Cardiac Paraganglioma: Diagnostic and Surgical Challenges (original) (raw)
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Cardiac Paraganglioma—A Rare Subset of a Rare Tumor
World Journal of Cardiovascular Diseases, 2017
Background: Cardiac paraganglioma is a rare entity of an uncommon neuroendocrine tumor. Clinically, non-secreting tumors are often diagnosed because of their growth effects, secreting tumors present symptoms related to catecholamine. Correct diagnosis of a paraganglioma can be reached by biochemical investigations and imaging. Surgical resection is the treatment of choice and has to be planned carefully and interdisciplinarily. Aim: On the basis of a patient with a vague clinical presentation and an unclear situation after primary investigations, we highlight the diagnostic challenge of this rare subset of paragangliomas. Case presentation: We present the case of a 42year-old woman whose unspecific symptoms and further investigations revealed a paracardiac mass with unknown local behavior and dignity. Surgical resection and histopathological examination led to the diagnosis of a cardiac paraganglioma. Conclusion: Cardiac paragangliomas are extremely rare, but may be treated curatively by resection after careful preoperative investigation and interdisciplinary planning.
International journal of cardiology, 2013
Background: We describe our experience of the case of a young female who presented with chest pain and was found to have an intra-pericardial mass that was later confirmed to be a paraganglioma. Although preoperative magnetic resonance imaging (MRI) did not show any left atrial invasion, the patient died in the peri-operative period due to irreparable damage of the cardiac structures from extensive neoplastic invasion. We then performed a retrospective analysis of the available literature to find the factors associated with adverse surgical and long-term outcomes in patients with cardiac paraganglioma. Methods: We found 93 case reports of cardiac and/or pericardial paragangliomas in the literature. After exclusions, 82 cases were included in the final analysis. The patients were divided into two groups based on the outcome of surgical management. Univariate analysis was performed using SPSS software (Chicago, IL version 18), and the statistical significance was defined as a p-value b 0.05. Results and conclusions: The comparison of available demographic, clinical, pathological and laboratory parameters between the deceased and the surviving patients revealed that only the intra-cardiac location (p-value = 0.021) and the development of metastases (p-value b 0.001) were independently associated with increased surgical and long-term mortality, respectively. The size of a paraganglioma, its functional status or invasion into the surrounding structures does not appear to affect short-or long-term survival in these patients. The Kaplan-Meier survival curve showed excellent long-term prognosis for patients with a complete surgical removal of the neoplasm. Based on our experience, we also suggest preoperative imaging with a three dimensional cardiac CT and evaluation for cardiac transplantation before embarking on the surgical resection of these tumors.
A rare case of cardiac paraganglioma presenting as anginal pain: a case report
Cases Journal, 2009
Introduction: Primary cardiac paraganglioma is a very rare tumor with less than sixty reported cases in the literature. The clinical presentation is variable, but is most commonly manifested by hypertension and symptoms related to the catecholamine excess. Case Report: We report a case of a 35 year old man who presented with anginal pain and hypertension. He was found to have a cardiac mass on the computed tomographic scan and echocardiogram. He underwent surgical exploration of the mass which on biopsy was found to be a 'Cardiac Paraganglioma'. Surgical resection of the tumor was successfully done and the patient is doing well five years after the surgery without any evidence of recurrence. His blood pressure, however, failed to normalize and needed single agent antihypertensive therapy. Conclusion: Cardiac paragangliomas have a relatively favorable outcome if diagnosed and resected in time. We briefly review the literature regarding the diagnosis, treatment and prognosis of this rare tumor.
A Series of Two Patients With Cardiac Paragangliomas
AACE Clinical Case Reports, 2020
Objective: The objective of this report is to present 2 cases of cardiac paragangliomas (PGLs), and to outline the presentation, management, and associated genetic mutations. Methods: Case 1, a 38-year-old female, presented with a 12-month history of paroxysmal palpitations, headaches, and weight loss. Her investigations included plasma free metanephrines and urinary metanephrines, 68-gallium DOTATATE positron emission tomography/computed tomography, and cardiac imaging. Case 2, a 28-year-old male, presented with a hypertensive crisis and abdominal pain on a background of hypertension. Given his abdominal pain, he was investigated with an abdominal computed tomography (CT) scan, followed by plasma free metanephrines and urinary metanephrines, echocardiogram, and 123-iodine meta-iodobenzylguanidine single-photon emission CT. Results: Case 1 had an elevated plasma normetadrenaline of 6,750 pmol/L (reference range is <900 pmol/L) and 3-methoxytyramine of 1,845 pmol/L (reference range is <110 pmol/L). 68-gallium DOTATATE positron emission tomography/computed tomography showed an avid Abbreviations: CT = computed tomography; MRI = magnetic resonance imaging; PGL = paraganglioma; SDH = succinate dehydrogenase Plasma free metanephrines Reference range
Synchronous Presentation of Cardiac and Abdominal Paragangliomas
The Annals of Thoracic Surgery, 2012
A 28-year-old male presenting with a hypertensive crisis was found to have synchronous right atrial and retrocaval masses. Serum normetadrenaline was elevated in keeping with functional paragangliomas. After preoperative optimization both masses were successfully excised, including a saphenous vein graft to the right coronary artery. Serum catecholamines returned to the normal range postoperatively and all antihypertensive therapy was ceased.
Right atrial paraganglioma: an unusual primary cardiac tumour
European Journal of Cardio-thoracic Surgery, 2008
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. #