Clinical profile of pheochromocytoma and paraganglioma with normal plasma free metanephrines / 北京大学学报(医学版)

OBJECTIVE@#Plasma free metanephrines (MNs) have been widely used as an initial test for pheochromocytoma and paraganglioma (PPGL). PPGL without MNs elevation has been reported on rare occasions. The objective of this study was to analyze the clinical profile of sporadic PPGL patients with normal MNs.@*METHODS@#In the study, 104 patients with sporadic PPGL diagnosed by histopathology in Peking University First Hospital from March 2015 to January 2020 were enrolled. All the patients had plasma MNs result, of whom, eight (7.69%) were with normal MNs. The reasons for their medical visits, clinical manifestations, the levels of plasma free MNs, 3-methoxytyramine (3-MT), catecholamines and chromogranin A (CgA), and the imaging findings were documented. Their preoperative diagnosis, perioperative medical management, and intraoperative blood pressure were analyzed. All the data mentioned above were compared with the MNs elevated group. The postoperative follow-up for MNs normal patients were applied.@*RESULTS@#For the eight PPGL patients with normal plasma MNs, the most common clinical symptoms were sweating (3/8), abdominal and back pain (3/8), headache (2/8), palpitations (2/8), and fatigue (2/8). There were no significant differences in plasma free 3-MT and catecholamines' diagnostic positive rate between the MNs normal group and MNs elevated group, but the rate for plasma CgA was significantly decreased in the MNs normal group (2/5 vs. 41/43, P=0.005). No significant difference was found for the incidence of typical findings by enhanced CT between the two groups. In these eight MNs normal patients, six were diagnosed with PPGL by the previous history of PPGL, typical symptoms and CT findings, or elevation of 3-MT, CgA levels or positive results of PET-CT; two patients were misdiagnosed as nonfunctioning adenoma or primary aldosteronism. All these MNs normal patients underwent preoperative management with alpha adrenergic receptor blockers, of whom, one had an average intraoperative arterial pressure < 60 mmHg during surgery. The median follow-up time for the eight patients was 1.5 (0.5－4.5) years. No evidence of new tumors was found on the enhanced CT scans. Two MNs normal patients' plasma 3-MT and (or) CgA decreased to normal.@*CONCLUSION@#For patients with adrenal or retroperitoneal tumors, typical symptoms or a previous history of PPGL, normal plasma MNs is not a sufficient exclusion for PPGL. Plasma 3-MT, catecholamine, CgA results and the imaging findings are helpful for the diagnosis of PPGL. We recommend patients with suspected MNs normal PPGL take alpha adrenergic receptor blockers as preoperative blockade, but should avoid overdose. Postoperative follow-up for patients with normal MNs should focus on the positive biochemical markers before surgery.

Characteristics of Adrenal Incidentalomas in a Large, Prospective Computed Tomography-Based Multicenter Study: The COAR Study in Korea

PURPOSE: Previous studies on adrenal incidentalomas (AIs) are limited by their retrospective design, small numbers of patients, Western populations, or use of an outdated imaging technique. We investigated the characteristics of AIs in Korean patients and compared them with those reported in the largest retrospective study in Italy to discover the effects of improved imaging techniques and ethnicity differences. MATERIALS AND METHODS: This was a prospective, multicenter, cross-sectional observational study including 1005 Korean patients. Levels of plasma adrenocorticotrophic hormone, 24-h urinary free cortisol (UFC), serum cortisol after a 1 mg-dexamethasone suppression test, 24-h urinary fractionated metanephrine, and plasma aldosterone and plasma renin activity were measured. All AIs were characterized using computed tomography (CT). RESULTS: Compared with the results of the Italian study, AIs in Korean patients were observed more frequently in men and predominantly on the left side. Korean patients with AIs were slightly younger, and fewer patients underwent surgery. Most AIs were nonfunctional in both studies, while fewer subclinical hypercortisolism and more primary aldosteronism (PA) cases were detected in Korean patients. In our study, high UFC levels showed very low sensitivity, compared to those in the Italian study. In pheochromocytoma or PA cases, there were no hormonal differences between the studies. AIs in Korean patients were smaller, such that a lower cutoff size for detecting adrenocortical carcinoma (ACC) could be warranted. CONCLUSION: Recent advances in CT technology were leveraged to provide accurate characteristics of AIs and to detect smaller ACCs.

Pheochromocytoma Developed in a Boy with Multiple Endocrine Neoplasia Type 2A Confirmed by the RET Proto-Oncogene Mutation / 임상소아혈액종양

A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.

Update on Modern Management of Pheochromocytoma and Paraganglioma

Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong.

Pheochromocytoma Developed in a Boy with Multiple Endocrine Neoplasia Type 2A Confirmed by the RET Proto-Oncogene Mutation / 임상소아혈액종양

A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.

Feocromocitoma-paraganglioma: revisión de tema / Pheochromocytoma-paraganglioma: a review

Resumen: el feocromocitoma es una enfermedad poco frecuente, correspondiente a un tumor derivadode las células cromafines, originado en la médula de la glándula suprarrenal. Cuando este tumor se origina en los paraganglios el término correcto es paraganglioma. Las manifestaciones clínicas son variables y secundarias a la masa tumoral o a la producción exagerada de catecolaminas. Los síntomas originados por el crecimiento de la masa tumoral incluyen dolor abdominal, malestar epigástrico, obstrucción de estructuras cercanas como la vía biliar y los uréteres, y, cuando está localizado en otros sitios como el mediastino, disnea. Los síntomas secundarios a la hiperproducción de catecolaminas son diaforesis, palpitaciones, cefalea, crisis hipertensivas, hipertensión sostenida, constipación, náuseas, vómito y, en la forma más exagerada, crisis catecolaminérgicas. El diagnóstico se realiza mediante la medición de metanefrinas libres en orina de 24 horas o en plasma, y como alternativa se puede realizar medición de catecolaminas en orina de 24 horas. La medición de ácido vanilmandélico es poco sensible, lo mismo que la medición de catecolaminas en plasma, la cual no es recomendada. El tratamiento de esta enfermedad es quirúrgico. En aquellos pacientes con feocromocitomamaligno o paraganglioma se puede realizar una estrategia de observación activa y en quienes tengan progresión de la enfermedad el uso de quimioterapia o, según el grado de compromiso, el uso de metayodobencilguanidina marcada con yodo-131 es la terapia de elección. El uso de terapia molecular diana utilizando inhibidores de tirosina quinasa es un área de investigación activa.

Abstract: Pheochromocytoma is a rare disease, corresponding to a tumor originated in the chromaphincells, and located in the adrenal medulla. When the tumor is located in the paraganglia the appropriate term is paraganglioma. Clinical manifestations are variable and secondary to the tumoral local extension or to the overproduction of catecholamines. Symptoms secondary to local growth of the tumor includes abdominal pain, epigastric discomfort, obstruction of nearby structures such as biliary tree and ureters, and when is located in other regions such as the mediastinum, dyspnea. Symptoms secondary to overproduction of catecholamines are diaphoresis, headache, hypertensive emergencies, sustained hypertension, constipation, nausea, vomiting and, in the most extreme form, catecholaminergic crisis. Diagnosis is based on the measurement of free plasmatic or urinary metanephrines.As an alternative can be used 24 hours urinary catecholamines. The detection of vanillylmandelic acid and plasma catecholamines has poor sensibility and is no longer recommended. The treatment of this entity is surgical resection. In those patients with malignant pheochromocytoma or paraganglioma an observation and watch strategy can be used. If disease progression is detected, the use of chemotherapy or iodine-131-labeled meta-iodobenzylguanidine, according to the severity of compromise, is the next step. The use of targeted molecular therapies using tyrosine kinase inhibitoris an area under active research.

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis / 영남의대학술지

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma has been rarely reported, whereas only a few cases of Cushing syndrome accompanied by opportunistic infections have been reported. We experienced a patient with pheochromocytoma with ectopic Cushing syndrome complicated by invasive aspergillosis. A 35-year-old woman presented with typical Cushingoid features. Her basal plasma cortisol, ACTH, and 24-hour urine free cortisol levels were significantly high, and 24-hour urine metanephrine and catecholamine levels were slightly elevated. The endogeneous cortisol secretion was not suppressed by either low- or high-dose dexamethasone. Abdominal computed tomography (CT) revealed a heterogeneous enhancing mass measuring approximately 2.5 cm in size in the left adrenal gland. No definitive mass lesion was observed on sellar magnetic resonance imaging. On fluorine-18 fluorodeoxyglucose positron emission tomography/CT, a hypermetabolic nodule was observed in the left upper lung. Thus, we performed a percutaneous needle biopsy, which revealed inflammation, not malignancy. Thereafter, we performed a laparoscopic left adrenalectomy, and its pathologic finding was a pheochromocytoma with positive immunohistostaining for ACTH. After surgery, the biochemistry was normalized, but the clinical course was fatal despite intensive care because of the invasive aspergillosis that included the lungs, retina, and central nervous system.

A 5-Year Prospective Follow-Up Study of Lipid-Rich Adrenal Incidentalomas: No Tumor Growth or Development of Hormonal Hypersecretion

BACKGROUND: Current guidelines for follow-up of adrenal incidentalomas are extensive and hampered by lack of follow-up studies. We tested the hypothesis that small lipid-rich adrenal incidentalomas, initially characterized by tumor size 20 mm for the patient with largest tumor growth and those with subclinical hypercortisolism. All patients had normal 24-hour urinary metanephrines and normetanephrines. Low attenuation (<10 HU) was demonstrated in 97% of 67 masses re-evaluated with unenhanced CT. CONCLUSION: None of the patients developed clinically relevant tumor growth or new subclinical hypercortisolism. Biochemical screening for pheochromocytoma in incidentalomas demonstrating <10 HU on unenhanced CT is not needed. For such incidentalomas <40 mm, it seems sufficient to perform control CT and screen for hypercortisolism after 5 years.

Development and Validation of Liquid Chromatography-Tandem Mass Spectrometry Method for Quantification of Plasma Metanephrines for Differential Diagnosis of Adrenal Incidentaloma

High-resolution imaging techniques have increased the detection rate of adrenal incidentaloma. We developed a method of liquid chromatography-tandem mass spectrometry (LC-MS/MS) for detection of plasma free metanephrine (MN) and normetanephrine (NMN) and evaluated its analytical performance and clinical efficacy in differential diagnosis of adrenal incidentaloma. After solid-phase extraction, chromatographic isolation of the analytes and internal standard was achieved by column elution in the LC-MS/MS system. The analytes were detected in multiple-reaction monitoring mode by using positive electrospray ionization: MN, transition m/z 180.1-->165.1; NMN, m/z 166.1-->134.1. This method was validated for linearity, precision, accuracy, lower limits of quantification and detection, extraction recovery, and the matrix effect. Plasma concentrations of MN and NMN of 14 patients with pheochromocytoma were compared with those of 17 healthy volunteers, 10 patients with essential hypertension, and 60 patients with adrenal adenoma. The assay's linear range was 0.04-50.0 and 0.08-100.0 nmol/L for MN and NMN, respectively. Assay imprecision was 1.86-7.50%. The accuracy ranged from -7.50% to 2.00%, and the mean recovery of MN and NMN was within the range 71.5-95.2%. Our LC-MS/MS method is rapid, accurate, and reliable and useful for differential diagnosis of adrenal incidentaloma.

A Case of Paroxysmal Pheochromocytoma with Normal Catecholamine Levels / 대한내과학회지

A 53-year-old woman had a 1.7 cm left adrenal mass on an abdominal computed tomography (CT) scan. She presented with paroxysmal headache, palpitation, sweating, and hypertension. The patient was highly suspected of having a pheochromocytoma, but measurements of 24-hour urinary metanephrine, catecholamines, and vanillylmandelic acid were normal. Plasma and urine catecholamine levels were within the normal range even during paroxysmal episodes. A scintigraphic study with 131I-metaiodobenzylguanidine (MIBG) revealed selective concentration of the radiotracer, corresponding to the CT mass. The patient underwent a left adrenalectomy and the pathological examination confirmed the diagnosis of pheochromocytoma. In this report, we describe a rare case of a symptomatic pheochromocytoma with normal catecholamine levels. Our case illustrates that routine nuclear scintigraphy, such as 131I-MIBG, should be performed even in cases with normal hormonal testing for all patients with high clinical suspicion of pheochromocytoma.

Abordaje laparoscópico transperitoneal de feocromocitomas y paragangliomas / Transperitoneal laparsoscopic surgery for pheochromocytomas and paragangliomas

Los feocromocitomas adrenales y extra-adrenales o paragangliomas funcionantes constituyen una causa inusual de hipertensión arterial pasible de ser tratada mediante el tratamiento laparoscópico...El abordaje laparoscópico de feocromacitomas adrenales funcionantes constituye un método seguro y efectivo, siendo considerado actualmente el procedimiento de elección. Los avances de los métodos por imágenes de localización pre-operatoria y el continuo desarrollo quirúrgico laparoscópico permitirán, en un futuro no muy lejano, imponer el acceso laparascópico también como vía de elección en feocromocitomas extra-adrenales o paragangliomas. La alta incidencia de bilateralidad y compromiso extraadrenal justifica, a nuestro entender, la triple evaluación imagenológica (TAC-RMN-Centellografía MIBG) en todos los pacientes.

Pheochromocytoma, papillary thyroid carcinoma

A 53-year-old woman presented with labile and difficult to control hypertension on 3 different anti-hypertensive medications. Abdominal computed tomography and ultrasonography of the thyroid gland showed a 1.8 cm thyroid nodule. Fine needle aspiration biopsy of the thyroid nodule revealed papillary thyroid carcinoma. Serum thyroid stimulating hormone and free thyroxine, calcitonin, carcinoembryonic antigen, intact parathyroid hormone, and calcium levels were within normal limits. A 24-hour urine metanephrine showed significant elevation in urine metanephrine of approximately 3 times the upper limit of normal, and the result of 131I-metaiodobenzyleguanjdjne [131I-MIBG] scintography confirmed that the adrenal mass was pheochromocytoma. Right adrenalectomy and total thyroidectomy were performed. The final pathology was pheochromocytoma and papillary thyroid carcinoma. An analysis of c-ret porto-oncogene mutation yielded a negative result. This unusual association of 2 tumors represents a new entity

Clinical Characteristics of Pheochromocytoma in Korean Children / 대한소아내분비학회지

PURPOSE: Pheochromocytoma is a catecholamine-producing tumor that arises from the chromaffin cells of the adrenal medulla or the sympathetic ganglia. This study was undertaken to evaluate the clinical profiles of children with pheochromocytoma. METHODS: We retrospectively reviewed the clinical, radiological and outcome of 16 children who had been diagnosed as pheochromocytoma from May, 1984 to July, 2006 in the department of pediatrics, Seoul National University Hospital. RESULTS: The mean age at diagnosis was 12.3+/-3.4 years (range, 7.1-20 yrs). The male to female ratio was 10:6. The major symptoms were hypertension in 87.5%, sweating in 75%, tachycardia in 68.8% and headache in 62.5%. The sensitivities of 24 hour urinary norepinephrine and metanephrine were 100 %. All tumors were localized by combined study of CT, ultrasonography, MRI or (123)I-MIBG scan. Single (123)I- MIBG scan had sensitivity as 92.9%. Sporadic cases were 13 (81.3%). Bilateral pheochromocytomas were observed in 6 patients (37.5%). The pheochromocytoma developed in 6 patients with underling disease; von Hippel-Lindau (VHL) syndrome (3), neurofibromatosis (1), and cyanotic congenital heart diseases (2). Adrenalectomy was performed in 13 patients, and tumorectomy was in 3 patients. Two patients were diagnosed as malignant pheochromocytoma. CONCLUSION: Because the symptoms are deceptive and variable, the hormonal and radiological study should be performed carefully in suspected cases. The pheochromocytoma can now be operated safely. The tumor might present as a component of other syndromes such as multiple endocrine neoplasia type 2, neurofibromatosis, and VHL syndrome. Thus comprehensive consideration is needed at initial diagnostic approach.

A case of adrenocortical adenoma clinically mimicking pheochromocytoma / 대한내과학회지

The coexpression of cortical and medullary features in a single adrenal cortical cell has been recognized, leading to terms such as cortico-medullary cells. Here, we reported a case of adrenocortical adenoma consisting of cortico-medullary cells that clinically mimicked pheochromocytoma. A 52-year-old woman was admitted to our hospital complaining of an 8-month history of paroxysmal palpitation with refractory hypertension. A 24-hour urine study revealed increased norepinephrine and metanephrine levels. Computed tomography of the abdomen revealed a 1.0x0.9-cm mass in the left adrenal gland. The patient subsequently underwent unilateral laparoscopic adrenalectomy for a presumptive pheochromocytoma. Light microscopic findings of the left adrenal mass indicated an adrenocortical adenoma, but electron microscopy identified lipid vacuoles and smooth endoplasmic reticulum, along with dense core neurosecretory granules, so-called cortico-medullary cells. This is the first report of the detection of cortico-medullary cells in adrenocortical adenoma presenting as pheochromocytoma in Korea.

Association of a retroperitoneal liposarcoma and an adrenal medullary hyperplasia feigning a pheochromocytoma

A 45-year old woman presented with a recent paroxysmal hypertension and a flush syndrome. Physical examination revealed an abdominal mass with positive left lumbar contact. The urine metanephrine was high. The CT scan and the Magnetic Resonance imaging [MRI] showed a 10 cm retgroperitoneal tumour apart from the left adrenal gland and driving back the left kidney. The diagnosis of ectopic pheochromocytoma was suspected and the patient was operated. A tumour resection widened to the left adrenal gland and to the kidney was performed The specimen histology showed a well-differentiated liposarcoma associated to an adrenal medullary hyperplasia [AMH] Postoperatively blood pressure was stabilizsed at normal levels. The review of literature show that AMH mimicking a pheochromocytoma is rare and its association with a giant retroperitoneal liposarcoma has never been reported before

Diagnostic Value of 24-hours Urinary Total Metanephrine As a Screening Test of Patients with Suspected Pheochromocytoma / 대한내분비학회지

BACKGROUND: A pheochromocytoma is a rare cause of secondary hypertension. Its diagnosis is important as the hypertension is usually curable by resection of the tumor, whereas the condition is potentially lethal if undetected. Biochemical confirmation of excessive catecholamine production is a prerequisite to a definitive diagnosis. Various studies from other countries have indicated that measuring of the urinary metanephrine, using a specific procedure, is the single most reliable screening test for all patients suspected of having a pheochromocytoma. However, the diagnostic value of urinary metanephrine has never been reported in Korea. We investigated the diagnostic value of 24-hours urinary metanephrine in patients with a suspected pheochromocytoma. METHODS: This was a retrospective evaluation study, which included 95 patients with sustained hypertension and paroxysmal symptoms, and 38 patients with asymptomatic adrenal incidentaloma at Seoul National University Hospital, between July 2000 and October 2002. We performed the 24- hour urinary total metanephrine test on all patients. The diagnosis of a pheochromocytoma was applied only when confirmed by pathological analysis of a resected specimen. The possibility of a pheochromocytoma was ruled out when all biochemical tests were normal, which were performed at least in duplicate, or there was no evidence of a mass in abdominal radiological studies or histological verification. We determined the upper reference limit for urinary metanephrine as 1.3mg/day and calculated the sensitivity and specificity of the 24-hour urinary metanephrine test. RESULTS: Seventeen patients were diagnosed with a pheochromocytoma. The total metanephrine measurement had sensitivities and specificities of 82.4 and 73.3% in all the patients, 90.9 and 66.7% in patients with hypertension and paroxysmal symptoms, and 66.7 and 90.6% in patients with adrenal incidentaloma, respectively. CONCLUSION: The urinary total metanephrine measurement had relatively lower sensitivities and specificities than in other countries(sensitivity: 83~100%, specificity: 80~98%). The sensitivity of urinary metanephrine was relatively high in patients with hypertension and paroxysmal symptoms, and the specificity was high in patients with an adrenal incidentaloma. We suggest that normetanephrine and metanephrine should be separately measured, and a reasonable upper reference limit be used. It may also be necessary to measure urinary metanephrine together with urinary catecholamine or VMA to improve the diagnostic value of the urinary metanephrine test.

A Clinical Analysis of Pheochromocytoma / 대한내분비외과학회지

Pheochromocytoma manifesting hypertensive episodes, is a catecholamine-secreting neoplasm, that arises from the chromaffin cells This clinical study was performed on 12 cases of pheocromocytoma treated surgically at the Department of Surgery of Chonnaom National University from January 1988 to June 1995. The results are as follows: 1) The male to female ratio was 1:2. The prevalent ages were the fifth decades (33%) and third, forth decades (25%). 2) The most frequent clinical symptoms and signs were hypertention and palpitation (83.3%), followed by headache, dyspnea, faintness and chest discomfort. 3) 41.7% of the patients presented clinical symptoms and signs less than 1 year of duration. 4) The 24-hour urine VMA was elevated in 100% of 10 cases. metanephrine was elevated in 77.8% of 9 cases. 5) Preoperative localization of tumor was easily acchieved by CT, US, MRI, 131-I MIBG scan, and angiography. 6) The alpha-adrenergic blocker was used preoperatively in 9 cases to control hypertension. 7) The tumor was located in the left adrenal gland in 6 cases (50%). right adrenal gland in 5 cases(41.7%), and the abdominal paraganglia in 1 case (8.3%). 8) In pathologic and clinical findings, 4 cases were malignant. Among them, 2 were recurred, mass excision was impossable in 1 case. 9) post operative blood pressure was returned to normotensive level in 70%, post operative 24- hour urine VMA and metanephrine were found to be within normal levels and significantly decreased in the major of cases. In conclusion, CT,US and MRI have good diagnostic results in preoperative localization of pheochromocytoma.and it is safe to operate pheochromocytoma with compatible preoperative preparation. Also, it is important to closely follow up with recurring or metastasis in pathologically proven malignant pheochromocytoma.

A Clinical Analysis of Pheochromocytoma / 대한내분비외과학회지

Pheochromocytoma manifesting hypertensive episodes, is a catecholamine-secreting neoplasm, that arises from the chromaffin cells This clinical study was performed on 12 cases of pheocromocytoma treated surgically at the Department of Surgery of Chonnaom National University from January 1988 to June 1995. The results are as follows: 1) The male to female ratio was 1:2. The prevalent ages were the fifth decades (33%) and third, forth decades (25%). 2) The most frequent clinical symptoms and signs were hypertention and palpitation (83.3%), followed by headache, dyspnea, faintness and chest discomfort. 3) 41.7% of the patients presented clinical symptoms and signs less than 1 year of duration. 4) The 24-hour urine VMA was elevated in 100% of 10 cases. metanephrine was elevated in 77.8% of 9 cases. 5) Preoperative localization of tumor was easily acchieved by CT, US, MRI, 131-I MIBG scan, and angiography. 6) The alpha-adrenergic blocker was used preoperatively in 9 cases to control hypertension. 7) The tumor was located in the left adrenal gland in 6 cases (50%). right adrenal gland in 5 cases(41.7%), and the abdominal paraganglia in 1 case (8.3%). 8) In pathologic and clinical findings, 4 cases were malignant. Among them, 2 were recurred, mass excision was impossable in 1 case. 9) post operative blood pressure was returned to normotensive level in 70%, post operative 24- hour urine VMA and metanephrine were found to be within normal levels and significantly decreased in the major of cases. In conclusion, CT,US and MRI have good diagnostic results in preoperative localization of pheochromocytoma.and it is safe to operate pheochromocytoma with compatible preoperative preparation. Also, it is important to closely follow up with recurring or metastasis in pathologically proven malignant pheochromocytoma.