A Rare Case of Primary Pulmonary Artery Myofibroblastic SarcomadImaging and Pathologic Features Ran Jing,1 Hao Tang,1 Kangjun Shen,1 Junming Luo,2,3 and Xinmin Zhou,1 Changsha City, People’s Republic of China, and Kansas City, Missouri

Primary myofibroblastic sarcomas of the pulmonary artery are very uncommon, but early detection is critical. Common clinical symptoms include shortness of breath, chest discomfort, and syncope. Patients diagnosed with a pulmonary tumor have a high risk of fatal pulmonary embolism. In this case study, we identified and diagnosed pulmonary artery myofibroblastic sarcoma in a young man by its imaging and pathologic characters. Surgery to excise the tumor was successfully performed and the operation was considered to have improved his prognosis. Postoperative examinations did not show any evidence of residual tumor, recurrence, or metastasis in the lungs or cardiac tissue. Based on the results of this case study, we concluded that the imaging and pathologic features of primary pulmonary artery myofibroblastic sarcoma can assist physicians in making a prompt diagnosis and an immediate surgical and treatment plan to greatly improve prognosis.

Primary cardiac tumors resulting in pulmonary artery embolism are extremely rare, with a prevalence of 0.0001e0.0003%.1 As previously reported, the most common primary tumors of the heart include the myxoma or other tumors that are malignant in nature.1e3 Patients diagnosed with a pulmonary tumor have a high risk of fatal pulmonary embolism. We report the prompt diagnosis of and successful surgery on a young man with a rare case of primary pulmonary artery myofibroblastic sarcoma. We have also reviewed other reported cases and discussed alternative therapies for diagnosed patients. 1 Cardiology Department, Xiangya Hospital of Central South University, Changsha City, People’s Republic of China. 2

Veterans Affairs Medical Center at Kansas City, Kansas City, MO.

3

Pathology Department, Xiangya Second Hospital of Central South University, Changsha City, People’s Republic of China. Correspondence to: Xinmin Zhou, MD, Cardiothoracic Surgery Department, Xiangya Second Hospital of Central South University, 139 Renmin Road, Changsha City, Hunan Province 410011, People’s Republic of China; E-mail: [email protected] Ann Vasc Surg 2014; 28: 491.e13–491.e17 http://dx.doi.org/10.1016/j.avsg.2012.10.036 Ó 2014 Published by Elsevier Inc. Manuscript received: July 23, 2012; manuscript accepted: October 17, 2012; published online: December 18, 2013.

CASE REPORT An 18-year-old man was admitted to Xiangya Hospital of Central South University, Changsha City, People’s Republic of China. The patient had no family history of cardiac disease. He reported a progressive shortness of breath over a 1-month period with a subsequent episode of syncope, without any obvious cause. Then he began to show dyspnea during exercise, such as running. It seemed that his dyspnea did not relate to syncope or cough, but it would improve when he lied down. This symptom was getting worse in the last month. His breath sounds were attenuated on the right side and rhonchi could be heard in both lungs; however, he did not have a cough and his body temperature was normal. The pulmonary component of the second heart sound was absent on auscultation. The physical examination revealed neither jugular vein distention nor pedal edema. Together, these diagnostic examinations showed us that the pulmonary mass looked like a solid tumor. We did not see any liquid region on the transthoracic echocardiography (TTE) or computed tomography (CT) images, which indicated to the surgeon that the tumor was not an abscess. The patient’s full blood cell count and biochemical examinations were normal, but an electrocardiogram (ECG) revealed that his right ventricle was hypertrophic. Both CT and positron emission tomography (PET) scans indicated an obstruction of the right

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Fig. 1. Digital subtraction angiography (DSA) reveals a filling defect indicating the location and morphology of the tumor (A and B). A small aperture between the tumor and the pulmonary artery wall can be seen.

A computed tomography scan reveals the tumor (highlighted) in the pulmonary trunk and its bifurcation. The pulmonary artery is dilated and obstructed (C and D).

ventricular outflow tract, pulmonary trunk, and pulmonary bifurcation, caused by a single tumor (Figs. 1 and 2). CT images also revealed that his right lung was only 50% of normal volume. Digital subtraction angiography (DSA) revealed a small aperture between the tumor border and the pulmonary artery wall, which allowed for blood flow (Fig. 1). However, to investigate the patient’s cardiac function, TTE was subsequently performed. Both the right ventricle and the right atrium were dilated (43 mm and 41 mm, respectively). Although the aortic diameter was normal (20 mm), the pulmonary diameter was dilated to 29 mm. Mean pulmonary arterial pressure was also significantly increased to 20 mm Hg. The patient was diagnosed with pulmonary artery myofibroblastic sarcoma and was at a high risk of fatal pulmonary artery embolism. It was decided that the patient’s heart function was sufficient to undergo an operation, so an emergency surgery was carried out to excise the tumor. Under moderate hypothermic circulatory conditions (28 C), a median sternotomy incision was made to expose the tumor, which had a pedicle (5  5 mm) that was attached to the lateral pulmonary valve by its capsule. The incision exposed the tumor from the pulmonary valve to bifurcation of the pulmonary trunk. One side of the tumor was extended along the pulmonary artery and

bifurcation without adhesion. The other side was extended into the right ventricular infundibulum. It appeared that the tumor had arisen from the lateral pulmonary valve, and that the tumor’s intact capsule had restricted the tumor size and movement into the pulmonary artery. The tumor did not invade the lungs or cardiac tissue, and there was no adhesion or lymphatic metastasis. The tumor was excised approximately 5 mm around its pedicle and extracted from the pulmonary trunk (Fig. 3). Postoperative TTE revealed mild pulmonary regurgitation. After surgery, the size of the right ventricle and the right atrium decreased considerably (33 mm and 31 mm, respectively). The pulmonary diameter was also reduced to 21 mm; however, the mean pulmonary arterial pressure remained high at 25 mm Hg. Histologic classification of the tumor was a prerequisite in the recommendation of further treatment for the patient. Hematoxylineeosin staining revealed an abundance of spindle tumor cells, which were arraying in bundles and knitted, and that displayed an increased mitoses and cytoplasm ratio (Fig. 4A and B). Moreover, numerous immunohistochemical markers were examined on sections of the tumor using antibodies against Bcl-2, CD34, CD68, myogenin, S-100, cytokeratin, CD117, myoepithelial cells, vimentin, and actin. Of the markers analyzed, only vimentin and actin showed

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Fig. 2. The white arrow of the positron emission tomography and computed tomography images reveal that the tumor occupies the majority of the pulmonary artery

trunk (A and B). The cross point also shows that the tumor occupies part of the pulmonary bifurcation (C and D).

positive immunostaining (Fig. 4C and D), while other markers were all negative. Because of these pathologic findings, it was confirmed that the tumor was a myofibroblastic sarcoma arising from cells of mesenchymal origin. After surgery, the patient’s symptoms improved significantly and he was discharged 8 days later. Markers including carcinoembryonic antigen, carbohydrate antigen, a-fetoprotein, and carcinoma antigen 199 were 0.54 ng/mL, 2.74 ku/L, 0.92 ng/mL, and 6.0 ku/mL, respectively, after surgery. Therefore, bioimmunotherapy (hypodermic injection of interferona-2a 500 wu for 3 weeks) and low-dose radiotherapy (50 cGy each day for 10 days) were recommended to the patient.

this case study provides a detailed report of a young man who was diagnosed with pulmonary artery myofibroblastic sarcoma, with a view to assist in the early diagnosis and treatment of future cases. The patient did not have an extensive history of severe hypoxia or dyspnea, attributed to the findings that the tumor could move with the pulmonary valve that it was attached to, and that it did not adhere to the pulmonary artery wall, allowing for blood flow. However, physicians should be aware of the possibility of pulmonary embolism if a patient experiences progressive shortness of breath, hypoxia, unexplained syncope, and/or right heart failure. Emergency TTE aided physicians to investigate the patient’s cardiac function and pulmonary blood flow, and to subsequently diagnose pulmonary embolism at an early stage. DSA was initially performed to analyze the blood flow through the

DISCUSSION Although primary myofibroblastic sarcomas of the pulmonary artery are very uncommon,4,5

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Fig. 3. A specimen of the sarcoma excised along the pulmonary bifurcation and the right ventricular outflow tract shows an octopus-like morphology, with a size of 9 cm  10.5 cm.

pulmonary artery (Fig. 1A and B); however, this technique may not be necessary if noninvasive examinations can provide sufficient information regarding cardiac function and tumor location. Indeed, CT and PET identified the location of the tumor and allowed for imaging of the tumor in relation to its surroundings. CT and PET are therefore valuable tools to assist surgeons to decipher the optimal method of operation to relieve obstruction and avoid lethal pulmonary embolism.3,6 Although sarcomas may evolve by lymphatic or hematologic metastasis,6 no other foci was detected by CT and PET scanning (Figs. 1C and D and 2). In this particular case, the tumor did not invade the lungs; however, in instances where sarcomas are present in the lung, a pneumonectomy should be considered if the patient can tolerate surgery, in order to clear the pulmonary trunk obstruction.7 Because there is a possibility that the primary tumor could be myxoma,2 surgeons should be extremely careful when choosing their surgical approach. Open heart surgery may be a better alternative to avoid disintegration of the fragile tumor upon removal and to prevent lethal pulmonary embolism during interventional surgery. In summary, the imaging and pathologic features in this case of primary pulmonary artery myofibroblastic sarcoma were (1) it was a single tumor with no additional foci detected; (2) it had good mobility and did not adhere to the pulmonary artery, allowing for blood flow; and (3) it did not invade the pulmonary or cardiac tissue even though it had arisen from the lateral pulmonary valve.

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In agreement with other reported cases,4,7 this case study confirms that early detection of primary pulmonary sarcoma and prompt surgery are critical to relieve right ventricular obstruction and pulmonary hypertension and therefore improve prognosis. To maintain pulmonary blood flow and right ventricular function for surgery, short-term administration of sodium nitroprusside and oxygen could further improve prognosis. Primary pulmonary sarcoma usually arises from the pulmonary valve and may lead to valve stenosis, or it may extend into the pulmonary vasculature as seen in this case (Fig. 3).5,7 Reconstruction of the right outflow tract and protection of right ventricular function after tumor resection may therefore be a challenge for surgeons. If the tumor invades the right ventricular outflow tract or pulmonary tissue, artificial valved conduits, such as bovine jugular vein or homograft/heterograft, can be used for right ventricular outflow tract reconstruction under hypothermic circulatory arrest. Valved artificial patches can also be used for pulmonary reconstruction in adult patients. In case of requirement, valved bovine jugular vein conduits were prepared for the patient in this study. If the tumor invades the right ventricle, heartelung transplantation may be the only surgical approach; however, palliative surgery to relieve right ventricular outflow tract obstruction would still improve a patient’s quality of life if the tumor is too large to make a complete resection. The patient in this case study was monitored for 12 months postsurgery. He recovered well and reported a good quality of life. Postoperative examinations including CT and TTE did not show any evidence of residual tumor, recurrence, or metastasis in the lungs or cardiac tissue within the 12 months. In conclusion, although primary myofibroblastic sarcomas of the pulmonary artery are rare, early detection is critical. Once the diagnosis of the primary tumor has been established, especially where a large mass causes pulmonary obstruction, emergency surgery is highly recommended. Surgery can excise the tumor and recover lung perfusion rapidly to reduce morbidity and mortality. This study suggests that the imaging and pathologic features of primary pulmonary sarcomas can assist physicians in making a prompt diagnosis and an immediate surgical and treatment plan to greatly improve prognosis.

We thank Drs. Duo Li, Xiaobo Liao, and Di Jing, who kindly provided many wonderful suggestions for this manuscript.

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Fig. 4. Histologic hematoxylineeosin staining of the sarcoma at (A) 200 magnification and (B) 400 magnification. Immunohistochemical analysis of

tumor sections reveal positive staining for (C) vimentin and (D) smooth muscle actin (C and D: 400 magnification).

REFERENCES

4. Maruo A, Okita Y, Okada K, et al. Surgical experience for the pulmonary artery sarcoma. Ann Thorac Surg 2006;82:2014e6. 5. Hu XP, Xu JP, Liu NN. Primary pulmonary artery sarcoma: surgical management and differential diagnosis with pulmonary embolism and pulmonary valve stenosis. J Card Surg 2009;24:613e6. 6. Bloomberg RD, Butany JW, Cusimano RJ, Leask RL. Primary cardiac sarcoma involving the pulmonary artery and valve. Can J Cardiol 2003;19:843e7. 7. Awonuga AO, Shavell VI, Imudia AN, et al. Pathogenesis of benign metastasizing leiomyoma: a review. Obstet Gynecol Surv 2010;65:189e95.

1. Bakaeen FG, Reardon MJ, Coselli JS, et al. Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 2003;186:641e7. 2. Tempe DK, Dutta D, Minhas H, et al. A rare case of myxoma in the right ventricular outflow tract extending to the pulmonary artery. Ann Card Anaesth 2010;13:167e8. 3. Kumagai K, Sai S, Endo M, Tabayashi K. Right ventricular myxoma obstructing the pulmonary artery during early childhood. Gen Thorac Cardiovasc Surg 2008;56: 351e3.

A rare case of primary pulmonary artery myofibroblastic sarcoma--imaging and pathologic features.

Primary myofibroblastic sarcomas of the pulmonary artery are very uncommon, but early detection is critical. Common clinical symptoms include shortnes...
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