Primary Cardiac Paraganglioma Research Articles

Multimodal cardiac imaging assisted tumor characterization and surgical planning of a patient with rare primary cardiac paraganglioma.

Multimodal cardiac imaging assisted tumor characterization and surgical planning of a patient with rare primary cardiac paraganglioma.

Surgical Treatment of Primary Cardiac Paragangliomas: A Single-Center Experience.

Cardiac paragangliomas are rare neuroendocrine tumors that will cause significant morbidity if left undiagnosed. Because of the paucity of cohort data, their rapid diagnosis and appropriate management still pose unique challenges to cardiac surgeons. We aimed to investigate the clinical features and surgical management of primary cardiac paragangliomas in our single center. From May 2014 to October 2020, patients diagnosed with primary cardiac paragangliomas retrospectively were reviewed. Demographic data, clinical presentation, preoperative imaging methods, surgical resection, perioperative management, histological analysis, and outcomes were recorded. Postoperative follow up also was reviewed. With multiple imaging methods, including echocardiography, computed tomography, positron-emission tomographic-computed tomography, and biochemical tests, there were five cases of primary cardiac paraganglioma verified by postoperative immunohistochemical staining, two of which were hormonally active. There were no metastatic cardiac paragangliomas, according to positron-emission tomographic-computed tomography, and all patients accepted surgical treatment. Preoperative adrenoceptor blockade was given to hormonally active patients, accordingly. Complete resection of the tumor was accomplished under cardiopulmonary bypass in each case. Tumor distribution included two masses on the roof of the left atrium, two masses in the right atrioventricular groove, and one between the ascending aorta and main pulmonary artery. Immunohistochemical staining for chromogranin, neuron-specific enolase, synaptophysin, and S-100 were positive, which were typical of cardiac paraganglioma. There were no operative deaths. All the patients had an uneventful recovery except one patient who underwent low cardiac output syndrome. During follow up (mean 4.2 years, range 0.6-7.0 years), all patients were well with New York Heart Association class I or II. Only one patient developed thyroid carcinoma three years after surgery but with no paraganglioma recurrence during periodic computed tomography, and this patient recovered well after thyroidectomy. Although cardiac paragangliomas are rare and may present surgical challenges for clinicians, surgical resection remains the choice of treatment with favorable outcomes through a multidisciplinary heart team. Moreover, lifelong surveillance still is recommended to detect possible recurrence or associated nonchromaffin tumors in time.

Commentary: What lessons can a surgeon learn from something rare?

Primary cardiac paragangliomas (PCPs) are rare neuroendocrine tumors of the heart. In this issue of the Journal, Chan and colleagues1 share their center's experience in managing patients with PCP, presenting a cohort of 19 patients with a perioperative mortality of 10.6%. The surgical steps are complex yet through experience and wise application of oncologic principles, this center is now a site of established excellence.

Management of primary cardiac paraganglioma.

Cardiac paraganglioma is a rare tumor that most surgeons have limited experience treating. The objective of this study is to examine the management and outcomes for cardiac paraganglioma treatment when cared for by a multidisciplinary cardiac tumor team. We reviewed our institutionally approved cardiac tumor database from March 2004 to June 2020 for cardiac paraganglioma. These prospectively collected data were retrospectively reviewed. Patient characteristics were presented for individual patients and as summary statistics. Demographic and clinical data were also reported as median and interquartile range for continuous variables and frequencies and proportions for categoric variables. Kaplan-Meier curves were used to depict the patient survival from surgery. There were 21 cases of primary cardiac paraganglioma, 19 of whom had surgical resection with 3 refusing offered surgery. Of 19 resected tumors, 13 originated from the left atrium and 6 originated from the roots of the pulmonary artery and the aorta. Complex procedures were required, including aortic and pulmonary root replacement and 8 autotransplants. All tumors had complete gross resection with no identifiable disease left behind, but 4 of these had microscopically positive margins. None of the patients had local recurrence of disease. There was 1 case of metastatic paraganglioma with death at 4years postsurgery. Operative mortality was 10.6%. Survival from surgery was 88.2%, 71.8%, and 71.8% and 1, 5, and 10years, respectively. Cardiac paraganglioma presents a surgical challenge. Mortality and long-term survival after surgical resection are acceptable but may require complex resection and reconstruction.

Primary unresectable locally invasive biatrial paraganglioma presenting with chest pain

Paragangliomas, neuroendocrine tumors of neural crest origin, commonly occur in the adrenal medulla where they are called pheochromocytoma. Primary cardiac paragangliomas are uncommon whereby they arise in the left atrium and, less frequently, right atrium. We present a 43-year-old female with a cardiac paraganglioma presenting with ischemic and positional chest pains. CT scanning showed an infiltrative 15×10cm unresectable tumor with intense peripheral enhancement and central hypoattenuation located above the right atrium. The clinical impression was that of a high-grade sarcoma. Histopathological examination revealed tumor cell nests in a distinctive ‘Zellballen’ pattern with positive synaptophysin, chromogranin and GATA-3 staining confirming the diagnosis of paraganglioma. Immunohistochemical studies revealed that the tumor lacked the expression of SDHB and therefore indicating a SDHB gene mutation. The tumor was nonfunctioning, and therefore the absence of the classic adrenergic manifestations contributed to the late diagnosis of the condition. In conclusion, whereas preoperative diagnosis is challenging with nonfunctional paragangliomas, it is recommended that paragangliomas be considered in the differential diagnosis of cardiac neoplasms.

Excision of a Sub-Coronary Artery Primary Cardiac Paraganglioma of the Right Atrioventricular Groove

Paragangliomas are neoplasms of neural crest origin arising from extra-adrenal chromaffin tissue. Less than 2% of all paragangliomas are located in the thoracic cavity with primary cardiac paragangliomas especially rare, typically originating from the left atrium. They are usually benign although malignancy can occur, commonly in association with hereditary pheochromocytoma-paraganglioma syndrome. Patients may present with symptoms related to either catecholamine excess or local compression however most are asymptomatic. Given their rarity, surgical experience with cardiac paragangliomas is limited, with simple excision, excision with reconstruction, autotransplantation and even orthotopic heart transplantation described. We present the case of a primary cardiac paraganglioma in an asymptomatic 38-year-old male with hereditary pheochromocytoma-paraganglioma syndrome and a family history of fatal malignant paraganglioma. The mass was located in the right atrioventricular groove beneath the right coronary artery (RCA) in juxtaposition with the right atrial appendage. Only three primary cardiac paragangliomas of the right atrioventricular groove have been reported previously. Given his family history, a decision for intervention was made after both the size of the mass on serial imaging and his serum catecholamines were noted to be increasing on follow-up. After median sternotomy, the neoplasm was enucleated with the use of cardiopulmonary bypass and cardioplegic arrest to allow for a still operative field. Multiple feeding vessels between the mass and the RCA were ligated with clips and diathermy. The postoperative course was uneventful with normalisation of his serum catecholamines and discharge day 5 postoperatively.

Extraadrenal Biatrial Cardiac Paraganglioma: Diagnosis, Histological Criteria and Surgical Management

Primary cardiac paragangliomas are rare heart tumors that originate from the neural crest. We report a case of successful surgical treatment of a noncatecholamine-producing cardiac paraganglioma in a 25-year-old white male patient with dyspnea on exertion and intermittent palpitations. Echocardiography revealed solid 4.6- × 4.3-cm tumor mass located in close relation to the left and right atria. A magnetic resonance image of the heart demonstrated the solid tumor mass adjacent mainly to the right atrium and in part to the left atrium with an early and strong arterial uptake of the contrast medium (see arrow in Fig 1).

Primary Cardiac Paraganglioma Arising from Interatrial Septum

Primary Cardiac Paraganglioma Arising from Interatrial Septum

Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma

About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an asymptomatic 49–year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management.

The Heart of the Matter

b l PRESENTATION In this case, a man with multiple cardiac symptoms suffers cardiac arrest during his medical evaluation, is revived, and is then treated for a very unusual tumor. The patient was a 46-year-old, previously healthy man who presented with complaints of shortness of breath and chest heaviness. Further inquiry revealed generalized fatigue and numerous episodes of profuse sweating over the preceding 2 months. A review of systems was otherwise negative.

Multimodality Evaluation of Cardiac Paraganglioma

We report 2 rare cases of primary cardiac paraganglioma demonstrated with multimodality imaging. Both a 46-year-old woman who presented with hypertension (case 1) and a 46-year-old woman without any symptoms (case 2) had mediastinal tumors depicted by CT and MRI. Both cases showed intense focal uptakes in the tumors with iodine-123-meta-iodobenzylguanidine SPECT. Case 2 also underwent 18F-FDG PET/CT and revealed a focal uptake. CT angiography detected the arterial feeder and venous drainages, information that was useful for the operation. After surgical removal, pathology confirmed primary cardiac paraganglioma in both cases. 18F-FDG PET/CT was also useful in ruling out residual or recurrent tumors.

Cardiac Paraganglioma: Diagnostic and Surgical Challenges

Primary cardiac paragangliomas are rare extra-adrenal tumors. Though they account for less than 1% of all primary cardiac tumors, they are considerable sources of morbidity and mortality. In this case review, we discuss the challenges associated with the diagnosis and management of cardiac paragangliomas.

Utility of Cardiac CT and MRI for the Diagnosis and Preoperative Assessment of Cardiac Paraganglioma

Cardiac paragangliomas are rare cardiac tumors that are usually benign. Surgical excision can be curative. We report a case of 39-year-old male who, during the work up of acute coronary syndrome with coronary angiography, cardiac computed tomography (CT) and magnetic resonance imaging (MRI), was found to have cardiac paraganglioma. The tumor was intrapericardial, arising at the level of proximal left anterior descending artery. The tumor was completely resected and the postoperative course was uneventful. At 3-months follow-up the patient was asymptomatic with normal ventricular function. Cardiac CT and MRI are valuable in characterizing and preoperative planning of primary cardiac paragangliomas.

Right atrial paraganglioma: an unusual primary cardiac tumour

We present a case of an unusual presentation of a rare primary cardiac tumour. There are no more than 50 previously reported cases of primary cardiac paraganglioma in the world literature and only a small proportion of these involve the right side of the heart. Diagnosis is difficult and surgical management is varied and complex.

A primary right atrium paraganglioma in a 15-year-old patient

Thoracic pheochromocytomas account for less than 2% of reported cases, while primary cardiac paragangliomas are even rare. The following case illustrates a 15-year-old patient with primary right atrium paraganglioma. This patient was referred for paroxysmal hypertension and excessive perspiration. Pheochromocytoma was suspected and then confirmed by very high serum nor-metanephrine which increased more than 30-fold above the upper limit of normal. 131I-metaiodobenzylguanidine (MIBG) scintigraphy showed high uptake only in the middle mediastinum, but not in the adrenal glands or elsewhere. Both contrast CT and gated MRI of the chest disclosed a 5.0 x 4.0 cm2 mass in the right atrium. Coronary angiography demonstrated the mass with feeding vessels from the right coronary artery. When the patient's blood pressure was well controlled with doxazosin and metoprolol, surgery was then performed. A 6.0 x 4.9 x 4.0 cm3 round solid right atrium paraganglioma weighing 41.7 g was resected. The second day after surgery, serum nor-metanephrine and urinary noradrenaline levels dropped rapidly to normal range, and the patient was free of clinical symptoms with normal BP. Postoperative cardiac function, as measured by echocardiogram, was normal. Although cardiac paraganglioma may be difficult to resect, it can be cured.