Primary pulmonary artery sarcoma extending retrograde into the superior vena cava

Correspondence Primary Pulmonary Artery Sarcoma Extending Retrograde into the Superior Vena Cava

This letter was referred to Drs. José Portillo-Sanchez and Yasser Hessein-Abdou, who reply in this manner:

To the Editor: We are writing in regard to the article by Portillo-Sanchez and colleagues.1 Our group has a strong interest in cardiac sarcoma; we have operated on 10 patients with primary pulmonary artery sarcoma and have reported the first 8 of those.2 We have never seen a pulmonary artery sarcoma grow retrograde into the right ventricle— indeed, all have grown distally, within the pulmonary artery trunks. This case resembles the clinical and pathologic presentation of intravenous leiomyomatosis. This entity begins in the uterine veins and can occur long after a hysterectomy. It can occupy the entire vena cava, extending contiguously into the right heart, or it can break off and present as a right atrial tumor, extending into the pulmonary artery. These tumors tend to have a coiled appearance much like the one shown in the article and might be free within the vessel or attached. An intravenous leiomyomatosis tumor resembles a leiomyoma but can have bizarre nuclei and has been mistaken for low-grade leiomyosarcoma.3 It is thought to be a variant of malignant perivascular epitheloid cell tumor (PEComa). Pelvic imaging, particularly of the left uterine veins, would be useful in this case. We congratulate the authors on a well-presented case and successful surgical outcome. We maintain a pulmonary artery sarcoma registry and would be very interested in reviewing the pathology of this tumor with the authors.

To the Editor: In regard to the comment of Drs. Blackmon and Reardon, we appreciate their interest in our case.1 Pulmonary artery intimal sarcoma is a rare, highly fatal disease, and retrograde extension into the right ventricle is an extremely rare manifestation of the condition. Retrograde extensions have been reported before.2,3 In our patient, the retrograde growth of the tumor was confirmed during surgery: the tumor was attached to the pulmonary artery wall, with retrograde extension into the right-sided cavities. We sent biopsy material to 2 independent pathology laboratories, which concluded that the primary tumor



Fig. 1 Subintimal proliferation of cells in a focally myxoid stroma (H & E, orig. x100).

Shanda H. Blackmon, MD, Michael J. Reardon, MD, Department of Cardiovascular Surgery, The Methodist Hospital, Houston, Texas

References 1. Portillo-Sanchez J, Hessein-Abdou Y, Puga-Alcalde E, PerezMartinez MA, Del Carmen Jimenez-Meneses M, CamachoPedrero A, Valdepenas-Herrero L. Primary pulmonary artery sarcoma extending retrograde into the superior vena cava. Tex Heart Inst J 2011;38(1):77-80. 2. Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009;87(3):977-84. 3. Vural C, Ozen O, Demirhan B. Intravenous lipoleiomyomatosis of uterus with cardiac extension: a case report. Pathol Res Pract 2011;207(2):131-4.

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Fig. 2 High-grade sarcoma with a high mitotic index (H & E, orig. x400).

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patient was approximately 21 months from the onset of symptoms. Necropsy was proposed but not accepted. We hope that this information is useful. If Drs. Blackmon and Reardon need more information, we will be happy to provide it. If they wish, we are willing to send them the slides of the histopathologic preparations. Fig. 3 The endothelial cells that line the tumor are seen to be positive for CD31, while the tumor cells themselves are negative.

was vascular-based undifferentiated intimal sarcoma. The biopsy material displayed highly atypical spindle cells, nuclear polymorphism, and mitosis (Figs. 1 and 2). Immunohistochemical analysis was positive for vimentin and for CD34 and CD31 cell markers (Fig. 3), while negative for S-100, smooth-muscle actin, and desmins. Unfortunately, systemic metastases developed recently, and the patient died. The total survival time for this

Value of Cardiac Magnetic Resonance in Diagnosing Takotsubo Cardiomyopathy

To the Editor: I have read with great interest the article by Korlakunta and colleagues 1 in the Texas Heart Institute Journal regarding the diagnostic value of cardiac magnetic resonance in takotsubo cardiomyopathy. The main difficulty in diagnosing takotsubo cardiomyopathy lies in distinguishing it, in some patients, from ST-segment– elevation myocardial infarction (STEMI). In patients with takotsubo cardiomyopathy that presents with chest pain, ST-segment elevation, and elevated levels of cardiac biomarkers (thereby mimicking STEMI), coronary angiography with ventriculography should be the diagnostic procedure of choice. There are 2 reasons for this: first, the consequences of missing a diagnosis of STEMI by far outweigh the risk of the procedure; and second, a negative angiogram can avert the side effects of unnecessary thrombolytic therapy. In third-world countries, thrombolysis is the mainstay of therapy for STEMI because it is less expensive than cardiac catheterization or (in many cases) because catheterization laboratories are simply not available. Texas Heart Institute Journal

José Portillo-Sanchez, MD, PhD, Internal Medicine Department; and Yasser Hessein-Abdou, MD, PhD, Cardiology Department; University General Hospital, Ciudad Real, Spain

References 1. Portillo-Sanchez J, Hessein-Abdou Y, Puga-Alcalde E, PerezMartinez MA, Del Carmen Jimenez-Meneses M, CamachoPedrero A, Valdepenas-Herrero L. Primary pulmonary artery sarcoma extending retrograde into the superior vena cava. Tex Heart Inst J 2011;38(1):77-80. 2. Choi EY, Yoon YW, Kwon HM, Kim D, Park BE, Hong YS, et al. A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction. Yonsei Med J 2004;45(3):547-51. 3. Ozbek C, Emrecan B, Orgen Calli A, Gurbuz A. Intimal sarcoma of the pulmonary artery with retrograde extension into the pulmonic valve and right ventricle. Tex Heart Inst J 2007; 34(1):119-21.

In order to avoid missing a more serious diagnosis, it is advisable that patients who present with ST elevation and elevated cardiac biomarkers undergo coronary angiography to rule out ischemia, even if electrocardiographic and echocardiographic findings are more suggestive of takotsubo cardiomyopathy. Noninvasive imaging, including cardiac magnetic resonance, can be reasonable to confirm a suspected diagnosis of takotsubo cardiomyopathy in more stable patients who do not show ST elevation and elevated cardiac biomarkers. In conclusion, takotsubo cardiomyopathy should be a diagnosis of exclusion in patients who present with ST-segment elevation and elevated levels of cardiac biomarkers. Hesham R. Omar, MD, Internal Medicine Department, Mercy Hospital & Medical Center, Chicago, Illinois

References 1. Korlakunta H, Butkevich A, Muthupillai R, Cheong BYC. Biventricular takotsubo cardiomyopathy: cardiac magnetic resonance imaging as useful diagnostic tool. Tex Heart Inst J 2011;38(1):88-9.

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Spontaneous Pneumopericardium as the First Indication of an Intrapericardial Bronchogenic Cyst

To the Editor: We read with great interest the case presented by Peruz­ zi and colleagues 1 and would like to make a few comments. The authors successfully managed a case that involved a large pericardial effusion accompanied by pneumopericardium, with initial signs of diastolic compression of the heart. This was subsequent to an angioplasty procedure and the successful implantation of 2 bare-metal stents. Pneumopericardium is a rare, life-threatening condition that can compromise cardiac function. It can be observed as an iatrogenic sequela of numerous surgical interventions, or it can arise from natural intrathoracic causes. Other than cardiac surgery, the causes of pneumopericardium are as various as acute asthma,2 thoracic trauma, mechanical ventilation (in neonates), fistula formation from esophageal or gastric ulcers, and rupture of bronchogenic cysts. In the report by Peruzzi and colleagues, we believe that, in the absence of coronary artery perforation, an intrapericardial bronchogenic cyst is the most probable explanation for pericardial fluid in the presence of pneumopericardium. Rupture of the cyst and the ensuing inflammatory response possibly led to the large pericardial effusion and to imminent cardiac tamponade. Bronchogenic cysts arise from an abnormal budding of the ventral diverticulum of the foregut or the tracheobronchial tree during embryogenesis. They may arise from many locations, depending upon the time of their formation during embryogenesis, including the pericarinal, paratracheal, and intrapulmonary regions, along the esophagus, and below the diaphragm. The heart is one of the atypical sites of such cysts.3-5 The incidence of bronchogenic cysts is approximately 6% to 15% of This letter was referred to Dr. Massimo Ricci, who replies in this manner: To the Editor: We read Dr. Karangelis’s letter concerning a possible explanation for the case of post-angioplasty pneumopericardium that we presented.1 As Dr. Karangelis and his associates say, bronchogenic cysts are very rare malformations that can be completely asymptomatic or can present with unusual signs or symptoms.2 Cardiac manifestations include pneumopericardium and atrial fibrillation.3 Our patient did not undergo computed tomographic scanning of the chest, because he never referred (before coronary angioplasty) to any symptoms that would indicate the need for such testing. 322

primary mediastinal masses.4 Although they most often are detected incidentally upon routine chest radiography, they can become symptomatic: an intrapericardial bronchogenic cyst can present with symptoms of chest pain, shortness of breath, and compression of the heart and vessels, depending upon the location of the cyst and its size.5 That bronchogenic cysts can cause cerebral air embolism, pneumomediastinum, and pneumopericardium has been documented.6 Perhaps, in the documentation of this patient’s case, there exists a pre-procedural computed tomogram or magnetic resonance image that could confirm or disprove our hypothesis. In any event, we think it important to consider intrapericardial bronchogenic cyst in the patient’s differential diagnosis.

Dimos Karangelis, MD, PhD, Georgios I. Tagarakis, MD, PhD, Georgia Kalafati, MD, PhD, Nikolaos B. Tsilimingas, MD, PhD, Department of Cardiovascular & Thoracic Surgery, University Hospital of Larissa, Thessaly, Greece

References 1. Peruzzi M, Frati G, Rose D, Chirichilli I, Santo C, Ricci M. Spontaneous pneumopericardium after coronary angioplasty. Tex Heart Inst J 2010;37(6):728-9. 2. Agarwal MP, Giri S, Jain R, Sharma V. Spontaneous pneumopericardium in acute asthma. Int J Emerg Med 2010;3(2):141. 3. Gomes MN, Hufnagel CA. Intrapericardial bronchogenic cysts. Am J Cardiol 1975;36(6):817-22. 4. Kobza R, Oechslin E, Jenni R. An intrapericardial bronchogenic cyst. Interact Cardiovasc Thorac Surg 2003;2(3):27980. 5. Prates PR, Lovato L, Homsi-Neto A, Barra M, Sant’Anna JR, Kalil RA, Nesralla IA. Right ventricular bronchogenic cyst. Tex Heart Inst J 2003;30(1):71-3. 6. Jung S, Wiest R, Figerio S, Mattle HP, Hess CW. Cerebral air embolism caused by a bronchogenic cyst. Pract Neurol 2010; 10(3):164-6.

Had a bronchogenic cyst been present, we doubt that our patient’s symptoms would have disappeared so suddenly after pericardial drainage. Moreover, we did not observe the onset of mediastinitis or any other inflammatory reaction.

Massimo Ricci, MD, Department of Cardiac Surgery, University of Rome Sapienza, Rome, Italy

References 1.

Peruzzi M, Frati G, Rose D, Chirichilli I, Santo C, Ricci M. Spontaneous pneumopericardium after coronary angioplasty. Tex Heart Inst J 2010;37(6):728-9.

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2. Takeda S, Miyoshi S, Minami M, Ohta M, Masaoka A, Matsuda H. Clinical spectrum of mediastinal cysts. Chest 2003; 124(1):125-32. 3. Magro A, Micallef J, Falzon S, Agius M, Busuttil W, Montefort S. An unusual cause of chest pain in a young man: bronchogenic cysts and their cardiac manifestations. J R Coll Physicians Edinb 2010;40(1):29-32.

Texas Heart Institute Journal

Letters to the Editor should be no longer than 2 double-spaced typewritten pages and should contain no more than 6 references. They should be signed, with the expectation that the letters will be published if appropriate. The right to edit all correspondence in accordance with Journal style is reserved by the editors.

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