Location, localization and surgical treatment of cardiac pheochromocytoma

Aravot, Dan; Banner, Nicholas R.; Cantor, Abraham M.; Theodoropoulos, Stergios; Yacoub, Magdi H.

doi:10.1016/0002-9149(92)91324-w

“…MRI is a good imaging modality for the detection of intracardiac, juxtacardiac, and juxtavascular PHEO, because it reduces cardiac and respiratory motion-induced artifacts (102), whereas the use of T2 sequences enables better differentiation from surrounding tissues (104). MRI can be carried out with or without using iv contrast agents (which are nevertheless very safe and do not cause the release of catecholamines) (105)(106)(107), and thus no preparation with adrenergic blockade is necessary.…”

Section: Mri Imagingmentioning

confidence: 99%

Current Approaches and Recommended Algorithm for the Diagnostic Localization of Pheochromocytoma

Ilias

¹

,

Pacák

²

2004

The Journal of Clinical Endocrinology & Metabolism

View full text Add to dashboard Cite

Pheochromocytomas (PHEO) (1-3) are catecholamineproducing tumors that arise from chromaffin cells. PHEO are mostly situated within the adrenal medulla, although in about 9 -23% of cases, tumors develop from extraadrenal chromaffin tissue (adjacent to sympathetic ganglia of the neck, mediastinum, abdomen, and pelvis) and are often referred to as paragangliomas (4 -6).PHEO situated in the adrenal gland are identified more commonly than those in extraadrenal tissues, because clinicians usually focus on the adrenal gland as a main source of catecholamine production. Although they usually choose a proper imaging technique to attempt the localization of PHEO in the adrenal gland, they are often confused as to which algorithm to follow and what technique to choose for the detection of extraadrenal PHEO. Furthermore, physicians often neglect the facts that up to approximately 25% of patients with apparent sporadic PHEO may, in fact, be carriers of germline mutations, indicating hereditary disease with a predisposition for extraadrenal, often multifocal, PHEO (7); that in children, multifocal and extraadrenal PHEO are found in up to 30 -43% of cases (6, 8 -12); that malignant PHEO account for up to 26 -35% of cases (13)(14)(15)(16)(17)(18); that the prevalence of malignancy in sporadic adrenal PHEO is 9% (5); and that about 10% of patients with PHEO present with metastatic disease at the time of their initial work-up (19). After initial failed surgery, patients with metastatic PHEO are commonly reevaluated using metaiodobenzylguanidine (MIBG) scintigraphy, a modality that should actually be performed before surgery to confirm that a tumor was indeed a PHEO (5-9% of the population harbor an adrenal tumor, most commonly a benign adenoma) (20 -22) or to rule out metastatic disease. These patients are then reoperated upon, suffering additional surgery-related complications and substantial financial expenses. Some clinicians consider the presence of sporadic unilateral small PHEO as indicative of benign disease. This was concluded in older studies of series of patients with . However, more recent works from large series of patients with benign and malignant disease in the United States and Europe do not support this view; rather, they support the opinion that there are no absolute clinical, imaging, or laboratory criteria to predict malignancy and clinical course of PHEO (5, 16, 19,[26][27][28][29][30][31][32]. Nevertheless, PHEO tumors with a diameter larger than 5 cm have a higher potential to metastasize, and such patients should be followed more frequently (33). Consequently, it seems that ruling out metastatic PHEO before initial surgery would be useful, because the detection of other lesions may dramatically affect treatment and follow-up.Localization of PHEO should be attempted using at least two imaging modalities. Anatomical imaging studies [computed tomography (CT) and magnetic resonance imaging (MRI)] should be combined with functional (nuclear medicine) imaging studies for optimal results to locate primary, recurr...

show abstract

“…The diagnostic value of TEE in localizing a paraganglioma has been disputed (3,4). However, as demonstrated in our patient, TEE may provide significant additional information and details on topographic relationships that may assist the cardiothoracic surgeon in planning and conducting the surgical procedure.…”

Section: Discussionmentioning

confidence: 69%

“…If these techniques fail to localize an extraadrenal catecholamine-producing tumor, additional imaging procedures are required, including MIBG radionuclide scintigraphy, octreotide scanning, and thoracic and abdominal aortography that may permit detection of aorta-derived blood supply of extraadrenal tumors. MIBG scintigraphy, a functional rather than topographic test that allows scanning of the entire body, has proved to be superior to CT and MRI in localizing cardiac paragangliomas (3,4,7) and thus has recently assumed a prominent role in the localization of pheochromocytomas. The compound is taken up by pheochromocytomas, ganglioneuromas, neuroblastomas, and other neural crest tumors as well as some carcinoids.…”

Section: Discussionmentioning

confidence: 99%

Greetings from below the aortic arch! The paradigm of cardiac paraganglioma

Heufelder¹

1996

Journal of Clinical Endocrinology & Metabolism

View full text Add to dashboard Cite

A 42-yr-old female, mother of five children, was referred to this institution for further evaluation of recurrent episodes of severe arterial hypertension. Blood pressures taken on several occasions during the past 3 yr by her general practitioner had documented recurrent hypertensive crises with systolic/diastolic measurements of up to 300/145 mm Hg. These episodes were frequently accompanied by headache, palpitations, sweating, nausea, and pallor followed by weakness. Her past medical history was remarkable only for transient hepatitis B following blood transfusion in 1970 and Cesarian section in 1986. Family history was negative for multiple endocrine neoplasia (MEN) type 2A and 2B syndromes, von Recklinghausen's neurofibromatosis, and von Hippel-Lindau's disease. Her medications included intermittent usage of furosemide (40 mg/day) for leg edema and L-thyroxine (200 pg/day) for a history of hypothyroidism. Diagnostic tests before referral had revealed an elevated 24-h urinary excretion of vanillylmandelic acid at 27 mg/24 h (0.3-7.7 mg/24 h) and normal 24-h urinary excretion of catecholamines. In addition, on computed tomography of the abdomen, a right adrenal mass had been detected. Physical examination revealed moderate obesity (170 cm, 83 kg) and marked hypertension (180/115 to 230/120 mm Hg) but was otherwise normal. No heart murmurs were heard, and fundoscopic and skin examination were unremarkable. Twentyfour hour blood pressure monitoring demonstrated a systolic mean of 170 mm Hg without diurnal rhythm.Serum electrolytes were normal except for mildly elevated corrected calcium levels of 2.6-2.8 mmol/L (2.0-2.5 mmol/L) and a low potassium level of 3.3 mmol/L (3.5-5.5 mmol/L). All other routine blood tests were within normal limits. Total 24-h urinary catecholamine measurements revealed markedly elevated concentrations of norepinephrine at 912 pg/24 h (cl00 pg/24 h), metanephrines at 4.5 mg/24 h (<1.3 mg/24 h), and vanillylmandelic acid at 18 mg/24 h (<8 mg/24 h), whereas epinephrine and dopamine concentrations were normal. Thyroid function tests revealed complete suppression of basal TSH at CO.03 mU/L (0. Transthoracic echocardiography demonstrated left ventricular diameters at the upper range of normal and moderate mitral valve insufficiency. Magnetic resonance imaging (MRI) of the adrenal glands confirmed the presence of a 1.5 X 2.5 cm right adrenal mass which, on T,-weighted images, failed to reveal high signal intensity. In search for a catecholamine-producing tumor at an extraadrenal site, 13'1-meta-iodobenzylguanidine (MIBG) whole body imaging was performed and revealed significant uptake medially in the mediastinum at the level of the 6th vertebral body (Fig. 1). A CT scan of the mediastinum localized a well-demarcated mass of 5 cm in diameter adjacent to the carina without obvious infiltration into neighboring structures. ECG-triggered MRI of the mediastinum located the mass on top of the left atrium and just below the carina. High signal intensity of this mass was demonstrated on T,-weighted ...

show abstract

“…However, unusual clinical features frequently occur in patients with extraadrenal tumors and may include a mediastinal mass, upper airway obstruction, or gross hematuria and micturition-associated hypertensive crisis due to a tumor located in the bladder. Less than 50 cases of paragangliomas affecting the heart have been reported to date (2)(3)(4). Due to improved diagnostic localization techniques, cardiac pheochromocytomas are detected with increasing frequency.…”

Section: Discussionmentioning

confidence: 99%

“…Early diagnosis and precise localization are mandatory because complete surgical resection may be curative in the majority of cases (4). However, in many instances, appropriate surgical management of cardiac paraganglioma may be only achieved with full thickness excision of the atria1 wall requiring cardiopulmonary bypass, followed by atria1 reconstruction using a pericardial patch (4). Intraoperative transsection of the great vessels, coronary artery bypass, and even cardiac autotransplantation may be required in rare instances where excision would otherwise remain incomplete FIG. 3.…”

Section: Discussionmentioning

confidence: 99%

Greetings from below the aortic arch! The paradigm of cardiac paraganglioma.

Heufelder

¹

,

Hofbauer

²

1996

The Journal of Clinical Endocrinology & Metabolism

1

0

View full text Add to dashboard Cite

A 42-yr-old female, mother of five children, was referred to this institution for further evaluation of recurrent episodes of severe arterial hypertension. Blood pressures taken on several occasions during the past 3 yr by her general practitioner had documented recurrent hypertensive crises with systolic/diastolic measurements of up to 300/145 mm Hg. These episodes were frequently accompanied by headache, palpitations, sweating, nausea, and pallor followed by weakness. Her past medical history was remarkable only for transient hepatitis B following blood transfusion in 1970 and Cesarian section in 1986. Family history was negative for multiple endocrine neoplasia (MEN) type 2A and 2B syndromes, von Recklinghausen's neurofibromatosis, and von Hippel-Lindau's disease. Her medications included intermittent usage of furosemide (40 mg/day) for leg edema and L-thyroxine (200 pg/day) for a history of hypothyroidism. Diagnostic tests before referral had revealed an elevated 24-h urinary excretion of vanillylmandelic acid at 27 mg/24 h (0.3-7.7 mg/24 h) and normal 24-h urinary excretion of catecholamines. In addition, on computed tomography of the abdomen, a right adrenal mass had been detected. Physical examination revealed moderate obesity (170 cm, 83 kg) and marked hypertension (180/115 to 230/120 mm Hg) but was otherwise normal. No heart murmurs were heard, and fundoscopic and skin examination were unremarkable. Twentyfour hour blood pressure monitoring demonstrated a systolic mean of 170 mm Hg without diurnal rhythm.Serum electrolytes were normal except for mildly elevated corrected calcium levels of 2.6-2.8 mmol/L (2.0-2.5 mmol/L) and a low potassium level of 3.3 mmol/L (3.5-5.5 mmol/L). All other routine blood tests were within normal limits. Total 24-h urinary catecholamine measurements revealed markedly elevated concentrations of norepinephrine at 912 pg/24 h (cl00 pg/24 h), metanephrines at 4.5 mg/24 h (<1.3 mg/24 h), and vanillylmandelic acid at 18 mg/24 h (<8 mg/24 h), whereas epinephrine and dopamine concentrations were normal. Thyroid function tests revealed complete suppression of basal TSH at CO.03 mU/L (0. Transthoracic echocardiography demonstrated left ventricular diameters at the upper range of normal and moderate mitral valve insufficiency. Magnetic resonance imaging (MRI) of the adrenal glands confirmed the presence of a 1.5 X 2.5 cm right adrenal mass which, on T,-weighted images, failed to reveal high signal intensity. In search for a catecholamine-producing tumor at an extraadrenal site, 13'1-meta-iodobenzylguanidine (MIBG) whole body imaging was performed and revealed significant uptake medially in the mediastinum at the level of the 6th vertebral body (Fig. 1). A CT scan of the mediastinum localized a well-demarcated mass of 5 cm in diameter adjacent to the carina without obvious infiltration into neighboring structures. ECG-triggered MRI of the mediastinum located the mass on top of the left atrium and just below the carina. High signal intensity of this mass was demonstrated on T,-weighted ...

show abstract

Location, localization and surgical treatment of cardiac pheochromocytoma

Cited by 52 publications

References 6 publications

Current Approaches and Recommended Algorithm for the Diagnostic Localization of Pheochromocytoma

Current Approaches and Recommended Algorithm for the Diagnostic Localization of Pheochromocytoma

Greetings from below the aortic arch! The paradigm of cardiac paraganglioma

Greetings from below the aortic arch! The paradigm of cardiac paraganglioma.

Contact Info

Product

Resources

About