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Cardiac Chromaffin Cell Pheochromocytoma

Editor: Roopma Wadhwa Updated: 4/17/2023 4:43:48 PM


Cardiac pheochromocytomas (CPs) are very rare primary neuroendocrine catecholamine-secreting tumors that arise from the sympathetic paraganglia, which is made up of chromaffin cells. Paraganglia are non-neuronal cells that originate from the neural crest. The incidence of primary cardiac tumors ranges from 0.01 to 0.3% by autopsy.[1]. Thus far, CP is the rarest form of primary cardiac tumors. Nearly 98% of pheochromocytomas are found in the abdomen, with the adrenal gland being the most common site).[2] Less than 2% of pheochromocytomas are found in the chest, with the heart being the least common site. Most of the reported cases of CP are benign.[2] Although malignant pheochromocytomas are uncommon, the ones arising from the heart appear to be more aggressive and more difficult to resect. In clinical data of a small sample including 158 patients, of which 137 having detailed imaging done, 85% (117/137) were found to have an intra-pericardiac tumor that either arose from branchiomeric paraganglia (in the roots of the great vessels including pulmonary artery, pulmonary vein, vena cava, and aorta), or visceral autonomic paraganglia (interatrial or interventricular groove).[2][3][2] CP is an extremely vascular tumor that mostly obtains its blood supply from the coronary circulation (right coronary artery > left circumflex artery > left coronary artery > left anterior descending artery).[2][4][5]


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Although there is no clear data available, recent reports showed that genetic mutations and familial etiology contribute up to 25% to 50% of pheochromocytomas.[4] In the literature review, one case of cardiac pheochromocytoma associated with multiple endocrine neoplasia (MEN) was reported [6]. Other genetic syndromes have been found to be associated with pheochromocytomas in general, [4][7] including Multiple Endocrine Neoplasia type 2 (RET gene), neurofibromatosis 1 (NF1), Von Hippel-Lindau disease (VHL gene), adrenocortical adenoma and/or endocrine tumors as in Carney’s triad and paraganglioma syndromes type 1, 3, and 4 (SDH gene). Thirty percent (30%) of patients with malignant adrenal pheochromocytomas and forty-eight percent (48%) of patients with malignant paragangliomas (extra-adrenal pheochromocytomas) are found to have germline mutations of succinate dehydrogenase subunit B (SDH).[4] The CPs can also be associated with the above syndromes.


Cardiac pheochromocytomas (CPs) represent the rarest form of primary cardiac tumors. The incidence of primary cardiac tumors ranges from 0.01 to 0.3% by autopsy.[1] Only less than 2% of pheochromocytomas are found in the chest, and the CP is the least common of all. Less than 300 patients with CP are described in the PubMed database.[2] The mean age at diagnosis is 40 years old (ranges from 12 to 85 years) with equal distribution in both genders, although data is limited. In general, catecholamine-secreting tumors occur less than 0.2% of patients with hypertension.[8]


Cardiac pheochromocytomas are catecholamine secreting tumors that arise from chromaffin cells of the sympathetic paraganglia in the heart. They are often characterized by excessive, episodic catecholamine production and/or secretion (like epinephrine, dopamine, and norepinephrine) that can lead to different signs and symptoms including hypertension, sweating, headache, dizziness, perspiration, palpitations, and tachycardia.[2]


Macroscopically, CP are highly vascular tumors with well-defined hemorrhagic cut surfaces and rarely have an obvious pedicle.[2] Usually, these tumors are highly adjacent to the surrounding tissues without direct invasion. However, extensive myocardial infiltration may also be noted. In a report of 14 patients, the mean weight of the tumor was 76 gram (ranges, 12.5 to 137 gram). Their maximal diameter ranged from 1.5 to 15 cm (mean diameter 5.3).

Histologically, like most of the paragangliomas, this tumor is mainly composed of chief cells grouped in clusters or organoid (“Zellbellen”) fashion with surrounding capillary networks. Pigmented CPs were also reported in a few patients.[2][9]

Malignancy diagnosis is mainly based on metastasis rather than histologic features.

History and Physical

The most common presentation of cardiac pheochromocytomas is with catecholamine-related signs and symptoms[2] including palpitations, sweating, diaphoresis, hypertension, tachycardia, dizziness, headache, syncope, perspiration, and flushing. Unlike other primary cardiac tumors, shortness of breath is uncommon. Also although uncommon, some patients may present with chest pain. The likely explanation of the chest pain is that the tumor itself may mimic angina if it does not get enough blood supply. Sometimes initial presentation can be nonspecific with fever, fatigue, and weight loss. These tumors are rarely asymptomatic.


Once there is a suspicion of a pheochromocytoma, one should keep in mind that 98% of these tumors are intra-abdominal (90% in the adrenal medulla), and less than 2% are found in the chest. Of these in the chest, CP is the rarest one.

Biochemical Testing

  • Cardiac pheochromocytoma can be confirmed or excluded with biochemical testing by using serum and urine metanephrines.[10] Plasma metanephrines have sensitivity and specificity of 99% and 89%, respectively, while urine metanephrines have sensitivity and specificity of 97% and 93%, respectively. Although less accurate compared to metanephrines, urine and total serum catecholamines can be obtained as well.
  • Once pheochromocytoma is diagnosed with chemical testing, then the tumor has to be localized. Since the abdomen, especially the adrenal gland, is the most common site, intra-abdominal imaging should be done to rule out intra-abdominal pheochromocytoma.

Tumor Localization

The most difficult part of this tumor is not diagnosing it, but localizing it. There is a number of imaging studies (including but not limited to CT or enhanced CT, PET scan, MRI, echocardiogram, labeled metaiodobenzylguanidine (MIBG), or Tc labeled octreotide) that can be used. The most important of those studies will be reviewed below.[11][12][13][14][3]

  • Iodine labeled metaiodobenzylguanidine (MIBG) is the most effective method to screen for cardiac pheochromocytomas. It is very useful to identify the tumor and also detect the presence of metastasis.
  • Echocardiogram: transthoracic echocardiogram (TTE) is less sensitive than transesophageal echocardiogram (TEE) as tumors are often retro-pericardiac. When the tumor is firmly and extensively adherent to the pericardium, both of them are less sensitive.
  • Magnetic Resonance Imaging (MRI): cardiac MRI is very sensitive compared to CT scan and echocardiogram for localization. It can also identify the relation of the tumor to the surrounding structures.
  • Cardiac angiogram (cardiac catheterization): This is indispensable pre-operative imaging of CP. It can localize both the tumor and the feeding vessels.

In summary: MIBG, MRI, and coronary angiogram are necessary imaging modalities for tumor localization after laboratory confirmation and they should be pursued before patient is scheduled for surgical intervention.

Treatment / Management

The most effective treatment for cardiac pheochromocytomas is surgical resection.[15][16][17] However, blood pressure has to be controlled pre-operatively. Blood pressure normalization can be achieved with alpha-blockers (doxazosin, urapidil, phenoxybenzamine) initiated 2 to 3 weeks prior to the surgery. Other medications that can be used are calcium channel blockers (like amlodipine, nifedipine) and angiotensin-converting enzyme inhibitors.[18] It is very important to avoid using beta-blockers until good blood pressure control with alpha-blockers has been achieved. Isolated beta-blocker use can cause a catecholamine storm and hypertensive crisis.[18] It is recommended to start beta-blockers at least a few days prior to surgery to control heart rate. There is no known role for chemoradiation.[4](B3)

Differential Diagnosis

In general, differential diagnosis of pheochromocytomas is broad, and may include but not be limited to adrenal pheochromocytomas, hyperthyroidism, thyroid storm, cardiac arrhythmias, carcinoid tumor, migraine, stroke, medullary thyroid carcinoma, mastocytosis, etc.

In patients with a confirmed cardiac mass, the differential diagnosis may include pericardial mass, abscess, or metastasis.

Surgical Oncology

The most effective treatment of cardiac pheochromocytomas is surgical resection[19]


The one- year and 5- year survival rate in a report of 91 patients who had benign CP were 98.2% and 78.8%, respectively.[2][20][21] Although data is limited, overall prognosis and recovery are quite good after complete resection.[4] For malignant CP, the prognosis is not clear yet, as not enough data is available.


Complications can be related to either the disease itself or the procedure. Since it is a catecholamine-secreting tumor, if not treated, it can cause ischemic heart disease, myocardial infarction, cardiac arrhythmias, cardiomyopathy, pulmonary edema, stroke, and hypertensive crisis. The procedure is high risk. CP is a highly vascular tumor. In a report of 100 patients, 5 died due to massive intraoperative bleeding.[2]


A collaboration between Cardiology and Endocrinology would establish the initial diagnosis while Cardiothorasic Surgery would complete the surgical treatment. Other specialties should be involved in the overall patient care including Electrophysiology and Oncology specialists as needed.

Enhancing Healthcare Team Outcomes

An interprofessional approach including a cardiologist, a cardiothoracic surgeon, an endocrinologist, an oncologist, an electrophysiologist, an intensivist, an internist, and the nurse may be required for proper care and management of patients with CP. The overall prognosis is quite good after total resection.



Nomoto N, Tani T, Konda T, Kim K, Kitai T, Ota M, Kaji S, Imai Y, Okada Y, Furukawa Y. Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study. Journal of cardiothoracic surgery. 2017 Nov 28:12(1):103. doi: 10.1186/s13019-017-0672-7. Epub 2017 Nov 28     [PubMed PMID: 29183343]


Wang JG, Han J, Jiang T, Li YJ. Cardiac paragangliomas. Journal of cardiac surgery. 2015 Jan:30(1):55-60. doi: 10.1111/jocs.12455. Epub 2014 Oct 20     [PubMed PMID: 25331372]

Level 1 (high-level) evidence


Dong X, Meng X, Zhang T, Zhao L, Liu F, Han X, Liu Y, Zhu H, Zhou X, Miao Q, Zhang S. Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China. Frontiers in cardiovascular medicine. 2021:8():780382. doi: 10.3389/fcvm.2021.780382. Epub 2022 Jan 5     [PubMed PMID: 35071353]

Level 2 (mid-level) evidence


El-Ashry AA, Cerfolio RJ, Singh SP, McGiffin D. Cardiac paraganglioma. Journal of cardiac surgery. 2015 Feb:30(2):135-9. doi: 10.1111/jocs.12479. Epub 2014 Dec 22     [PubMed PMID: 25533017]

Level 3 (low-level) evidence


Alraddadi HO, Alsagheir A, Belley-Côté EP, McClure GR, Whitlock RP. Cardiac pheochromocytoma encasing the left main coronary artery. Journal of cardiac surgery. 2018 Apr:33(4):176-178. doi: 10.1111/jocs.13560. Epub 2018 Mar 24     [PubMed PMID: 29573472]


Zhang C, Ma G, Liu X, Zhang H, Deng H, Nowell J, Miao Q. Primary cardiac pheochromocytoma with multiple endocrine neoplasia. Journal of cancer research and clinical oncology. 2011 Sep:137(9):1289-91. doi: 10.1007/s00432-011-0985-1. Epub 2011 Jun 26     [PubMed PMID: 21706326]

Level 3 (low-level) evidence


Amar L, Bertherat J, Baudin E, Ajzenberg C, Bressac-de Paillerets B, Chabre O, Chamontin B, Delemer B, Giraud S, Murat A, Niccoli-Sire P, Richard S, Rohmer V, Sadoul JL, Strompf L, Schlumberger M, Bertagna X, Plouin PF, Jeunemaitre X, Gimenez-Roqueplo AP. Genetic testing in pheochromocytoma or functional paraganglioma. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2005 Dec 1:23(34):8812-8     [PubMed PMID: 16314641]


Stein PP, Black HR. A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience. Medicine. 1991 Jan:70(1):46-66     [PubMed PMID: 1988766]


Mikolaenko I, Galliani CA, Davis GG. Pigmented cardiac paraganglioma. Archives of pathology & laboratory medicine. 2001 May:125(5):680-2     [PubMed PMID: 11300945]

Level 3 (low-level) evidence


Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, Keiser HR, Goldstein DS, Eisenhofer G. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002 Mar 20:287(11):1427-34     [PubMed PMID: 11903030]

Level 2 (mid-level) evidence


Degrauwe S, Monney P, Qanadli SD, Prior J, Beigelmann-Aubry C, Masci PG, Eeckhout E, Muller O, Iglesias JF. Intrapericardial paraganglioma: The role of integrated advanced multi-modality cardiac imaging for the assessment and management of rare primary cardiac tumors. Cardiology journal. 2017:24(4):447-449. doi: 10.5603/CJ.2017.0091. Epub     [PubMed PMID: 28831780]


Patrianakos AP, Iliopoulos D, Marketou M, Skalidis EI, Parthenakis FI. Cardiac Paraganglioma: Multimodality Imaging of a Rare Tumor. JACC. Case reports. 2021 Feb:3(2):273-275. doi: 10.1016/j.jaccas.2020.11.047. Epub 2021 Feb 17     [PubMed PMID: 34317516]

Level 3 (low-level) evidence


Duan Y, Xu R, Liu W, Cui X, Li K, Yang X, Shi Q, Cheng Z, Chen L. PET/CT in a patient with cardiac paraganglioma. The international journal of cardiovascular imaging. 2021 Apr:37(4):1473-1477. doi: 10.1007/s10554-020-02101-2. Epub 2020 Nov 20     [PubMed PMID: 33216251]


Gahremanpour A, Pattakos G, Reul RM, Mirzai-Tehrane M. Diagnostic Imaging and Treatment of a Left Atrial Paraganglioma. Texas Heart Institute journal. 2017 Aug:44(4):296-298. doi: 10.14503/THIJ-16-5749. Epub 2017 Aug 1     [PubMed PMID: 28878589]


Ceresa F, Sansone F, Rinaldi M, Patanè F. Left atrial paraganglioma: diagnosis and surgical management. Interactive cardiovascular and thoracic surgery. 2010 Jun:10(6):1047-8. doi: 10.1510/icvts.2009.231043. Epub 2010 Mar 2     [PubMed PMID: 20197349]

Level 3 (low-level) evidence


Sook M, Hamoir E, de Leval L, Duquenne S, Larbuisson R, Joris J, Meurisse M, Defraigne J, Radermecker MA. Cardiac paraganglioma: diagnostic work up and review of the literature. Acta chirurgica Belgica. 2012 Jul-Aug:112(4):310-3     [PubMed PMID: 23008998]

Level 3 (low-level) evidence


Yang T, Li Y, Zhang HM, Wang HY, Song YH. Resection of Cardiac Pheochromocytoma With Cardiopulmonary Bypass. The Annals of thoracic surgery. 2021 Mar:111(3):e153-e155. doi: 10.1016/j.athoracsur.2020.06.035. Epub 2020 Aug 20     [PubMed PMID: 32828749]


Pacak K. Preoperative management of the pheochromocytoma patient. The Journal of clinical endocrinology and metabolism. 2007 Nov:92(11):4069-79     [PubMed PMID: 17989126]

Level 3 (low-level) evidence


Zheng J, Li J, Li J, Zhong L, Xu S, Zhou H. Surgical Treatment of Primary Cardiac Paragangliomas: A Single-Center Experience. The heart surgery forum. 2021 Dec 14:24(6):E1023-E1026. doi: 10.1532/hsf.4255. Epub 2021 Dec 14     [PubMed PMID: 34962466]


Liu XP, Miao Q, Liu XR, Zhang CJ, Ma GT, Liu JZ. Outcomes of surgery for functional cardiac paragangliomas: A single-center experience of 17 patients. The Journal of thoracic and cardiovascular surgery. 2019 Apr:157(4):1556-1564. doi: 10.1016/j.jtcvs.2018.09.013. Epub 2018 Sep 29     [PubMed PMID: 30401531]


Ramlawi B, David EA, Kim MP, Garcia-Morales LJ, Blackmon SH, Rice DC, Vaporciyan AA, Reardon MJ. Contemporary surgical management of cardiac paragangliomas. The Annals of thoracic surgery. 2012 Jun:93(6):1972-6. doi: 10.1016/j.athoracsur.2012.02.040. Epub 2012 Apr 25     [PubMed PMID: 22537533]

Level 2 (mid-level) evidence