Mediastinal Paraganglioma: a challenge to the echocardiographic

Transcription

1 Case - based learning from ESC Cardiologists of Tomorrow Look for the answer outside the heart Mediastinal Paraganglioma: a challenge to the echocardiographic 1 diagnosis and endovascular treatment 1 On behalf of Sergio Raposeiras Roubín University Clinical Hospital of Santiago de Compostela. SPAIN RM. Agra Bermejo, J. Elices Teja, P. Cabanas Grandio, S. Gestal Romari, E. Pereira Lopez, B. Alvarez Alvarez, C. Gonzalez Cambeiro, R. Fandino Vaquero, A. Martinez Monzonis J.R.G. JUANATEY

2 Case Report 59 years old DIABETES MELLITUS ARTERIAL HYPERTENSION OBSTRUCTIVE SLEEP APNEA SYNDROME 2 episodes of SUPRAVENTRICULAR TACHYCARDIA (adenosine --- B-blockers) Ortopnea since 2 months J.R.G. JUANATEY

3 CHEST RADIOGRAPHY

4 TRANSTORACIC ECHOCARDIOGRAPHY

5 TRANSTORACIC ECHOCARDIOGRAPHY

6 TRANSTORACIC ECHOCARDIOGRAPHY

7 TRANSTORACIC ECHOCARDIOGRAPHY

8 TRANSTORACIC ECHOCARDIOGRAPHY

9 TRANSTORACIC ECHOCARDIOGRAPHY

10 TRANSESOPHAGEAL ECHOCARDIOGRAPHY J.R.G. JUANATEY

11 TRANSESOPHAGEAL ECHOCARDIOGRAPHY J.R.G. JUANATEY

12 TRANSESOPHAGEAL ECHOCARDIOGRAPHY J.R.G. JUANATEY

13 COMPUTED TOMOGRAPHY

14 CORONARY ANGIOGRAPHY

15 CORONARY ANGIOGRAPHY

16 CORONARY ANGIOGRAPHY

17 CORONARY ANGIOGRAPHY

18 CORONARY ANGIOGRAPHY

19 CORONARY ANGIOGRAPHY

20 CORONARY ANGIOGRAPHY

21 ECHO-ENDOSCOPY

22 PARAGANGLIOMA Catecho lamines J.R.G. JUANATEY

23 PARAGANGLIOMA 90% chromaffin-cell-originating tumors are located in the adrenal gland and termed pheochromocytomas. The remaining 10% are extra adrenal and are termed paragangliomas or chemodectomas Paragangliomas appear in the abdomen, pelvis, neck and mediastimun. Mediastinal paraganglioma are rare, originating from para-aortic (middle mediastinum) and para-vertebral (posterior mediastinum) sympathetic chain ganglia. Similar to pheochromocytoma, paraganglioma tumors may secrete catecholamines, however in majority of cases they are non-functional. The diagnosis of paraganglioma is based on clinical symptoms, imaging exams (echocardiography, computed tomography or magnetic resonance imaging), and biochemical tests. J.R.G. JUANATEY

24 PARAGANGLIOMA Up to 50% are asymptomatic and the diagnosis is incidental. Clinical symptoms may be related to catecholamine hypersecretion (hypertention / hyperhydrosis) or to a mass effect resulting in complains of hoarseness, dysphagia, shortness of breath and chest pain. Biochemical diagnosis of functional paragangliomas is made by measuring catecholamines and their metabolites in urine. The main imaging methods used for the diagnosis are CT and magnetic resonance imaging, both of which have a sensitivity of nearly 98%. Treatment of choice is complete surgical resection, which is difficult due to hypervascularization and anatomical juxtaposition of the tumor. Combined incision and extracorporeal circulation are often necessary. Due to the large number of small vessels in this area, which contains various major structures, hemostatic clips or a harmonic scalpel could be used in order to guarantee efficient hemostatic control. J.R.G. JUANATEY

25 PARAGANGLIOMA Mediastinal paragangliomas are rare, only approximately 150 cases having been reported in the literature, and two-thirds of these tumors are located in the anterior or middle mediastinum. Mediastinal paragangliomas are derived from the para-aortic and paravertebral ganglion chain. Multicentric tumors are observed in 23% of cases, and there seems to be no specific distribution in the remaining cases. Mediastinal paragangliomas are highly vascularized tumors that adhere to adjacent mediastinal structures, such as the heart, large blood vessels, trachea, and spine, and surgical management is therefore difficult. The differential diagnosis includes Castleman's disease, neuroendocrine tumors, lymphomas, and hemangiomas. J.R.G. JUANATEY

26 IMPLICATIONS TO CLINICAL PRACTICE Primary cardiac tumours are a rare disorder and have an incidence between 0.002% and 0.2% in an autopsy series. They are usually treatable and can often be diagnosed with echocardiography, computed tomography (CT), or magnetic resonance (MR) imaging. Benign tumours account for 75% of this total, being myxomas the most common. In general, 75% of myxomas are located in the left atrium. But cardiologist must note the differential diagnosis with other left atrial masses, as lipomas (16%), fibroelastomas (16%), hemangiomas (6%), fibromas (3%), rhabdomuomas (1%) and rare cases of neurofibromas, teratomas and paragangliomas. The Paragangliomas usually appear as large, echogenic left atrial masses at echocardiography and like a circumscribed, heterogeneous masses with low attenuation at CT. J.R.G. JUANATEY

27 DISCUSSION MEDIASTINAL Echocardiogrphic diagnosis PARAGANGLIOMA Endovascular treatment J.R.G. JUANATEY

28 Thanks for your attention J.R.G. JUANATEY