Neuroblastoma is the most common extracranial solid tumor in children, and accompanies various clinical symptoms including hypertension. Hypertension is associated with catecholamines secreted from the tumor, and is usually not severe. We report one case of malignant hypertension with cardiac failure in a patient with adrenal neuroblastoma, successfully treated with adrenalectomy. A 3 year-old boy complained of protrusion of the chest wall. Physical examination revealed severe hypertension with cardiac failure. The levels of metabolites of catecholamine were increased in blood (norepinephrine >2000 pg/mL) and urine (norepinephrine 1350.5 ug/day). Abdominal CT showed a 7 cm-sized solid mass arising from the right adrenal gland. After stabilizing the hemodynamics with oral phenoxybenzamine, right adrenalectomy was performed. Pathological diagnosis was a ganglioneuroblastoma. The hypertension and cardiac failure were resolved after tumor removal.
Adrenal Glands ; Adrenalectomy ; Catecholamines ; Child ; Ganglioneuroblastoma ; Heart Failure ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Malignant ; Neuroblastoma ; Phenoxybenzamine ; Physical Examination ; Polyenes ; Thoracic Wall

Pheochromocytoma is a catecholamines secreting tumor that usually appears in the adrenal medulla, sympathetic ganglia and extra-adrenal chromaffin tissue. About 10% of this disease is detected in the extra-adrenal chromaffin tissue which is called paraganglioma. The three major clinical symptoms of pheochromocytoma are headache, syncope and hypertension. Approximately 0.1% of hypertensive patients have pheochromocytoma. The extra-adrenal paraganglioma is found in abdominal sympathetic nerve ganglion or Zukerkandle's organ with great frequency, but it also appears, albeit rare, in the cervical ganglion, thoracic cavity, bladder, and pelvic cavity. Some cases of paraganglioma in bladder are being reported internationally, but domestic reports are rare. We report a case of paraganglioma originating in the urinary bladder of patient who visited with acute renal failure associated with malignant hypertension. We treated him with phenoxybenzamine and later with partial cystectomy. High blood pressure was well controlled and acute renal failure was resolved.
Acute Kidney Injury* ; Adrenal Medulla ; Catecholamines ; Cystectomy ; Ganglia, Sympathetic ; Ganglion Cysts ; Headache ; Humans ; Hypertension ; Hypertension, Malignant ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Phenoxybenzamine ; Pheochromocytoma ; Syncope ; Thoracic Cavity ; Urinary Bladder*

Pheochromocytoma during pregnancy is very rarely discovered, and this condition is life threatening to both mother and fetus. Maternal and fetal survival depend a lot on an early diagnosis, a correct medical therapy and a correct timing of delivery and surgery. We describe a case of pheochromocytoma during pregnancy. A patient was transferred to our hospital during gestational week 24 with severe hypertension, generalized edema. Pheochromocytoma caused by a left adrenal mass was diagnosed. The patient was treated with titrated dose of phenoxybenzamine, propranolol, and intravenous nicardipine, but intermittent severe hypertension was developed. At 33 weeks gestation, fetal distress was developed, and emergent cesarean section was done. Two weeks after delivery, she underwent a successful left adrenalectomy. Two months after surgery, all antihypertensive medications were discontinued and her blood pressure have remained normal range.
Adrenalectomy ; Blood Pressure ; Cesarean Section ; Early Diagnosis ; Edema ; Female ; Fetal Distress ; Fetus ; Humans ; Hypertension ; Mothers ; Nicardipine ; Phenoxybenzamine ; Pheochromocytoma* ; Pregnancy* ; Propranolol ; Reference Values

OBJECTIVEThe aim of the present study is to compare the effects of two alpha1-adrenoceptor antagonist terazosin and alfuzosin together with one alpha-adrenoceptor antagonist phenoxybenzamine on androgen-independent prostate cancer cell lines PC-3 and DU145.

METHODSTwo androgen- independent cell lines, PC-3 and DU145, were used to determine the cell viability, colony-forming ability as well as cell cycle characteristics after exposure to these three drugs.

RESULTSThis study showed that terazosin inhibited not only prostate cancer cell growth but also colony-forming ability, which is the main target of clinical treatment. On the other hand, alfuzosin and phenoxybenzamine have no effect on cell viability and colony forming ability of PC-3 and DU145. In addition, the terazosin inhibits cell growth through G(1) phase cell cycle arrest.

CONCLUSIONThis study provided the evidence that alpha1-adrenoceptor antagonist terazosin may have a therapeutic potential in the treatment of advanced androgen-independent prostate cancer.

Adrenergic alpha-Antagonists ; pharmacology ; Antineoplastic Agents ; pharmacology ; Cell Cycle ; drug effects ; Cell Line, Tumor ; Cell Survival ; drug effects ; Dose-Response Relationship, Drug ; Humans ; Male ; Phenoxybenzamine ; pharmacology ; Prazosin ; administration & dosage ; analogs & derivatives ; pharmacology ; Prostatic Neoplasms ; pathology ; Quinazolines ; pharmacology

Pheochromocytoma during pregnancy is very rare tumor, and this condition is life threatening to both mother and fetus. We describe a case of pheochromocytoma antenatally diagnosed at the midtrimester in gestation. A patient was transferred to National Medical Center during gestational week 24 with severe hypertension, generalized edema. Pheochromocytoma caused by a left adrenal mass was diagnosed. The patient was treated with titrated dose of phenoxybenzamine, propranolol, and intravenous nicardipine, but, intermittent severe hypertension was developed. At 33 weeks gestation, fetal distress was developed, and emergent c-sec was done. Two weeks after delivery, she underwent a successful left adrenalectomy. Two months after surgery, all antihypertensive medications were discontinued and her blood pressure have remained normal range.
Adrenalectomy ; Blood Pressure ; Edema ; Female ; Fetal Distress ; Fetus ; Humans ; Hypertension ; Mothers ; Nicardipine ; Phenoxybenzamine ; Pheochromocytoma* ; Pregnancy Trimester, Second ; Pregnancy* ; Propranolol ; Reference Values

OBJECTIVETo discuss the standards for clinical functional gradation and preoperative preparation of pheochromocytoma.

METHODSAccording to the preoperative clinical manifestations and 24 hr urine catecholamine, 172 cases of pheochromocytomas were divided into 4 grades. Functionary grade 0 including 22 patients was given no volume expansion. Functionary Grade 1 consisted of 17 cases, 10 of which were given phenoxybenzamine orally 5 - 10 mg/d for 1 week (therapeutic group), the rest were control group; the results were analyzed by the chi(2) test. Functionary Grade 2 including 120 patients had phenoxybenzamine orally 30 - 240 mg/d for 4 weeks, hemodynamics and microcirculation image were standards for evaluating volume expansion. Functionary Grade 3 consisted of 13 patients including 1 with acute heart failure, 2 and 10 patients with past history of cerebral hemorrhage and hypertensive crisis respectively, they were treated with enough phenoxybenzamine and other emergent measures.

RESULTSThe perioperative blood pressure of Functionary Grade 0 had no fluctuation. The blood pressure of therapeutic group of Functionary Grade 1 had small range fluctuation (< 20 mm Hg), that of the control group was large (> 40 mm Hg). Chi(2) = 13.12, P < 0.01. The hemodynamics of Functionary Grade 2 and Grade 3 recovered within 24 hours postoperatively and no complications occurred.

CONCLUSIONAccording to the function of pheochromocytoma, it is safe and efficient to use different preoperative preparations. Hemodynamics and microcirculation image are golden standards for evaluating preoperative preparations.

Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adult ; Blood Pressure ; Dopamine ; blood ; Epinephrine ; blood ; Female ; Humans ; Male ; Monitoring, Intraoperative ; Norepinephrine ; blood ; Phenoxybenzamine ; therapeutic use ; Pheochromocytoma ; diagnosis ; surgery ; Premedication ; Preoperative Care ; standards ; Vasodilator Agents ; therapeutic use

BACKGROUND: Pheochromocytoma may arise within the adrenal medulla, or in other locations where sympathetic ganglia or chromaffin tissues are known to exist. Approximately 0.1% of hypertensive patients have pheochromocytoma. Most of this hypertension can be cured by surgical removal of the tumor, but lethal complication can develop if proper treatment is not prepared. Therefore, accurate diagnosis and preoperative preparation is very important. The objective of this study was to evaluate the clinical characteristics of pheochromocytoma and the blood pressure change following tumor removal. METHOD: The medical records of 45 patients [29 cases of intra-adrenal, 16 cases of extra-adrenal (paraganglioma)] diagnosed with of pheochromocytoma at Korea University Medical Center between 1991 and 2001 were reviewed. RESULTS: Twenty of the cases were male and 25 were female. The mean age of these patients was 43 years old. Hypertension, headaches, palpitations and impaired glucose tolerance were observed more frequently in cases of intra-adrenal tumor than in those of extra-adrenal. In the extra-adrenal group, abdominal pain, hypertension and nausea were more frequently observed. A biochemical study showed that the sensitivity of the test for catecholamines and metabolites in 24 hours urine was over 80%. For the localization of tumors we used abdominal CT and MIBG. The sensitivities of the CT and MIBG for the tumor localization were 97 and 91%, respectively. Blood pressures during the operations were effectively controlled by preoperative treatment with phenoxybenzamine (non-competitive, non-selective -adrenoreceptor antagonists). CONCLUSION: Surgical treatment cured 23 of the 30 cases of hypertension with pheochromocytoma. We have to take careful approaches in the care of the patient who may have pheochromocytoma, due to the various clinical signs and symptoms.
3-Iodobenzylguanidine ; Abdominal Pain ; Academic Medical Centers ; Adrenal Medulla ; Adult ; Blood Pressure ; Catecholamines ; Diagnosis ; Female ; Ganglia, Sympathetic ; Glucose ; Headache ; Humans ; Hypertension ; Korea ; Male ; Medical Records ; Nausea ; Paraganglioma ; Phenoxybenzamine ; Pheochromocytoma* ; Tomography, X-Ray Computed

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

MEN IIa is the rare disorder consisted of thyroid medullary carcinoma, pheochromocytoma, and hyperparathyroidism. We experienced the case in which 42 year-old male patient with thyroid medullary carcinoma and pheochromocytoma complicated by acute myocardial infarction. During the process of conventional treatment of acute myocardial infarction, paroxysmal hypertension occurred for several times. We sought for the cause of paroxysmal hypertension, and found pheochromocytoma by the radiologic imaging study and the biochemical study and we found the 4X4 cm sized neck mass by palpation. After stabilizing his blood pressure by the use of phenoxybenzamine, we removed the pheochromocytoma in right adrenal gland and the medullary thyroid cancer, by right adrenalectomy and total thyroidectomy respectively. Thereafter, his subjective symptoms and objective signs were improved. We report the case with review of literatures.
Adrenal Glands ; Adrenalectomy ; Adult ; Blood Pressure ; Carcinoma, Medullary ; Humans ; Hyperparathyroidism ; Hypertension ; Male ; Multiple Endocrine Neoplasia Type 2a ; Multiple Endocrine Neoplasia* ; Myocardial Infarction* ; Neck ; Palpation ; Phenoxybenzamine ; Pheochromocytoma ; Thyroid Gland ; Thyroid Neoplasms ; Thyroidectomy

We report a case in which a 63-year-old male patient with pheochromocytoma developed persistent hypotension during surgery despite rapid volume replacement and administration of vasopressors. The patient was prepared for surgery with phenoxybenzamine for 13 days. Anesthesia was induced with thiopental sodium and maintained with N2O, O2, and enflurane. Sodium nitroprusside (SNP) was initiated and titrated based upon intraarterial blood pressure. Hypertensive episode during tumor manipulation was effectively managed by increased infusion of SNP. After surgical removal of tumor, this patient developed profound hypotension, which was aggressively managed by intravenous administration of crystalloid and blood as well as dopamine and epinephrine. However, this hypotension was persistent and aggravated. Accordingly, Infusion of norepinephrine (Levophed(R))was started and then the patient recoverd from his hemodynamic aberrations. We conclude that the cause of the persistent hypotension was cumulative and residual effect of preoperative phenoxybenzamine. Therefore, norepinephrine should be readily available for the treatment of hypotension resistant to other pharmacologic interventions.
Administration, Intravenous ; Anesthesia ; Blood Pressure ; Dopamine ; Enflurane ; Epinephrine ; Hemodynamics ; Humans ; Hypotension* ; Male ; Middle Aged ; Nitroprusside ; Norepinephrine* ; Phenoxybenzamine ; Pheochromocytoma* ; Thiopental