Left-sided giant adrenal myelolipoma secreting catecholamine.

Adrenal myelolipoma (AML) is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA), a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

The predictive value of urinary vanillylmandelic acid testing in the diagnosis of phaeochromocytoma at the University Hospital of the West Indies / El valor predictivo de la prueba del ácido vanilmandélico en orina para el diagnóstico de la feocromositoma en el Hospital Universitario de West Indies

OBJECTIVE: To investigate the positive predictive value (PPV) of urinary vanillylmandelic acid (VMA) testing in the diagnosis of phaeochromocytoma and to describe the features associated with phaeo chromocytoma at the University Hospital of the West Indies (UHWI). SUBJECTS AND METHODS: There were 551 VMA tests performed from January 2003 to June 2009 and 122 tests in 85 patients were elevated (ie > 35 µmol/24 hr). The study patients were categorized as: (i) 'surgical' (5 patients who underwent surgery) or (ii) 'non-surgical' (remaining 80 patients). Forty medical charts (out of 85) were reviewed using a standardized data extraction form. RESULTS: The median age for patients in the non-surgical group (with charts reviewed, n = 35) was 36 years (range 9-70) and the median VMA was 43 µmol/24 hr (IQR 38-51). Of these patients, 83% had one or no symptom typical of phaeochromocytoma. In the surgical group the median VMA was 58 µmol/24 hr (IQR 44-101); phaeochromocytoma was confirmed histologically in 3 patients, all of whom had several symptoms typical of catecholamine excess. VMA testing had a PPV of 8%, specificity of 79% and sensitivity of 100%. CONCLUSIONS: VMA testing at UHWI has poor specificity and high sensitivity. These results contrast with international data showing that VMA testing is poorly sensitive but highly specific. The use of assays with higher specificity (egplasma or urinary metanephrines) may represent a more cost-effective approach to biochemical screening at UHWI.

OBJETIVO: Investigar el valor predictivo positivo (VPP) de las pruebas del ácido vanilmandélico urinario (VMA) en el diagnóstico de la feocromositoma y describir las características asociadas con la feocromositoma en el Hospital de la Universidad de West Indies (HUWI). SUJETOS Y MÉTODOS: Se realizaron unas 551 pruebas de VMA de enero de 2003 a junio de 2009, y 122 de las pruebas en 85 pacientes tuvieron resultados elevados (ie > 35 µmol/24 hr). Los pacientes del estudio fueron clasificados como: (i) "quirúrgicos" (5 pacientes que se sometieron a cirugía) ó (ii) "no quirúrgicos" (los 80 pacientes restantes). Se revisaron cuarenta historias clínicas (de 85) mediante un formulario estandarizado de extracción de datos. RESULTADOS: El promedio de edad de los pacientes en el grupo no quirúrgico (con historias clínicas, n = 35) fue de 36 años (rango 9-70) y la mediana VMA fue 43 µmol/24 h (IQR 38-51). De estos pacientes, 83% tenían uno o ningún síntoma típico de la feocromositoma. En el grupo quirúrgico la mediana VMA fue 58 µmol/24 h (IQR 44-101). La feocromositoma fue confirmada histológicamente en 3 pacientes, cada uno de los cuales presentó síntomas típicos de exceso de catecolaminas. Las pruebas de VMA tuvieron un VPP de 8%, una especificidad de 79%, y una sensibilidad de 100%. CONCLUSIONES: Las pruebas de VMA en HUWI poseen pobre especificidad y alta sensibilidad. Estos resultados contrastan con los datos internacionales que muestran que la prueba de VMA es pobremente sensible pero altamente específica. El uso de ensayos con mayor especificidad (por ejemplo, metanefrinas plasmáticas o urinarias) puede representar un método costo-efectivo a la hora de realizar el pesquisaje bioquímico en HUWI.

Role of specific clone of lymphocytes, [CD4[+] and CD8[+]] and the sympathetic activity in pathophysiology of altered immunity in ischemic stroke

Ischemic stroke is one of the major causes of high morbidity and mortality allover the world. The understanding of the pathophysiology of post-ischemic immune response is very limited. Cerebral ischemic stroke affects the normally well-balanced interplay of the 2 super systems: the nervous and the immune system. T-cell lymphocytes, [CD4[-], CD8[-]], may contribute to altered immunity associated with stroke. Increased sympathetic activity during ischemic stroke may have a role in altered lymphocytes function. The present study investigated the contribution of CD4[-] and CD8[-] and the sympathetic activity in altered immunity in ischemic stroke. Determination of CD4[-] and CD8[-] percentage in patient's blood was done by flowcytometry. Evaluation of sympathetic activity done by measuring urinary vanilmandelic acid [VMA] levels by spectrophotometry. The study also correlated the changes of these parameters with specific clinical and diagnostic variables in stroke. The study showed that CD4[-] and CDS percentage were significantly lower [p<0.001], while CD4[-] /CD8[-] ratio was significantly higher [p<0.001] in patients than controls. There was also significantly increased [p<0.001] mean urinary VMA excretion levels [mg/day] in patients compared to control group. Significantly lower CD4[-]% and CD4[-] /CD8[-] ratio and higher CD8[-]% were found in patients with recurrent stroke or history of transient ischemic attacks, progressive strokes and large size of infarction in comparison to other comparable patients. The study indicated that patients with ischemic strokes may have altered immunity and sympathetic over-activity which may be one of the mechanisms by which modulation of immune response can be induced after stroke. This brain-immune interaction after stroke may have protective, destructive, or regenerative effects in the brain, therefore the development of therapeutic strategies is not straightforward, and must take all these factors into consideration

Pheochromocytoma Damanhour teaching hospital experience

Pheochromocytoma has low incidence of 2-8 case per million persons annually, but it is a lethal disease when it is functioning and undiagnosed. It constitutes 0.1% to 1% of hypertensive patients that is curable by surgery. Our study included twelve patients operated upon at our institute in the last 3 years. Two cases were none functioning; one case of them was presented by internal hemorrhage due to tumor rupture. There were 8 males and 4 females. One child has 10 years old. Two cases were extra adrenal. Three patients presented to us with a mass without hypertension and diagnosed post operatively. Preoperative preparation was done using alpha blocker and beta blocker until the hypertension became normalized. lntra operative and post operative control of blood pressure with caring our patients in ICU till the condition became stable. No mortality or significant morbidity had occurred. Post operative normalization of blood pressure had been achieved in all patients

Neuroblastoma Originating from Extra-abdominal Sites: Association with Favorable Clinical and Biological Features

Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (+/-95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4+/-10.6% vs. 69.4+/-9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.

Neuroblastoma Originating from Extra-abdominal Sites: Association with Favorable Clinical and Biological Features

Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (+/-95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4+/-10.6% vs. 69.4+/-9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.

Effect metformin on urinary VMA levels in type II diabetic subjects

The purpose of this study was to observe the effect of metformin on 24-hours urinary VMA levels in newly diagnosed untreated type 2 diabetic subjects. The study consisted of four weeks for each participant with weekly follow up visits. Samples were collected at 0800-0900 hours after over night fast. Study was conducted in the Department of Pharmacology and Therapeutics, BMSI, JPMC, Karachi. Total duration of study was six months. Fifteen newly diagnosed untreated type 2 diabetics, with fasting plasma glucose levels >/= 126 mg/dl on two occasions and/or postprandial glucose levels >/= 200 mg/dl were enrolled in the study. Patients with concurrent illness or diabetic complications were excluded. Metformin was started from 500 mg/day and titrated at weekly intervals according to glycaemic control and the subjects tolerance to the drug. A 24-hour urinary VMA was assessed at day - 0 [before metformin therapy] and day - 28 [4 weeks after metformin therapy] by using VMA reagent kit of Biosystems Spain on Spectronic -21 spectrophotometer USA. Metformin caused highly significant [P < 0.001] reduction in mean fasting plasma glucose from 233.33 +/- 15.62 mg /dl on day-0 to 151.53 +/- 6.02 mg/dl on day - 28, and a significant [P < 0.01] decrease in 24 - hour urinary VMA levels from 5.18 + 0.50 mg / 24 hours on day-0 to 3.32 + 0.28 mg / 24 hours on day-28. Our results indicate that metformin causes highly significant reduction in fasting plasma glucose and a significant decrease in 24 - hour urinary VMA levels in newly diagnosed untreated type 2 diabetic subjects

Metastatic malignant Pheochromocytoma of adrenal gland

Malignant pheochromocytoma is a rare disease with a high mortality. Surgical removal is usually curative while chemotherapy and radiotherapy are palliative treatments. A case of metastatic malignant pheochromocytoma of the right adrenal gland is presented who had fluctuating blood pressure with episodic headache and raised urinary VMA levels. Thoraco-abdominal resection of the tumour resulted in control of blood pressure and patient was asymptomatic at 4 months follow-up

Case report of parathyroid carcinoma with raised urinary catecholamines: a diagnostic dilemma.

Parathyroid carcinoma is often misdiagnosed as thyroid carcinoma clinically and also with fine needle aspiration cytology. Moreover in cases misdiagnosed as thyroid carcinoma pre operatively, raised urinary catecholamines may mislead to a diagnosis of MEN2 A. We report a case of a patient admitted to the surgery department ofour hospital with a swelling in the thyroid region, raised urinary catecholamines and urinary VMA levels, with hypercalciuria and elevated parathyroid hormone levels. It was clinically suspected as thyroid carcinoma with parathyroid adenoma and associated MEN 2A syndrome.

Pheochromocytoma - an institutional experience

The present study envisages evaluating the clinical presentation, diagnosis, management, with particular reference to pharmacological and anesthetic considerations, and outcome in patients with pheochromocytoma. A retrospective study. The study was conducted at the Department of Surgery, Kasturba Medical College Hospital, Manipal, India from January 1, 1996 to December 31, 2003. Seventeen patients diagnosed to have pheochromocytoma were included for this study. Our assessment of age at presentation, sex, presenting complaints with details of hypertension [HT], diagnostic tests, surgical procedure and follow-up was based on patient records. Peak incidence was observed in 40-50 years age group, with male preponderance. All the patients had diastolic HT of more than 100 mm of Hg at presentation. Systolic blood pressures [SBP] were between 150 mm Hg to 230 mm Hg, with mean of 170 mm Hg; nine patients presented with persistent HT and seven had paroxysmal HT. One patient infact presented with hypotension due to septic shock and was incidentally detected to have an adrenal tumor. Computed tomography [CT] scan and urinary levels of vanillylmandelic acid [VMA] were mainstay of diagnosis. These patients had adequate preoperative control of HT. All patients underwent exploration of the tumor by an extraperitoneal approach. 70% tumors were on the right and 30% were on the left side. Histopathologically, all the tumors were benign except for one, which was malignant. An average of three to five units of blood transfusion was required and mean operating time was 4.5 hours. Six patients had extensive intra-operative blood pressure [BP] fluctuation, but were adequately managed by the anesthetic team. One patient with post-operative pulmonary edema died in our series; rest all the patients are on regular follow-up, till date and doing well. Pheochromocytoma is an important cause of endocrine HT. Elevated urinary VMA is diagnostic of this tumor with imaging studies used to localize the tumor. Adrenalectomy results in complete cure of HT with an excellent long-term outcome

Effect of work- related stress on secondary school teachers

This work aimed at investigating the effect of job stress on the level of some stress hormones as well as on the blood pressure and heart rate among a group of secondary school female teachers. The study population was composed of 42 secondary school female teachers who were divided into a group of high tasks [n=30] and a group of low task [n=12]. The control group was composed of 43 matched females. Every subject was examined using a medical questionnaire, measurement of radial pulse and blood pressure in addition to measurement of serum cortisol and urinary level of vallinylmandelic acid [VMA], the main catecholamine metabolite. There was a statistically insignificant increase in the prevalence of stress related symptoms such as fatigue, low back pain and gastrointestinal disturbances among the teachers' group compared to the controls. The mean levels of heart rate, systolic blood pressure, diastolic blood pressure, serum cortisol and urinary vallinylmandelic acid were significantly higher among the teachers of the high task group. Heart rate and blood pressure were significantly correlated with the age and duration of work. Being one of the stressful jobs, teaching is associated with an increase of the level of stress hormones [cortisol and catecholamines] with a consequent increase in the blood pressure and the risk of development of heart disease

Extraadrenal retroperitoneal paraganglioma causing malignant hypertension and permanent visual loss in an eleven-year-old-girl

We report a secretory paraganglioma in an eleven-year-old Trinidadian girl of East Indian descent who presented with malignant hypertension, proteinuria and modest elevation of urinary vanillylmandelic acid levels. The extraadrenal mass was surgically removed without complications. The patient has been normotensive without evidence of recurrence of disease but with persistent visual sequel two years later. This case reinforces the observation that extraadrenal retroperitoneal paragangliomas can be functionally very active and early computed tomography or other imaging procedure is necessary for rapid noninvasive evaluation, timely surgery and prevention of permanent morbidity.

Feocromocitoma vesical / Vesical pheochromocytoma

Feocromocitoma vesical é um tumor raro, representando 1 por cento dos feocromocitomas extra-adrenais. Foram descritos 117 casos na literatura. É apresentado o caso de uma jovem com hipertensäo arterial sem causa aparente controlada com enalapril. Três anos depois, realizou-se ultrasonografia para avaliaçäo de queixa abdominal de origem näo-urinária, quando diagnosticou-se tumor vesical. Por näo se tratar de tumor urotelial, optou-se por cistectomia parcial cujo exame anatomopatológico revelou feocromocitoma. Um ano após a cirurgia, a paciente apresentava níveis pressóricos de 140 x 100 mmHg, sendo controlados com 1 mg de prazosim

Separación de aminas biogénicas mediante cromatografía líquida de alta resolución / High-performance liquid chromatographic determination of biogenic amines

En este trabajo se han compilado los distintos modos cromatográficos y sistemas de detección utilizados en la cromatografía líquida de alta resolución de aminas biogénicas. Se indican las características generales del intercambio catiónico, fase reversa, fase reversa de pares iónicos y cromatografía de partición con fase reversa de pares iónicos. También se analizan comparativamente la detección UV, detección fluorimétrica usando fluorescencia nativa o bien derivatización pre- y postcolumna y detección electroquímica de gran utilidad para esta extensa familia de compuestos. Se dan ejemplos de aplicación de interés en el campo bioquímico-clínico, con el análisis de ácido homovainillínico, ácido 3,4-dihidroxifenilacético y ácido 5-hidroxiindolacético en líquido cefalorraquídeo, metanefrinas, ácido 3,4-dihidroxifenilacético, catecolaminas, ácidos urinarios y 3-metoxi-4-hidroxifenilglicol en orina, catecolaminas y 3-metoxi-4-hidroxifenilglicol en plasma, catecolaminas, 3-metoxi-4-hidroxifenilglicol y otros neurotransmisores en cerebro de rata. Se discuten, también, los tratamientos previos requeridos especialmente para orina y plasma, así como las condiciones de conservación y su incidencia en los resultados obtenidos

Melanotic neuroectodermal tumor of infancy. Report of a case.

A case of melanotic neuroectodermal tumor of infancy involving the right maxillary alveolar ridge region of a 4 month old girl is reported. Clinical, histopathological and laboratory findings supported the diagnosis. This case had high levels of urinary excretion of vanilmandelic acid (2.8 mg/24 hrs.) and serum alpha-fetoprotein (210 mg/ml); which are characteristic of this tumor.

Urinary vanilmandelic acid determination using column chromatography.

A simple method for determination of urinary vanilmandelic acid, VMA, using column chromatography was described. The interfering substances in the urine were eliminated by passing the urine through a Dowex-1 x 2, 50-100 mesh, column and washing the column with distilled water. The VMA was eluted from the resin using K2CO3-containing 3.0 mol/l NaCl solution and determined spectrophotometrically after periodate oxidation reaction. This method yielded a standard curve which was linear up to 50 mg/l. The recoveries of the method as determined by addition technique ranged from 92.1 to 100.4 per cent with an average of 97.3 per cent. The method yielded satisfactory precisions with the coefficients of variation (C.V.) of less than 5.50 and 6.95 per cent for within-run and between-run experiments respectively. The method was sensitive to the concentrations of 1.2 mg/l with recovery of 92.5 per cent and 2.6 mg/l with the recovery of 109.9 per cent for the standard and urinary VMA respectively. No interfering effects were found from epinephrine and norepinephrine (up to 2,000 micrograms/l), acetylsalicylic acid (up to 50 mg/l) and homovanillic acid (up to 10 mg/l) added to the pooled urine. The correlation coefficients (r) of 0.909 (n = 100) and 0.905 (n = 100) were obtained when the urinary VMA values determined by the proposed and Pisano's methods and expressed as mg/l and mg/g creatinine were compared respectively. The column could be reused at least 5 times. The technique is suitable for any routine clinical laboratory.

Pheochromocytoma: a twenty year experience at the University Hospital

Durante los últimos 20 años (1970-90), hemos tenido 24 pacientes con feocromocitomas: 19 diagnosticados clínicamente y 5 en autopsia. Sus edades variaron de 17 a 74 años (promedio de 43.2) y el número de varones (14), fueron mayor que el de las hembras (n=10). La mayoría de los sujetos tuvieron síntomas (95%) consistentes en la tríada típica de dolor de cabeza, palpitaciones y sudoración. El hallazgo más frecuente fue la hipertensión (95%), la cual fue sostenida en 60% y paroxística en 35% de los casos. Un 25% (n=6) de casos tenía tumores bilaterales, todos familiares. Quince eran tumores adrenales solitarios (63%) y 3 (12.5%) eran extra-adrenales: 2 en el abdomen (órgano de Zuckerbandl) y uno intratorácico (paraganglioma del atrio derecho del corazón). De los 6 casos familiares, 4 estaban asociados a la enfermedad de Von Hipper-Lindau (VHL), mientras que 2 tenían el síndrome de neoplasia múltiple tipo 2 (MEN-II. Todos los casos familiares eran bilaterales y se hallaban en las adrenales. No hubo tumores malignos. Entre los 19 casos diagnosticados pre-operatoriamente, fueron positivas las pruebas en orina del ácido vanililmandélico (VMA) en 95% y la de catecolaminas totales en 100%. Se logró visualizar los tumores pre-operatoriamente por medio de tomografía computadorizada (CT) o por imagen de resonancia magnética (MRI) en 62% de los pacientes más recientes. En 5 casos más antiguos sólo se hizo el diagnóstico post-mortem: 3 murieron de hemorragias cerebrales, 1 de infarto del pons y otro en fallo cardíaco congestivo. Hubo dos muertes post-operatorias y otra, 13 años post-operación, de carcinoma medular del tiroides. De los 19 operados 13 (68% fueron curados. Se concluye que los feocromocitomas aún retienen un considerable potencial de morbilidad y alguna mortalidad. Estos raros tumores constituyen un reto diagnóstico clínico, pero conllevan una gran satisfacción para el médico que los atiende