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Mediastinal Paraganglioma
Mediastinal Paraganglioma Stephanie N. Buchanan, MD, Kevin M. Radecki, MD, and Lowell W. Chambers, MD Department of Surgery, Mount Carmel West Hospital, Mount Carmel Health System, Columbus, Ohio
Paragangliomas of the mediastinum are rare, with only approximately 150 cases reported in the literature. Surgical excision is the treatment of choice; however, these tumors often lie near critical vascular structures. Here we present the case of a patient with a mediastinal paraganglioma discovered during a diagnostic procedure. (Ann Thorac Surg 2017;103:e413–4) Ó 2017 by The Society of Thoracic Surgeons
P
aragangliomas of the mediastinum are rare, with only approximately 150 cases reported in the literature. Most are slow-growing, hypervascular tumors, historically associated with high morbidity and mortality due to locally invasive growth to the heart, great vessels, esophagus, and trachea. Surgical excision is the treatment of choice with complete resection providing the best longterm outcome. Here we present the case of a patient with a mediastinal paraganglioma discovered during a diagnostic procedure. A 67-year-old woman presented to her primary care physician with fever, malaise, and sweats of approximately 5 months’ duration. Further evaluation with computed tomography scan revealed 4 4 cm hilar adenopathy (Fig 1). Endoscopic bronchial ultrasound at an outside facility proved nondiagnostic, yielding only bloody return, and she was subsequently referred to thoracic surgery. On initial clinical exam, she was found to be healthy appearing without any external signs of pathology. A mediastinoscopy was recommended to evaluate the mass. The differential diagnosis at the time of surgery included infection, lymphadenopathy, neoplasm, and metastasis. During the procedure, the large mass seen on computed tomography was identified. A biopsy was obtained with return of pulsatile, systemic red blood. The bleeding was controlled with direct pressure and packing, and a sternotomy was performed. The mass was identified as the source of bleeding, and it was noted to be between the undersurface of the transverse aortic arch at the division of the left, main, and right pulmonary arteries. An additional biopsy was obtained, and bleeding was controlled with direct pressure. The specimen demonstrated possible carcinoid on frozen pathology. Due to the extent of resection that would be required and without further informed consent, the sternum was closed, and the patient was taken to the
intensive care unit. The patient had an otherwise uncomplicated hospital course. Final pathology revealed nests of cells with round nuclei and absent mitotic figures, positive synaptophysin, chromogranin, and S-100 immunostaining with negative cytokeratin AE1/AE3 and CAM5.2 immunostaining, consistent with paraganglioma. Urine and plasma catecholamine and metanephrine tests were within normal limits. The patient was referred to a tertiary care center where she underwent a sternotomy, and the mass was discovered to be involving the posterior wall of the ascending aorta. She was placed on cardiopulmonary bypass, the mass was removed, and the posterior wall of the ascending aorta was patched. She did well in the postoperative period, and her presenting symptomatology was largely resolved. There was no evidence of recurrence of the paraganglioma on postoperative imaging at 3 months.
Comment Paragangliomas are rare neuroendocrine neoplasms accounting for less than 0.3% of mediastinal masses, more commonly occurring in the posterior mediastinum [1, 2]. These tumors are slow growing and rarely malignant. Most paragangliomas do not produce symptoms, however, they can secrete cathecholamines leading to symptoms such as sweating, headaches, tachycardia, and hypertension. The majority are asymptomatic and non–catecholamine-secreting tumors, but they can produce chest discomfort or neurological symptoms. These tumors can be identified on imaging studies, often located at the bifurcation of the great vessels [3]. Differential diagnosis includes lymphadenopathy, duplication cyst, pericardial cyst, tracheal lesion, aortic arch aneurysm, or enlarged pulmonary artery. Surgical resection is the preferred treatment modality, but this can be difficult due to proximity to the heart, great vessels, esophagus, and trachea [4]. Preoperative embolization to reduce perioperative bleeding
Accepted for publication Oct 10, 2016. Address correspondence to Dr Buchanan, MD, c/o Graduate Medical Education, 793 W State St, Columbus, OH 43222; email: sbuchanan@ mchs.com.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
Fig 1. Mediastinal mass located between the ascending aorta and the branch point of the pulmonary arteries. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.10.031
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CASE REPORT BUCHANAN ET AL MEDIASTINAL PARAGANGLIOMA
may be considered for these highly vascular neoplasms. Median sternotomy with cardiopulmonary bypass is often required to obtain complete resection [5, 6]. Chemotherapy and radiation are not standardized due to the rarity of the disease, but overall they are ineffective and their role is limited [7]. Paragangliomas can recur and lifelong follow-up after resection is recommended [8]. Up to 25% are hereditary and genetic testing should be considered in all patients diagnosed with paragangliomas [4]. With the extensive operation often required to remove these tumors, patients with high suspicion of paragangliomas should be evaluated at centers with more robust cardiac surgery resources. Preoperative planning should include preparing the appropriate equipment and staff for cardiopulmonary bypass and cardiac patching. At our hospital center, we have limited cardiopulmonary bypass capabilities, and we did not have the appropriate homograft for aortic repair; therefore, this patient’s definitive surgery was best performed at a tertiary care hospital. With appropriate preoperative planning, complete resection of mediastinal paragangliomas is achievable.
Ann Thorac Surg 2017;103:e413–4
References 1. Al-Jehani Y, Saleh W, Al Halees Z, Ashour M. Successful resection of a huge paraganglioma utilizing cardiopulmonary bypass. Asian Cardiovasc Thorac Ann 2012;20:482–5. 2. Paul S, Jain SH, Gallegos RP, Aranki SF, Bueno R. Functional paraganglioma of the middle mediastinum. Ann Thorac Surg 2007;83:e14–6. 3. Wald O, Shapira OM, Murar A, Izhar U. Paraganglioma of the mediastinum: challenges in diagnosis and surgical management. J Cardiothorac Surg 2010;5:19. 4. Parisinos CA, Carnochan FM, Gregoriades ML, Monaghan H, Patel D, Walker WS. Mediastinal paraganglioma: time to panic? BMJ Case Rep 2011;1:4. 5. Kim D, Kim SW, Hong JM. Mediastinal paraganglioma: complete resection using video-assisted thoracoscopic surgery. Korean J Thorac Cardiovasc Surg 2014;47:197–9. 6. Mehta CK, Gillespie CT, Lin X, Yeldandi A, Decamp M, Bharat A. Rare Middle mediastinal paraganglioma mimicking metastatic neuroendocrine tumor. Ann Thorac Surg 2015;100:702–5. 7. Matsumoto J, Nakajima J, Takeuchi E, Fukami T, Nawata K, Takamoto S. Successful perioperative management of a middle mediastinal paraganglioma. J Thorac Cardiovasc Surg 2006;132:705–6. 8. Ghouri MA, Krishnan E, Singh A, Zaman T, Hallman CH. Mediastinal paraganglioma between the great vessels in an 81-year-old woman. Tex Heart Inst J 2013;40:189–92.
Paragangliomas of the mediastinum are rare, with only approximately 150 cases reported in the literature. Surgical excision is the treatment of choice; however, these tumors often lie near critical vascular structures. Here we present the case of a patient with a mediastinal paraganglioma discovered during a diagnostic procedure. (Ann Thorac Surg 2017;103:e413–4) Ó 2017 by The Society of Thoracic Surgeons
P
aragangliomas of the mediastinum are rare, with only approximately 150 cases reported in the literature. Most are slow-growing, hypervascular tumors, historically associated with high morbidity and mortality due to locally invasive growth to the heart, great vessels, esophagus, and trachea. Surgical excision is the treatment of choice with complete resection providing the best longterm outcome. Here we present the case of a patient with a mediastinal paraganglioma discovered during a diagnostic procedure. A 67-year-old woman presented to her primary care physician with fever, malaise, and sweats of approximately 5 months’ duration. Further evaluation with computed tomography scan revealed 4 4 cm hilar adenopathy (Fig 1). Endoscopic bronchial ultrasound at an outside facility proved nondiagnostic, yielding only bloody return, and she was subsequently referred to thoracic surgery. On initial clinical exam, she was found to be healthy appearing without any external signs of pathology. A mediastinoscopy was recommended to evaluate the mass. The differential diagnosis at the time of surgery included infection, lymphadenopathy, neoplasm, and metastasis. During the procedure, the large mass seen on computed tomography was identified. A biopsy was obtained with return of pulsatile, systemic red blood. The bleeding was controlled with direct pressure and packing, and a sternotomy was performed. The mass was identified as the source of bleeding, and it was noted to be between the undersurface of the transverse aortic arch at the division of the left, main, and right pulmonary arteries. An additional biopsy was obtained, and bleeding was controlled with direct pressure. The specimen demonstrated possible carcinoid on frozen pathology. Due to the extent of resection that would be required and without further informed consent, the sternum was closed, and the patient was taken to the
intensive care unit. The patient had an otherwise uncomplicated hospital course. Final pathology revealed nests of cells with round nuclei and absent mitotic figures, positive synaptophysin, chromogranin, and S-100 immunostaining with negative cytokeratin AE1/AE3 and CAM5.2 immunostaining, consistent with paraganglioma. Urine and plasma catecholamine and metanephrine tests were within normal limits. The patient was referred to a tertiary care center where she underwent a sternotomy, and the mass was discovered to be involving the posterior wall of the ascending aorta. She was placed on cardiopulmonary bypass, the mass was removed, and the posterior wall of the ascending aorta was patched. She did well in the postoperative period, and her presenting symptomatology was largely resolved. There was no evidence of recurrence of the paraganglioma on postoperative imaging at 3 months.
Comment Paragangliomas are rare neuroendocrine neoplasms accounting for less than 0.3% of mediastinal masses, more commonly occurring in the posterior mediastinum [1, 2]. These tumors are slow growing and rarely malignant. Most paragangliomas do not produce symptoms, however, they can secrete cathecholamines leading to symptoms such as sweating, headaches, tachycardia, and hypertension. The majority are asymptomatic and non–catecholamine-secreting tumors, but they can produce chest discomfort or neurological symptoms. These tumors can be identified on imaging studies, often located at the bifurcation of the great vessels [3]. Differential diagnosis includes lymphadenopathy, duplication cyst, pericardial cyst, tracheal lesion, aortic arch aneurysm, or enlarged pulmonary artery. Surgical resection is the preferred treatment modality, but this can be difficult due to proximity to the heart, great vessels, esophagus, and trachea [4]. Preoperative embolization to reduce perioperative bleeding
Accepted for publication Oct 10, 2016. Address correspondence to Dr Buchanan, MD, c/o Graduate Medical Education, 793 W State St, Columbus, OH 43222; email: sbuchanan@ mchs.com.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
Fig 1. Mediastinal mass located between the ascending aorta and the branch point of the pulmonary arteries. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.10.031
e414
CASE REPORT BUCHANAN ET AL MEDIASTINAL PARAGANGLIOMA
may be considered for these highly vascular neoplasms. Median sternotomy with cardiopulmonary bypass is often required to obtain complete resection [5, 6]. Chemotherapy and radiation are not standardized due to the rarity of the disease, but overall they are ineffective and their role is limited [7]. Paragangliomas can recur and lifelong follow-up after resection is recommended [8]. Up to 25% are hereditary and genetic testing should be considered in all patients diagnosed with paragangliomas [4]. With the extensive operation often required to remove these tumors, patients with high suspicion of paragangliomas should be evaluated at centers with more robust cardiac surgery resources. Preoperative planning should include preparing the appropriate equipment and staff for cardiopulmonary bypass and cardiac patching. At our hospital center, we have limited cardiopulmonary bypass capabilities, and we did not have the appropriate homograft for aortic repair; therefore, this patient’s definitive surgery was best performed at a tertiary care hospital. With appropriate preoperative planning, complete resection of mediastinal paragangliomas is achievable.
Ann Thorac Surg 2017;103:e413–4
References 1. Al-Jehani Y, Saleh W, Al Halees Z, Ashour M. Successful resection of a huge paraganglioma utilizing cardiopulmonary bypass. Asian Cardiovasc Thorac Ann 2012;20:482–5. 2. Paul S, Jain SH, Gallegos RP, Aranki SF, Bueno R. Functional paraganglioma of the middle mediastinum. Ann Thorac Surg 2007;83:e14–6. 3. Wald O, Shapira OM, Murar A, Izhar U. Paraganglioma of the mediastinum: challenges in diagnosis and surgical management. J Cardiothorac Surg 2010;5:19. 4. Parisinos CA, Carnochan FM, Gregoriades ML, Monaghan H, Patel D, Walker WS. Mediastinal paraganglioma: time to panic? BMJ Case Rep 2011;1:4. 5. Kim D, Kim SW, Hong JM. Mediastinal paraganglioma: complete resection using video-assisted thoracoscopic surgery. Korean J Thorac Cardiovasc Surg 2014;47:197–9. 6. Mehta CK, Gillespie CT, Lin X, Yeldandi A, Decamp M, Bharat A. Rare Middle mediastinal paraganglioma mimicking metastatic neuroendocrine tumor. Ann Thorac Surg 2015;100:702–5. 7. Matsumoto J, Nakajima J, Takeuchi E, Fukami T, Nawata K, Takamoto S. Successful perioperative management of a middle mediastinal paraganglioma. J Thorac Cardiovasc Surg 2006;132:705–6. 8. Ghouri MA, Krishnan E, Singh A, Zaman T, Hallman CH. Mediastinal paraganglioma between the great vessels in an 81-year-old woman. Tex Heart Inst J 2013;40:189–92.