PURPOSE: Laparoscopic adrenalectomy (LA) has become the standard treatment for benign adrenal neoplasm because of the procedure's minimal invasiveness and the patients' earlier recovery. The aim of this study was to evaluate the safety and effectiveness of laparoscopic adrenalectomy for treating pheochromocytoma. METHODS: 19 Operations were performed between March 1993 and July 2004 at Kyung-Hee medical center for treating pheochromocytoma, and the diagnosis was confirmed by the postoperative pathology. There were 5 cases treated with LA and 14 cases treated with open adrenalectomy (OA). The various clinical parameters (tumor location, tumor size, first oral feeding, hospital stay, hemodynamic change and operation time) were compared between the LA and OA procedures, retrospectively. RESULTS: The location of the tumor was 2 : 2 : 1 (left : right : extra-adrenal) in the LA group and 9 : 3 : 2 (left : right : both) in the OA group. The mean tumor size (cm) was 5.4 in the LA group and 6.3 in the OA group. The mean operation time (minutes) was 219 in the LA group and 202 in the OA group. The resumption of liquid diet (days) was 2.2 in the LA group and 3.0 in the OA group (P=0.037). The postoperative hospital stay (days) was 6.3 in the LA group and 8.5 in the OA group. The mean number of intraoperative hypertensive crisis was 1.42 in the LA group and 1.40 in the OA group. The number of cases requiring intraoperative transfusion was 2 of 5 in the LA group and 2 of 15 in the OA group. The use of antihypertensives (number of times) was 1.42 in the LA group and 1.40 in the OA group. The mean highest BP (mmHg) was 162 in the LA group and 165 in the OA group. CONCLUSION: Laparoscopic adrenalectomy for treating pheochromocytoma is a safe and effective procedure that provides the benefits of a minimally invasive approach.
Adrenal Gland Neoplasms ; Adrenalectomy ; Antihypertensive Agents ; Diagnosis ; Diet ; Hemodynamics ; Length of Stay ; Pathology ; Pheochromocytoma* ; Retrospective Studies

PURPOSE: We report herein 119 patients with pheochromocytoma at our institute over the last 23 years. MATERIALS AND METHODS: Between 1986 and 2009, 119 patients were diagnosed with pheochromocytoma at our institute. We reviewed the medical records of these patients. RESULTS: Of 119 patients, 45 were male and 74 were female, and mean age was 43.83 +/- 13.49 years. Forty-three patients (36.1%) were diagnosed incidentally, and 8 patients (6.7%) were found to have familial pheochromocytoma. The mean dimension of the tumors was 5.89 +/- 3.18 cm. 4 patients had bilateral tumors; three of these patients were found to have familial pheochromocytoma and 1 patient was diagnosed with malignant pheochromocytoma. A total of eight patients (6.7%) were found to have malignant pheochromocytoma. In 1 patient, metastasis to a lymph node was found at the time of diagnosis. Metastases were found at a mean of 49 +/- 25.83 (6-75) months after surgery in the other seven patients. 6 patients died of malignant pheochromocytoma at a mean of 31 +/- 28.71 months (1-81) after diagnosis, and the other 2 patients survived for 15 and 24 months, respectively. CONCLUSION: Approximately 35% of patients with pheochromocytoma are diagnosed incidentally, and the number of detected cases is increasing. Although familial pheochromocytoma was found only in 6.7% of the patients, genetic testing should be considered in all patients, especially in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors. Given that malignant pheochromocytomas are frequently diagnosed during the follow-up period, long-term follow-up is necessary to confirm the absence of recurrence or metastasis.
Adolescent ; Adrenal Gland Neoplasms/diagnosis/pathology ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Pheochromocytoma/*diagnosis/pathology ; Young Adult

Pheochromocytoma is a rare cause of hypertension in children. Hypertension is one of the common reasons of posterior reversible encephalopathy. Intracerebral hemorrhage is a serious and unexpected complication of hypertensive encephalopathy due to pheochromocytoma, and very rarely seen in the childhood. Intracerebral hemorrhages should be searched if there are hypertensive reversible signal changes on the brain. Susceptibility weighted imaging (SWI) is a more sensitive method than conventional MRI when demonstrating cerebral microhemorrhagic foci. This is the first report of SWI findings on intracerebral hemorrhages in basal ganglia, brain stem and periventricular white matter due to hypertensive encephalopathy in a child with pheochromocytoma.
Adolescent ; Adrenal Gland Neoplasms/*complications/diagnosis ; Brain/*pathology ; Diagnosis, Differential ; Female ; Humans ; Hypertensive Encephalopathy/*diagnosis/etiology ; Magnetic Resonance Imaging/*methods ; Pheochromocytoma/*complications/diagnosis

OBJECTIVETo evaluate the CT and MR findings of retroperitoneal ectopic pheochromocytoma.

METHODSTo analyze retrospectively the CT and MR images of 32 patients with retroperitoneal ectopic pheochromocytoma proved by pathology.

RESULTSThe lesions (benign=28, malignant=4) were located in the anterior pararenal space (ARS) (n=12), the perirenal space (PS) (n=13) and the posterior pararenal space (PRS) (n=7). The tumors showed heterogeneous density on unenhanced CT (n=25). Among the 23 cases with enhanced CT imaging, 19 cases had marked contrast and 4 had mild contrast. The enhancement patterns included whole enhancement (n=9), solid area enhancement (n=12), peripheral enhancement (n=1), and spotted enhancement (n=1). The tumors had heterogeneous signal on unenhanced MR (n=23), and usually showed enhancement at arterial, portal and delayed phases on 22 enhanced MR, while cystic area with no enhancement. The lesions usually had cystic changes (n=18), septa (n=16), vessels inside (n=9), hemorrhage (n=3), and calcification (n=3). Besides that the morphology had statistical significance (P=0.013), other indexes had no statistical significance (P>0.05) in differential diagnosis of benign and malignant retroperitoneal ectopic pheochromocytomas.

CONCLUSIONSRetroperitoneal ectopic pheochromocytomas have some CT and MR features, usually revealed as an oval mass, growing along the paravertebral axis, and often with cystic changes. Those signs combined with hypertension and elevated catecholamine level may lead to a correct diagnosis.

Adrenal Gland Neoplasms ; diagnostic imaging ; pathology ; Calcinosis ; Diagnosis, Differential ; Humans ; Hypertension ; Image Enhancement ; Pheochromocytoma ; diagnostic imaging ; pathology ; Retroperitoneal Space ; Retrospective Studies ; Tomography, X-Ray Computed

Paraganglioma in pregnancy is an extremely rare condition and its diagnosis is often delayed because the clinical symptoms can mimic those of preeclampsia or gestational hypertension. Here, we report the case of a 32-year-old, gravida 2, para 1 woman who presented with severe headache, palpitation, and sweating at 37 weeks' gestation. Although emergent cesarean section was performed on the assumption of severe preeclampsia, blood pressure fluctuated and heart rate remained tachycardiac. We suspected that she might have thromboembolic lesion in the chest or pheochromocytoma. Chest and abdominal computed tomography revealed a 4 cm mass in the left para-aortic space. Serum and urinary catecholamine levels were found to be significantly increased. She underwent laparoscopic mass removal and the pathology confirmed paraganglioma. When typical paroxysmal hypertension is accompanied by headache, palpitation, and sweating during pregnancy, adrenal tumors should be considered.
Adult ; Blood Pressure ; Cesarean Section ; Diagnosis ; Female ; Headache ; Heart Rate ; Humans ; Hypertension ; Hypertension, Pregnancy-Induced ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Pathology ; Pheochromocytoma ; Pre-Eclampsia* ; Pregnancy ; Sweat ; Sweating ; Thorax

Pheochromocytoma is a rare disorder and functioning tumor composed of chromaffin cells that secrete catecholamines. Patients with a pheochromocytoma 'crisis' have a high mortality in spite of aggressive therapy. We present a case with a severe acute catecholamine cardiomyopathy presenting ST segment elevation with cardiogenic shock after hemorrhage into a left suprarenal tumor. Intra-aortic balloon pump (IABP) support, combined with inotropic therapy, was performed. However, the patient deteriorated rapidly and was unresponsive to a full dose of inotropics and IABP. We decided to apply extracorporeal membrane oxygenation (ECMO) device for the patient. His clinical state began to improve 3 days after ECMO. After achieving hemodynamic stabilization, he underwent successful laparoscopic left adrenalectomy. He needed no further cardiac medication after discharge.
Adrenal Glands/pathology ; Adult ; Cardiomyopathies/*diagnosis/*etiology ; Catecholamines/*adverse effects ; Coronary Angiography/methods ; Diagnosis, Differential ; Electrocardiography/methods ; Extracorporeal Membrane Oxygenation/*methods ; Humans ; Intra-Aortic Balloon Pumping ; Male ; Myocardial Infarction/*diagnosis ; Pheochromocytoma/*therapy ; Time Factors ; Tomography, X-Ray Computed/methods

Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes* ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis* ; Norepinephrine ; Pathology ; Pheochromocytoma* ; Recurrence ; Spine*

Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis ; Norepinephrine ; Pathology ; Pheochromocytoma ; Recurrence ; Spine

PURPOSE: Pheochromocytoma is a catecholamine-secreting tumor that arises from the chromaffin cells of the sympathoadrenal system and presents with the typical symptoms of palpitation, diaphoresis, head ache, and hypertension. Some pheochromocytoma may be discovered during computed tomography of the abdomen while evaluating the abdomen and may be clinically unsuspected before surgery. The aim of this study was to analyze the clinical presentation of pheochromocytoma and to identify the charac teristics of clinically unsuspected pheochromocytomas. METHODS: From June 1995 to October 1999, 34 histopathologically confirmed pheochromocytomas were diagnosed at Samsung Medical Center, Seoul, Korea. The records of all 34 patients, who had undergone a surgical resection for pheochromocytoma and paraganglioma, were reviewed for demographic information, presenting signs and symptoms, bio chemical profiles that led to the laboratory diagnosis of pheochromocytoma, tumor localization studies, associated diseases, preoperative preparation, surgical procedures, and tumor pathology. RESULTS: The most prominent symptoms of pheochromocytomas was palpitation, which was present in 21 (62%) of the 34 cases. Hypertension, headache, and sweating were in 18 (53%), 17 (50%), and 9 (27%) patients, respectively. In 14 (41%) of the 34 patients, the pheochromocytoma were incidentally found exhibiting none of the typical symptoms. Eight (24%) were clinically unsuspected and were undiagnosed prior to surgery. A preoperative diagnosis of either retroperitoneal or pancreatic mass was made in six cases (18%), and a diagnosis of a nonfunctioning adrenal mass was made in two (6%). These eight patients underwent surgery without any preoperative pharmacological treatment. Sporadic cases accounted for 85% of the patients (29 patients), and associated conditions included MEN 2A in four (12%) and MEN 2B in one (3%).The sensitivities of the urinary evaluation were metanephrine 85%, norepinephrine 81%, epinephrine 73%, and vanillylmandelic acid (VMA) 73%. The combination of urinary metanephrine and VMA had a diagnostic sensitivity of 92%. The sensitivities of the localization modalities were CT 100%(33/33), MRI 100% (3/3). USG 94.8% (18/19), MIBG 70% (7/10). Four of the 8 patients who received no preoperative preparation and 10 of the 26 patients who received phenoxybenzamine daily for at least 2 weeks) had intraoperative hypertension or arrythmia. One patient received a laparoscopic adrenalectomy. The rest of the patients underwent surgery through open transabdominal incision. The mean tumor diameter of the malignant pheochromocytomas was 10.8 cm (range from 4.5 to 16 cm), and that of the benign pheochromocytoma was 6.1 cm (range from 3 to 10 cm) showing marked differences between two groups. This suggests that malignant tumors tend to be larger than benign tumors. Of the 4 malignant cases, recurrent disease occurred at the site of the original tumor in 1 patient, metastasis didnt occur in any case. CONCLUSION: High index of suspicion for a pheochromocytoma must be maintained in patients with retroperitonal and abdominal masses or isolated hypertension and in patients with multiple endocrine neoplasms. The readily available, sensitive assays for plasma and urinary catecholamines and their me tabolites and the newly developed modern imaging techniques, such as CT and I-131-MIBG, when used together, may help inmaking correct diagnosis of pheochromocytoma in patients without paroxysmal symptoms or hypertension.
3-Iodobenzylguanidine ; Abdomen ; Adrenalectomy ; Arrhythmias, Cardiac ; Catecholamines ; Chromaffin Cells ; Clinical Laboratory Techniques ; Diagnosis ; Epinephrine ; Head ; Headache ; Humans ; Hypertension ; Korea ; Magnetic Resonance Imaging ; Metanephrine ; Multiple Endocrine Neoplasia ; Multiple Endocrine Neoplasia Type 2a ; Multiple Endocrine Neoplasia Type 2b ; Neoplasm Metastasis ; Norepinephrine ; Paraganglioma ; Pathology ; Phenoxybenzamine ; Pheochromocytoma* ; Plasma ; Seoul ; Sweat ; Sweating ; Vanilmandelic Acid