorded as dead or alive on the last date of contact for calculation of survival.

Statistical analysis included calculation of mean and median age, survival, and disease-free interval. Kaplan-Meier survival curves were calculated using SPSS software (SPSS Inc, Chicago, IL).

Current patients were identified from an institutional database queried for the terms "pulmonary artery sarcoma," "surgery," and "chemotherapy." The pathology department database was also queried for PA specimens. The results of the two queries were reviewed to obtain the final list of 8 patients with primary PA sarcomas undergoing surgical therapy with curative intent. A retrospective medical record review in conjunction with telephone interviews was performed on all patients identified from this search.

Institutional Review Board approval was obtained for all patient queries, and consent was waived for the 8 patients included in the current analysis. Since 2005, all patient data have been collected prospectively for an institutional PA sarcoma database.

Symptoms and Signs
Patients with PA sarcomas present with a variety of cardiopulmonary complaints. A complete list of symptoms in study patients is compiled in Table 2. Lung involvement is present in about 50% of patients [3]. The typical duration of symptoms before diagnosis is 3 to 12 months, usually because of initial misdiagnosis. The initial differential diagnosis includes pulmonary embolus, pulmonary hypertension, congenital pulmonary stenosis, fibrosing mediastinitis, or lung tumor. Clinical findings include right ventricular dysfunction, pulmonary hypertension, and pulmonary insufficiency. A systolic ejection murmur is often noted. Evidence from our experience and that of others shows that patients with large pulmonary emboli, constitutional symptoms, uncharacteristic CT scans for thrombus, and especially patients with lesions refractory to anticoagulation should have further evaluation to diagnose primary PA sarcomas at the earliest stage possible. A recommended diagnosis and treatment algorithm is presented in Figure 1.


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  		Table 2 The Methodist Hospital-MD Anderson Cancer Center Primary Pulmonary Artery Sarcoma Resections, 1999–2006
 

Figure 1
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  		Fig 1. Diagnosis and treatment algorithm for patients who do not respond to initial anticoagulation, have atypical features on computed tomography (CT) scanning, or present with constitutional symptoms in addition to shortness of breath. (MRI = magnetic resonance imaging; PA = pulmonary artery.)

 
Imaging
PA sarcomas can be evaluated by computed tomography/computed tomography angiography (CT/CTA) scan, positron-emission tomography scan, magnetic resonance imaging (MRI) and angiography, transesophageal echocardiography, intravascular ultrasound, and intracardiac ultrasound. Findings on CT scan differentiating PA sarcomas from pulmonary emboli include hyperdense lesions with nonhomogenous attenuation from hemorrhage, beaded peripheral PAs, contiguously soft tissue–filled PAs with the entire lumen occupied, vascular distention from tumor growth, distal oligemia, and extravascular spread [27, 35, 45, 46].

The CT scans in all of our patients showed hyperdense lesions and vascular distention when the tumor filled the entire PA (Fig 2). We also found MRI to be even more specific for identifying PA sarcomas, because the tumor enhances with gadolinium contrast more than bland thrombus (Fig 3) [21, 46–51]. This feature also makes gadolinium-enhanced MRI helpful for monitoring such patients after resection if an intraluminal abnormality is noted. The degree of enhancement has been shown to correlate with the degree of tumor differentiation, content of myxoid matrix, and associated thrombus [23].


Figure 2
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  		Fig 2. (Panel a) A fluorodeoxyglucose-avid recurrent primary pulmonary artery sarcoma, (Panel b) A computed tomography scan and pathology specimen of pulmonary artery sarcoma. (Panel c) Recurrent tumor within lung parenchyma. (Panel d) Gadolinium-enhanced magnetic resonance image of a pulmonary artery sarcoma involving the trunk of the pulmonary artery and bilateral main pulmonary arteries.

 

Figure 3
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  		Fig 3. Five-year cumulative survival probability in the current case series of patients having no multimodality therapy (solid line) vs a combined series of historical control patients having multimodality therapy (dotted line).

 
Angiographic findings include polyploid filling defects, defects that move with the cardiac cycle, pressure gradients across the mass, and tumor neovascularity identified on bronchial arteriography [48, 52]. Intravascular ultrasound and transesophageal echocardiography images are helpful in determining the extent of involvement of the pulmonic valve [53–56]. Because tumor is more metabolically active than thrombus, findings from fluorodeoxyglucose-positron emission tomography (FDG-PET) include increased uptake within the tumor [57, 58], which was noted in one of our patients who underwent PET evaluation (Fig 2).

Pathophysiology
Most of these tumors are noted to arise from the dorsal surface of the main pulmonary trunk [4] and the intimal or subintimal surface [59, 60] as multipotential mesenchymal cells of the muscle anlage of the bulbus cordis [46]. The pulmonic valve is involved about 30% of the time [4]. A significant amount of intraluminal extension is often noted before any extension outside the lumen of the vessel, as our study also found. The lung periphery often becomes involved from emboli, infarction, and metastases [61].

Pathology
Moran and colleagues [3] recently reported longer survival associated with leiomyosarcoma and worse prognosis with rhabdomyosarcoma. With few cases available for review, other reports in the literature have not corroborated this histologic correlation with survival [5, 15, 21, 62]. Immunohistochemical staining of the tumor is routinely performed for desmin, cytokeratin, vimentin, and actin (smooth muscle specific and muscle specific) [3, 18]. These tumors also over-express mdm2 and have gains of 12q13–14 [3, 24]. Expression of apoptosis-related proteins, p53, and DNA fragmentation are also noted to occur [28].

Treatment Results From Review of the Literature
We reviewed 77 patients from the past 17 years of published data. Survival was not reported for 11 patients, and thus their data were recorded as missing. Some type of multimodality treatment was initiated in 20 patients, which consisted of two of the three following interventions: chemotherapy, radiation therapy, or operation. Only one type of intervention was commenced in 28 patients, consisting of operation alone, chemotherapy, or radiation therapy. Reports for 12 patients did not include data about whether the patients received additional therapy, and thus their data were recorded as missing. Attempted curative resections were done in 25 patients, much like all of the patients in our current series. A more palliative type of resection was done in 24 patients, including tumor debulking, thromboendarterectomy, or palliative pneumonectomy. Recorded data for 11 patients were not appropriate to determine what type of resection, if any, was performed. Patient data from our literature review are summarized in Table 3 [5, 10–44]. Mean and 5-year survival was 18 ± 3.5 months and 18.5% in the series of historical patients (Fig 4).


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  		Table 3 Summary of Demographics for Published Case Reports of Primary Pulmonary Artery Sarcoma Treatment for 17 Years a
 

Figure 4
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  		Fig 4. Five-year cumulative survival probability in the current case series (dotted line) vs a combined series (solid line) of all historical controls since 1990.

 
Treatment Results From Current Series
Eight patients underwent resection combined with chemotherapy or radiation therapy. Patients who were evaluated but did not undergo an operation are not included in this review. Patients were prospectively followed up, and data were recorded in our multi-institutional PA sarcoma database. The group comprised 7 men, and mean patient age was 54 years. All of the patients from the current series presented with a cough and dyspnea, and only 1 patient complained of pain. Patient demographic data from our current series are summarized in Table 2.

Radiographic evaluation in these patients included 8 with CT scan evaluation of the lesions, 6 with gadolinium-enhanced MRI, 2 with PET imaging, and 5 underwent coronary angiography. All tumors enhanced with gadolinium during MRI imaging and one of the two had increased FDG uptake compared with the background on PET imaging.

All patients had involvement of the main PA. Three patients had involvement of the pulmonary valve. Four patients had extensive lung involvement on one side of the chest. Two patients had angiosarcomas, 2 had leiomyosarcomas, and 1 patient each had a high-grade sarcoma (undifferentiated), spindle cell sarcoma, high-grade intimal sarcoma, or unspecified sarcoma.

All patients underwent median sternotomy and cardiopulmonary bypass for at least one of their procedures. Five patients had homograft replacement of the main PA, 3 required pulmonic valve replacement, and 5 underwent concomitant pneumonectomy. Local recurrence resulted in four repeat resections. The 30-day or hospital mortality was zero.

Two patients underwent neoadjuvant chemotherapy, and 6 patients underwent adjuvant chemotherapy. The therapy regimens ranged from Adriamycin (Pharmacia S.p.A, Milan, Italy) plus ifosfamide to gemcitabine plus taxane or dacarbazine regimens, and not enough patients had one type of chemotherapy regimen to determine any benefit. Sites of distant recurrence included the pancreas, adrenal gland, liver, small bowel, and brain. Median and 5-year survival was 71 months and 72.9% for the current series (Fig 4).

Treatment Results From Combined Analysis
When we combined our patients with the historical controls for analysis, the median and 5-year survival was 36.5 ± 20.2 months and 49.2% for patients undergoing a curative resection attempt compared with 11 ± 3 months and 0% for those undergoing tumor debulking, palliative pneumonectomy, exploration, or thromboendarterectomy (p = 0.000; Fig 5). Again, when the current and historical patients were combined, median and 5-year survival was 24.7 ± 8.5 months and 33.4% for those patient undergoing multimodality treatment compared with 8 ± 1.7 months and 30.6% for patients who had only one modality of therapy (p = 0.042; Fig 3). There was no statistically significant difference in survival comparing the current patients having multimodality therapy with historical controls from the literature.


Figure 5
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  		Fig 5. Five-year cumulative survival probability in the current case series plus other historical cases undergoing resection (dotted line) in the past 17 years vs a combined series of historical controls (solid line) undergoing tumor debulking, palliative pneumonectomy, or thromboendarterectomy.

 
Treatment Recommendations
The aggregated review of current and historical patients led to several recommendations for surgical evaluation and treatment. Although imaging may be helpful, it is usually impossible to determine resectability without surgical exploration. Criteria for an operation include adequate cardiopulmonary reserve, lack of disease outside the chest, disease that is considered resectable, and adequate lung function reserve if pneumonectomy is considered. Patients not considered candidates for a curative resection may also benefit from palliative resection, but consideration for neoadjuvant therapy before resection in all cases is recommended. This may permit tumor shrinkage, enhance resectability, and potentially neutralize micrometastatic disease. For an algorithm for initial evaluation of the patient with an intrapulmonary mass, please refer to Figure 1.

Resection of the full extent of the tumor is recommended. Frozen specimen analysis is useful to determine completeness of radial, proximal, and distal resection margins. If excision of the pulmonic valve is necessary for an Ro resection, then a homograft can be used for reconstruction. All patients should have at the minimum a CT scan to evaluate the extent of tumor and plan resection. Intraoperative frozen specimens can be obtained to confirm malignancy. Intraoperative transesophageal echocardiography is routinely used in our institution. Patients may benefit from resection even if a Ro resection cannot be achieved, but local recurrence should be expected.

The survival difference we have noted between patients undergoing complete resection vs incomplete resection, stenting, or palliative pneumonectomy leads us to recommend complete excision when possible. Beware of lung infarcts: The parenchyma may appear to have tumor involvement and instead have necrosis from embolization and lack of collateral blood supply to the lobe [2]. For cases that appear equivocal, transvenous suction catheter biopsy can be helpful [2]. We also noted a trend toward decreased survival in patients presenting with early lung parenchymal recurrence.

Surgical resection combined with neoadjuvant chemotherapy remains the therapy of choice for PA sarcomas. Unfortunately, many patients are not hemodynamically stable to undergo such therapy and will have adjuvant therapy instead. After reviewing 13 pathologic specimens, Burke and colleagues [15] reported a mean survival of 23 months. With resection alone, the mean survival ranges from a few days to 60 months [6, 18] in the current literature (Table 2). There appears to be no consensus regarding the benefit of adjuvant chemotherapy, with some in favor [5, 13, 20] and some against [48]. When a preoperative diagnosis can be made and the patient is stable, most agree to the clinical benefit neoadjuvant chemotherapy offers [20]. In the current review, patients undergoing a bimodality regimen appeared to have prolonged survival compared with those undergoing a single modality treatment regimen. On the basis of these data, our preference is to deliver neoadjuvant chemotherapy to patients who are stable and adjuvant therapy to patients who will not tolerate waiting for resection.

Surveillance
After resection, patients should have regular follow-up by a complete history, physical examination, and contrast-enhanced CT scan to detect early recurrence. Those patients with an intraluminal filling defect should have subsequent gadolinium-enhanced MRI to differentiate between tumor and thrombus. If thrombus is detected, they should remain on an anticoagulation therapy regimen until the defect resolves. If there is no resolution, then tumor should again be suspected and repeat resection should be considered. Please refer to Figure 6 for a postoperative evaluation algorithm that was developed at our own institution to eliminate unnecessary anticoagulation therapy for patients with new filling defects due to tumor recurrence after initial resection. Because one of our own patients was initially treated with anticoagulation therapy before being referred back to our center for repeat resection of a recurrent tumor, we have subsequently followed this algorithm for postoperative surveillance.


Figure 6
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  		Fig 6. Recommended schedule for primary pulmonary artery (PA) sarcoma postoperative evaluation. (CT = computed tomography; MRI = magnetic resonance imaging.)

 
Owing the rarity of this type and location of tumor, no prospective trials have evaluated these treatment regimens. Patients undergoing evaluation with a diagnosis of primary PA sarcoma have the opportunity to be included in our International Tumor Registry so that these small numbers of patients can be tracked to ensure optimal therapy can be determined. We used the limited survival data recorded by our own institution and others to develop a proposed PA sarcoma staging system (Table 4), in which stage I tumors are limited to the main PA, stage II tumors involve 1 lung plus a main PA, stage III tumors constitute bilateral lung involvement, and stage IV tumors present with extrathoracic spread. This staging system would require further validation by an International Tumor Registry with larger numbers of patients per category to test validation.


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  		Table 4 Proposed Staging System for Primary Pulmonary Artery Sarcomas
 

   Comment
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 Introduction
 Methods
 Comment
References
 
Primary PA sarcomas are rare tumors, with fewer than 250 cases reported. Although surgical resection is the standard of care, radical complete resection is rarely reported for tumors involving the main PA, pulmonic valve, or right ventricular outflow tract. In patients undergoing PA resection instead of thrombectomy, in conjunction with chemotherapy, our series reports a long median survival of 71 months. This improved survival is thought to be due to two factors: aggressive resection with a curative intent and multimodality therapy. Of the 8 patients in the current series, 5 are still living and 3 appear to have no evidence of disease to date. Radical PA resection in conjunction with chemotherapy is both safe and appears to improve survival.


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