Pheochromocytoma in Patients Suspected of Harboring Adrenal Meta stasis: Management and Clinical Predictors

ObjectiveTo study clinical management of patients with suspected adrenal metastasis and to assess whether there are clinical predictors of pheochromocytoma in this patient population.MethodsIn this retrospective cross-sectional study, we reviewed medical records of patients who had adrenalectomy for adrenal lesions or had adrenal biopsy performed between January 1997 and July 2007 in a large academic hospital. Patients who harbored adrenal masses that were suspected of being metastases were identified on clinical findings. Pathologic diagnosis, demographic data, clinical history, imaging studies, and laboratory test results were reviewed and compared among patients whose adrenal mass was determined to be metastasis, adenoma, or pheochromocytoma.ResultsOne-hundred sixty-three patients had adrenalectomy or had adrenal biopsy during the study period. Thirty patients (18%) had adrenal masses that were suspected of being metastases. Of the adrenal masses, 18 (60%) were metastases, 8 (27%) were benign adenomas, and 4 (13%) were pheochromocytomas. Eleven patients (37%) had biochemical testing for pheochromocytoma. Adrenal biopsy was performed without biochemical testing for pheochromocytoma in 9 patients (30%), including 2 subsequently found to have this tumor. Adrenalectomy was performed in 10 patients (33%) without biochemical testing for pheochromocytoma. Clinical parameters were similar among patients with metastasis, adenoma, or pheochromocytoma. There were no clinical predictors to suggest pheochromocytoma.ConclusionsPheochromocytoma occurs frequently in patients suspected of harboring adrenal metastasis, but this tumor is often not considered in clinical practice. The size and imaging characteristics of the adrenal mass and history of known metastasis may help clinicians in decision-making. Biochemical testing for pheochromocytoma should ideally be performed in all patients suspected of having adrenal metastasis. (Endocr Pract. 2008;14:967-972)     

Clinical Use and Utility of Metaiodobenzylguanidine Scintigraphy in Pheochromocytoma Diagnosis

ObjectiveTo examine the indications for metaiodo- benzylguanidine (MIBG) scintigraphy and to assess its performance in localizing pheochromocytoma in the post- computed tomography and magnetic resonance imaging era.MethodsIn this retrospective study, electronic and paper medical records of patients who underwent MIBG scintigraphy at a large academic hospital in Los Angeles, California, between January 1995 and July 2009 were reviewed for indications for MIBG scintigraphy, clinical history, biochemical test results, findings from imaging studies, and pathologic diagnoses. MIBG score was defined as follows: 3 (or intensive uptake) meant MIBG uptake of adrenal gland or other locus was higher than that of the liver; 2 (or moderate uptake) meant uptake was similar to that of the liver; 1 (or borderline uptake) meant uptake was lower than that of liver; and 0 (or negative uptake) meant background signal.ResultsNinety-eight patients underwent MIBG scintigraphy during the study period; the indica- tion was suspected pheochromocytoma in 75 cases. Pheochromocytoma diagnosis was excluded in 48 and con- firmed in 15. The remaining 12 patients had insufficient information in the medical records to render a diagnosis. Among the 63 patients, 47 received ¹³¹I-MIBG and 16 received ¹²³I-MIBG. Sensitivity was 73% and specificity was 69% if any adrenal uptake was considered positive, but increased to 90% if borderline uptake was considered negative. False results were more common in younger patients, but not correlated with biochemical test results. In patients with pheochromocytoma either excluded or con- firmed, the MIBG scintigraphy results were confirmatory in 63%, but misleading in 37%. MIBG scintigraphy results did not provide additional diagnostic value to any case and contributed to pheochromocytoma overdiagnosis and even unnecessary adrenalectomy.ConclusionsMIBG scintigraphy results are either confirmatory or misleading, and this imaging modal- ity is not necessary formost patients in modern practice.(Endocr Pract. 2010;16:398-407)     

Acute Myocardial Infarction Attributable to Adrenergic Crises in a Patient with Pheochromocytoma and Neurofibromatosis 1

ObjectiveTo describe a rare case of acute myocardial infarction in a patient with neurofibromatosis 1 and pheochromocytoma and to review the literature on the coexistence of these 2 diseases, the causes of myocardial injury in patients with pheochromocytoma, and the utility of genetic testing and pheochromocytoma screening for those patients and their families.MethodsWe present a case report, including the detailed clinical, laboratory, and radiographic data, results of adrenal mass pathology, and results of coronary angiography. We also survey other relevant reports available in the literature.ResultsA 43-year-old woman with a history of longstanding hypertension, neurofibromatosis 1, headaches, sweating, and palpitations presented to the hospital with chest pain and shortness of breath. She was found to have an acute myocardial infarction and pulmonary edema, as well as a right adrenal mass. A pheochromocytoma was suspected, and phenoxybenzamine was added to her treatment regimen. Cardiac catheterization showed nonobstructive coronary disease. The levels of plasma catecholamine metabolites were extremely high. The patient underwent uncomplicated laparoscopic right adrenalectomy 2 weeks after this admission. Surgical pathology confirmed the diagnosis of pheochromocytoma.ConclusionAdrenergic crisis attributable to pheochromocytoma can result in acute myocardial infarction even in the absence of obstructive coronary disease. Inclusion of pheochromocytoma in the differential diagnosis of hypertension in patients with neurofibromatosis is very important and helps avoid mistakes in the management of such patients. (Endocr Pract. 2007;13:269-273)     

Cardiac Abnormalities Associated with Pheochromocytoma and other Adrenal Tumors

ObjectiveTo study the specific cardiac abnormalities associated with pheochromocytoma and to suggest a strategy for evaluating cardiac function in patients with pheochromocytoma.MethodsIn this case-control study, we reviewed pathology records of patients seen at Cedars-Sinai Medical Center between 1997 and 2007; patients with adrenal or extra-adrenal pheochromocytoma and those with nonfunctioning benign or malignant adrenal tumors were identified. Patients with functioning adrenal adenomas that secreted cortisol or aldosterone were excluded. Clinical history, imaging, pathology, biochemical test results, electrocardiographic findings, and echocardiographic findings were compared between patients with pheochromocytoma and patients with nonfunctioning adrenal tumors.ResultsThe charts of 22 patients with pheochromocytoma and 35 patients with nonfunctioning adrenal tumors were included. No perioperative mortality was observed. The average age of patients with pheochromocytoma was similar to that of control patients (51.9 ± 3.9 years vs 60.2 ± 2.5 years, respectively), as was the number of patients with known cardiovascular diseases (2 [9%] in the pheochromocytoma group vs 5 [14%] in the control group). Two patients with pheochromocytoma (9%) exhibited myocardial damage. Abnormal electrocardiographic findings were present in 16 patients with pheochromocytoma (73%) and in 17 control patients (49%) (P = .1). QTc was prolonged in patients with pheochromocytoma compared with control patients (448.3 ± 9.7 ms vs 424.7 ± 4.5 ms, respectively; P = .02) and was correlated with levels of norepinephrine and normetanephrine, but not with levels of epinephrine and metanephrine or tumor size. ST-T abnormalities were present in 11 patients with pheochromocytoma (50%) and in 8 control patients (23%) (P = .04). Echocardiographic findings were normal in most patients with pheochromocytoma; abnormal left ventricular wall motion was documented in 3 patients with long QTc.ConclusionsThe specific electrocardiographic findings in patients with pheochromocytoma are prolonged QTc and ST-T abnormalities. Performing an electrocardiogram in patients with pheochromocytoma would be prudent. Echocardiography would be useful to examine LV wall motion in patients with long QTc. Coronary artery disease should be excluded in patients with significant ST- T changes. (Endocr Pract. 2009;15:10-16)     

Glucagon-Induced Pheochromocytoma Crisis

ObjectiveTo describe a previously asymptomatic woman who developed a glucagon-induced pheochromocytoma crisis during preparation for screening colonoscopy.MethodsWe present the patient’s clinical features, laboratory and imaging findings, and outcome and review the related literature.ResultsA 76-year-old woman received glucagon to inhibit intestinal motility before routine colonoscopy. She immediately developed severe hypertension, cardiac arrhythmia, and altered mental status. Her hospital course was complicated by encephalopathy and cardiac, respiratory, renal, and hepatic failure. Computed tomography of the abdomen showed a 6.5 × 4.8-cm mass in the left adrenal gland. Biochemical testing for pheochromocytoma revealed markedly elevated plasma catecholamines and metanephrines and urinary vanillylmandelic acid and metanephrine. She underwent a successful laparoscopic left adrenalectomy. Findings from histopathologic and immunohistochemical examination of the adrenal mass were diagnostic of pheochromocytoma.ConclusionsGlucagon administration induced catecholamine release from an occult pheochromocytoma, which caused multiorgan injury. Health care providers using glucagon must consider this rare, but life-threatening, complication. (Endocr Pract. 2011;17:e51-e54)     

Diagnostic Challenge of Pheochromocytoma in a Patient Receiving Levodopa for Parkinson's Disease

ObjectiveThe diagnosis of pheochromocytoma in patients receiving levodopa is challenging because the standard diagnostic biochemical tests may be confounded by dopaminergic therapy. We aim to showcase our experience with the diagnosis of pheochromocytoma in a patient with a known case of Parkinson’s disease who was receiving levodopa.MethodsWe present the case of an elderly male who was diagnosed as having pheochromocytoma while receiving dopaminergic therapy for Parkinson’s disease.ResultsA 75-year-old man presented with vague abdominal symptoms. Computed tomography revealed a 3.5 × 3.2 cm right adrenal mass with a well-defined margin. As revealed by magnetic resonance imaging, the mass was hypointense on T1-weighted and hyperintense on T2-weighted images. Biochemical tests revealed elevated levels of urinary dopamine, which was considered to be caused by levodopa therapy. However, concurrent elevation in urinary adrenaline and his metanephrine and vanillylmandelic acid levels suggested an underlying case of pheochromocytoma. An ¹²³I-metaiodobenzylguanidine (¹²³I-MIBG) scintigraphy scan performed under levodopa therapy showed positive tracer uptake in the right adrenal gland. Histopathology of the adrenalectomy specimen confirmed the diagnosis of pheochromocytoma.ConclusionOur experience with the present case indicates that although the standard diagnostic biochemical tests for pheochromocytoma may be confounded by dopaminergic therapy, ¹²³I-MIBG scintigraphy has diagnostic value for confirming pheochromocytoma even in patients receiving dopaminergic therapy. (Endocr Pract. 2014;20:e112-e115)     

Nonclassic Presentation of Pheochromocytoma: Difficulties in Diagnosis and Management of the Normotensive Patient

ObjectiveTo report an unusual presentation of pheochromocytoma along with challenges in diagnosis and management.MethodsWe report a clinical case history and describe diagnostic methods and pitfalls. The preoperative medical preparation in a normotensive patient is described.ResultsA 33-year-old man fell off a ladder, resulting in C6 paraplegia. After C7-T1 laminectomy, he was transferred to a rehabilitation center where he reported lightheadedness, shortness of breath, and chest pain during therapy sessions. A left adrenal mass was incidentally discovered during the workup to rule out a pulmonary embolism. He reported no history of hypertension, and no elevated blood pressure readings had been documented. Magnetic resonance imaging of the adrenal glands showed a well-defined left adrenal mass measuring 3.9 × 3.2 × 3.3 cm, which was hyperintense on T2-weighted images. Twenty-four hour urinary catecholamine concentrations were unremarkable; urinary metanephrines were markedly elevated. During hospital admission, blood pressure was in the low to normotensive range, requiring a cautious approach to α-adrenergic blockade and surgical preparation. He underwent uneventful laparoscopic left adrenalectomy; surgical pathology was consistent with pheochromocytoma.ConclusionsThis case illustrates a nonclassic presentation of pheochromocytoma and demonstrates that urinary catecholamines alone are not sufficient for a biochemical diagnosis of large pheochromocytomas. Preoperative preparation in normotensive patients can be achieved with α-adrenergic blockade, hydration, and liberal salt intake.     

Safety and Feasibility of Laparoscopic Resection for Large (≥ 6 CM) Pheochromocytomas Without Suspected Malignancy

ObjectiveTo determine whether laparoscopic adrenalectomy in patients without radiologic evidence of cancer compromises the perioperative and long-term outcomes in patients with large (≥ 6 cm) pheochromocytomas.MethodsWe analyzed a prospective adrenal database of consecutive patients who underwent adrenalectomy at our institution between September 2000 and September 2010. Patients with diagnosed pheochromocytoma who underwent laparoscopic adrenalectomy were included. Patients with tumors smaller than 6 cm were compared with those presenting with tumors 6 cm or larger.ResultsOne hundred fifty-seven patients underwent adrenalectomy, and there were 32 catecholamine-secreting tumors. Of the 33, 7 were excluded from the study because of open surgery. Thus, 25 patients presented with 26 pheochromocytomas and underwent laparoscopic adrenalectomy. Thirteen of the 25 patients (52%) were women. Mean age (± standard error of the mean) was 53 ± 3 years. Mean tumor size was 5.2 ± 0.5 cm, and 11 pheochromocytomas (42%) were 6 cm or larger. Tumor size was significantly different between the large pheochromocytoma and the small pheochromocytoma groups (7.6 ± 0.4 vs 3.6 ± 0.4 cm, P <.001), but there was no significant difference in intraoperative complications, estimated blood loss, cancer diagnosis, or recurrence. The length of stay was comparable between the 2 cohorts, and there were no incidents of capsular invasion or adverse cardiovascular events.ConclusionLaparoscopic adrenalectomy of pheochromocytomas larger than 6 cm is feasible and safe with comparable results to those achieved with laparoscopic adrenalectomy in patients with smaller pheochromocytomas. (Endocr Pract. 2012;18:720-726)     

Pheochromocytoma in Neurofibromatosis Type 1: When Should it Be Suspected

ObjectivesNeurofibromatosis type 1 (NF1) carries an increased risk of pheochromocytoma. Most experts recommend that NF1 patients be screened for pheochromocytoma if hypertension develops. We sought to compare NF1 and non-NF1 patients with pheochromocytoma.MethodsRetrospective analysis of a prospectively collected database of all patients undergoing pheochromocytoma resection by a single surgeon from 2003-2012. Statistical significance was evaluated using Fisher’s exact test for categorical variables and the Wilcoxon rank sum test for continuous variables.ResultsOf 56 patients undergoing pheochromocytoma resection, 6 (11%) had NF1. All 6 (100%) NF1 patients had pheochromocytoma diagnosed incidentally during work-up for another condition, whereas 28 of 50 (56%) non-NF1–associated pheochromocytomas were diagnosed incidentally (P = .071). Hypertension was present in 1 (17%) NF1 patient and in 37 (74%) of the non-NF1 patients (P = .011). Tumors were significantly smaller in NF1 patients compared with non-NF1 patients (median tumor dimension, 2.75 cm vs. 5.9 cm, respectively;P = .014).ConclusionAlthough NF1 patients have a well-known increased risk of developing pheochromocytoma, in the current series, all NF1 patients referred to the surgeon for adrenalectomy had pheochromocytoma diagnosed incidentally. Nevertheless, NF1 patients had significantly smaller tumors and less hypertension than other patients treated for pheochromocytoma, perhaps due to a higher frequency of imaging occasioned by their other neoplasms. The common recommendation to screen for pheochromocytoma when hypertension develops would have failed to spur screening in 83% of these NF1 patients. Routine screening for pheochromocytoma in all NF1 patients may be warranted after evaluating whether this is cost-effective in reducing morbidity and mortality. (Endocr Pract. 2014;20:792-796)     

Ordering Pattern and Performance of Biochemical Tests for Diagnosing Pheochromocytoma Between 2000 and 2008

ObjectiveTo examine what tests are ordered by physicians for pheochromocytoma diagnosis and how those tests perform in modern clinical practice.MethodsIn this case series, electronic medical records of patients seen between January 2000 and July 2008 at a large academic hospital in Los Angeles, California, were queried, and patients older than 15 years who underwent any 1 of 5 tests for pheochromocytoma (measurement of plasma catecholamines, plasma fractionated metanephrines, urinary catecholamines, urinary metanephrines, or urinary vanillylmandelic acid) were identified. Because testing was performed in various reference laboratories, test results were classified into 1 of 3 categories: (a) markedly elevated, (b) moderately elevated, or (c) normal. Patient demographics, clinical history, test results, imaging study findings, and pathology records were reviewed.ResultsA total of 3980 tests were ordered for 1898 patients. Pretest probability was 2.2% (based on 681 patients in whom pheochromocytoma was confirmed or excluded), and hypertension was the most common indication for testing. The number of patients tested and the number of tests ordered increased over the years. The ordering pattern stabilized since 2006 when urinary metanephrines, urinary catecholamines, and plasma metanephrines were ordered more frequently. Sensitivity was highest for urinary metanephrines and vanillylmandelic acid, specificity was highest for vanillylmandelic acid and urinary catecholamines, and positive likelihood ratio was highest for vanillylmandelic acid. Positive predictive value for markedly elevated test results was 39% to 83%, while that for moderately elevated test results was only 2% to 14%.ConclusionsOrdering pattern and test performance differ significantly from those recommended and reported by large centers. The best testing strategy should incorporate local experience. Categorizing test results as markedly elevated, moderately elevated, and normal is important for result interpretation. (Endocr Pract. 2009;15:313-321)     

Pheochromocytoma And Pregnancy:A Rare But Dangerous Combination

ObjectiveTo review the literature on pheochromocytoma in pregnancy (PIP) published during the 11-year period 1998 through 2008.MethodsWe searched MEDLINE data sources from 1998 through 2008 using the search terms “pheochromocytoma” and “pregnancy” and reviewed case reports of PIP published in English.ResultsWe identified 54 case reports in MEDLINE data sources, of which 51 reports including 60 cases were reviewed. In comparison with the previous decade, a decreased rate of antenatal diagnosis (from 83% to 70%) and increased maternal and fetal mortality (from 4% to 12% and from 10% to 17%, respectively) were observed. Prematurity was present in 53% of the infants of mothers with antenatally diagnosed pheochromocytoma who gave birth to a live infant. Hypertension was the most common manifestation (88%), with 33% of patients having antepartum hypertensive crises. Urinary catecholamines (64%) and metanephrines (40%) were the most commonly used tests, whereas urinary and plasma metanephrines were the most sensitive tests (100%). Ultrasonography had poor sensitivity (54%), especially during the third trimester. Magnetic resonance imaging was 100% sensitive for adrenal tumors but had only 50% sensitivity for extra-adrenal tumors. A syndromic or familial presentation was seen in 18% of patients, and 32% of patients had bilateral or extraadrenal tumors.ConclusionIn comparison with the previous decade, a decreased rate of antenatal diagnosis and increased maternal and fetal mortality were observed. Prematurity was a major morbidity associated with PIP. Hypertensive crises were more common during pregnancy. Urinary and plasma metanephrines had the highest sensitivity to detect PIP. Genetic screening should be offered to all pregnant women with pheochromocytoma. (Endocr Pract. 2010;16:300-309)     

Pheochromocytoma: A Cause of St-Segment Elevation Myocardial Infarction,Transient Left Ventricular Dysfunction,and Takotsubo Cardiomyopathy

ObjectiveTo report the case of a patient with a pheochromocytoma and apical left ventricular dysfunction that resolved after surgical resection of the pheochromocytoma, to review the effects of catecholamines on myocyte function and the concept that takotsubo cardiomyopathy (TC) is caused by excess catecholamines, and to illustrate the difficulty in the management of an acute coronary syndrome (ACS) during a hypertensive crisis attributable to a pheochromocytoma.MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies in a 60-year-old man with an ACS, TC, and an incidentaloma later diagnosed to be a pheochromocytoma. The association with TC and the pertinent literature are reviewed.ResultsA 60-year-old man was suspected of having myocardial ischemia on the basis of symptoms of paroxysmal chest pain extending to the left shoulder, diaphoresis, ST-segment elevation on an electrocardiogram, and elevated serial levels of cardiac enzymes. Coronary angiography did not reveal substantial coronary artery obstruction but detected ballooning of the apical, anterior, and inferior cardiac walls, consistent with TC. He had a history of labile hypertension and palpitations of 3 months’ duration. An adrenal mass detected on a prior computed tomographic scan and increased 24-hour urine catecholamine levels were consistent with a pheochromocytoma. Treatment with phenoxybenzamine was initiated, and he underwent a right adrenalectomy, which confirmed that the tumor was a pheochromocytoma and dramatically improved the patient’s condition.ConclusionPheochromocytomas manifest with labile blood pressures and should be considered in the differential diagnosis of ACS. This case also supports the concept that TC is caused by excess catecholamines. (Endocr Pract. 2012;18:e77-e80)     

Recurrent Takotsubo Cardiomyopathy Associated with Pheochromocytoma

ObjectiveTo describe a case of recurrent takotsubo cardiomyopathy in a patient with pheochromocytoma.MethodsWe present a case report, including clinical and laboratory data. In addition, the current relevant literature pertaining to pheochromocytoma and takotsubo syndrome is reviewed and summarized.ResultsIn 2004, an 81-year-old woman with no history of cardiac disease presented with chest discomfort, and takotsubo syndrome was diagnosed. No emotional or physical stressors were identified at that time. Her left ventricular systolic function normalized during that hospitalization. In 2007, the patient was readmitted to the hospital with chest discomfort and ST-segment elevation. Cardiac catheterization demonstrated only minor nonobstructive coronary artery disease. She was again found to have takotsubo syndrome with a classic apical hypokinetic segment. Treatment with a heart failure regimen was initiated, and she was screened for pheochromocytoma as the precipitant for her recurrent takotsubo cardiomyopathy. A 24-hour urine collection showed minimally elevated normetanephrine excretion of 719 μg (reference range, 148 to 560) and vanillylmandelic acid of 8.3 mg (reference range, < 8.0). The plasma normetanephrine level was 1.57pg/mL (reference range, < 0.9). Subsequent magnetic resonance imaging revealed a left adrenal mass (2 cm by 1 cm). Ultimately, the patient underwent left adrenalectomy, and the pathology report was consistent with pheochromocytoma. She has been asymptomatic since then, and a repeated echocardiogram demonstrated normal left ventricular systolic function.ConclusionIn patients presenting with takotsubo cardiomyopathy, a precipitating factor, such as emotional or physical stress, can often be identified. In some patients (such as our current case), however, pheochromocytoma may be the underlying disease and should be considered. (Endocr Pract. 2009;15:560-562)     

Pheochromocytoma mimicking an acute myocardial infarction

We report a 42-year-old female who presented with retrosternal pain, dyspnoea and nausea. Electrocardiography suggested a recent anterior myocardial infarction. However, emergency coronary angiography showed normal blood flow through all the coronary arteries. Paroxysmal hypertension raised the suspicion of a pheochromocytoma. Indeed, abdominal ultrasonography and computed tomography revealed a mass in the left adrenal gland. Elevated levels of plasma and urine catecholamines supported the diagnosis of pheochromocytoma. Left adrenalectomy was performed without complications and pathological examination revealed a 5.5 cm pheochromocytoma. After surgery, all antihypertensive medication was discontinued and the blood pressure returned to normal within several days. Currently, the patient is asymptomatic, has normal catecholamine levels and the electrocardiographic signs of ischaemia have resolved entirely. This case illustrates that a rare clinical entity such as pheochromocytoma should be considered in the differential diagnosis of acute coronary syndrome. (Neth Heart J 2007; 15:248-51.)     

Performance of Plasma Fractionated Free Metanephrines by Enzyme Immunoassay In the Diagnosis Of Pheochromocytoma and Paraganglioma in Children

ObjectiveTo establish pediatric reference ranges for plasma fractionated free metanephrines by enzyme immunoassay (EIA) and to evaluate its performance in the diagnosis of catecholamine-secreting tumors in the pediatric population.MethodsNormotensive children and children with suspected catecholamine-secreting tumors underwent measurement of plasma fractionated metanephrines by EIA to establish pediatric reference ranges. Children with suspected pheochromocytoma or paraganglioma also underwent magnetic resonance imaging or computed tomography from the neck to the pelvis and were followed up for a minimum of 1 year. Diagnosis of pheochromocytoma/ paraganglioma was confirmed by histologic examination. Pheochromocytoma/paraganglioma was excluded in children who had a histologic diagnosis other than pheochromocytoma/paraganglioma and in those who had no imaging evidence of tumor and no progression on follow-up.ResultsPlasma fractionated metanephrines were measured in 78 normotensive children (age range, 1.5-17 years) and in 38 children with suspected catecholamine-secreting tumors. Of the 38 children (age range, 6-17 years) with suspected pheochromocytoma/paraganglioma, 17 had a histopathologically proven catecholamine-secreting tumor. The newly derived pediatric upper reference limit for metanephrine (128 pg/mL) was higher than in adults (90 pg/mL), whereas the pediatric upper reference limit for normetanephrine (149 pg/mL) was lower than in adults (180 pg/mL). The manufacturer’s reference range for plasma fractionated metanephrines yielded a sensitivity of 100% and a specificity of 85.7%. Use of newly established pediatric reference ranges increased the specificity to 95.2% without altering the sensitivity (100%).ConclusionsPlasma fractionated metanephrines by EIA provide an accurate test with good sensitivity and specificity for the diagnosis of pheochromocytoma and paraganglioma in children. Use of pediatric reference ranges improves accuracy of the test. (Endocr Pract. 2012;18:694-699)     

Pathological and clinical outcomes of adrenalectomy: A multi-center experience in Saudi Arabia

ObjectiveTo determine the nature of adrenal pathology in patients undergoing adrenalectomy in Saudi Arabia over the last decade and compare it with the literature. We compared perioperative outcomes between minimally invasive adrenalectomy (MIA) and open adrenalectomy (OA).MethodsThis retrospective study included patients who underwent adrenalectomy at five tertiary care centers in Saudi Arabia from 2010 to 2020. We collected patients’ baseline and perioperative characteristics and detailed hormonal evaluation of adrenal masses.ResultsAmong 160 patients (mean age 44 ± 14.5 years; mean BMI 29.17 ± 5.96 kg/m²), 84 (51.5 %) were men and 51.5 % had left-sided adrenal masses. The mean tumor size was 6.1 ± 4.2 (1.0–19.5) cm, including 60 (37.5 %) incidentalomas and 65 (40.6 %) functioning masses. Histopathology revealed 74 (46.2 %) adenomas and 24 (15 %) cancers or metastases from other primary organs; 20 %, 8.8 %, and 2.5 % of patients had pheochromocytoma, myelolipoma, and 2.5 % ganglioneuroblastoma, respectively. MIA and OA were performed in 135 (84.4 %) and 21 (15.6 %) patients, respectively. Adrenalectomy was increasingly performed over three equal periods in the last decade (17.5 % vs 34.4 % vs 48.1 %), with increasing numbers of MIAs to replace OAs. OA patients had larger tumors and needed blood transfusion more frequently (47.6 % vs 10.8 %, p< 0.001). MIA was significantly associated with shorter operative time, shorter length of stay, and less blood loss. Postoperative complications occurred in 10 (6.2 %) patients and were significantly higher for OA (24 % vs 3.0 %, p< 0.001).ConclusionsThe majority of adrenal masses are benign. Herein, the observed functional and perioperative outcomes were comparable to those of available meta-analyses.     

Cardiac Complications as Initial Manifestation of Pheochromocytoma: Frequency,Outcome, and Predictors

ObjectiveTo examine the frequency, outcome, and clinical predictors of cardiac complications as the initial manifestation of pheochromocytoma.MethodsThe medical records of all 76 patients with pheochromocytoma or functional paraganglioma treated at Cedars-Sinai Medical Center, Los Angeles, California, from 1995 to 2011 were reviewed. The patients initially presenting with cardiac complications were identified, and their clinical, laboratory, and imaging characteristics were compared with those of the patients presenting with other complaints, especially hypertension and adrenal mass.ResultsOf the 76 patients, 9 (12%) presented with the following: 2 with acute heart failure, 1 with left ventricular thrombus, 3 with myocardial infarction, and 3 with severe arrhythmia. Failure to diagnose pheochromocytoma resulted in unnecessary invasive interventions in 2 patients. Recovery of cardiac function was excellent after resection of the tumor in all patients. In comparison with the 67 patients presenting with other complaints, the 9 with cardiac complications had similar demographics and cardiac risk factors but harbored larger tumors (6.7 ± 0.8 cm versus 4.4 ± 0.3 cm; P = .015) and exhibited higher biochemical marker levels (23.9 ± 9.0-fold versus 11.3 ± 2.4fold; P = .082), longer corrected QT interval (473 ± 8 ms versus 443 ± 6 ms; P = .015), and lower ejection fraction (43% ± 8% versus 66% ± 2%; P = .002).ConclusionIn this study, 12% of patients with pheochromocytoma initially presented with cardiac complications. Patients with large tumors and high levels of biochemical markers were more likely to develop cardiac injury. Our results confirm that the presence of pheochromocytoma should be ruled out in patients with cardiac diseases and features suggesting pheochromocytoma so that unnecessary interventions can be avoided and cardiac recovery can be achieved. (Endocr Pract. 2012;18:483-492)     

Association of Pheochromocytoma and Ganglioneuroma: Unusual Finding in Neurofibromatosis Type 1

ObjectiveTo report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies.MethodsWe present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature.ResultsA 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal,<100) and a 24-hour urine epinephrine excretion of 55 μg (normal,<20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an α-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen’s disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors.ConclusionThe current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies. (Endocr Pract. 2007;13:647-651)     

Comparison of Preoperative Alpha-blockade for Resection of Paraganglioma and Pheochromocytoma

ObjectivePhenoxybenzamine (nonselective, noncompetitive alpha-blocker) is the preferred drug for preoperative treatment of pheochromocytoma, but doxazosin (selective, competitive alpha-blocker) may be equally effective. We compared the efficacy of doxazosin vs phenoxybenzamine.MethodsWe conducted a prospective study of patients undergoing pheochromocytoma or paraganglioma resection by randomizing pretreatment with phenoxybenzamine or doxazosin at a single tertiary referral center. The high cost of phenoxybenzamine led to high crossover to doxazosin. Randomization was halted, and a consecutive historical cohort of phenoxybenzamine patients was included for a case-control study design. The efficacy of alpha-blockade was assessed with preinduction infusion of incremental doses of phenylephrine. The primary outcomes were mortality, cardiovascular complications, and intensive care unit admission. The secondary outcomes were hemodynamic instability index (proportion of operation outside of hemodynamic goals), adequacy of blockade by the phenylephrine titration test, and drug costs.ResultsTwenty-four patients were prospectively enrolled (doxazosin, n = 20; phenoxybenzamine, n = 4), and 15 historical patients treated with phenoxybenzamine were added (total phenoxybenzamine, n = 19). No major cardiovascular complications occurred in either group. The phenylephrine dose-response curves showed less blood pressure rise in the phenoxybenzamine than in the doxazosin group (linear regression coefficient = 0.008 vs 0.018, P = .01), suggesting better alpha-blockade in the phenoxybenzamine group. The median hemodynamic instability index was 14% vs 13% in the phenoxybenzamine and doxazosin groups, respectively (P = .56). The median highest daily cost of phenoxybenzamine was $442.20 compared to $5.06 for doxazosin.ConclusionPhenoxybenzamine may blunt intraoperative hypertension better than doxazosin, but this difference did not translate to fewer cardiovascular complications and is offset by a considerably increased cost.