Transient shock and myocardial impairment caused by phaeochromocytoma crisis.

A patient admitted to hospital after injury to the abdomen was found to have transient hypertension which was followed by profound hypotension. ST elevation developed and extensive myocardial akinesia was seen at echocardiography, but coronary angiograms at this stage were normal. After treatment with intravenous fluids and dopamine he progressively recovered normal cardiac function. A partly necrotic catecholamine secreting tumour was later removed from the abdomen and it is likely that a kick to the abdomen had damaged the tumour and the consequent release of catecholamine had triggered a phaeochromocytoma crisis.     

Neurogenic hypertension associated with an excessively high excretion rate of catecholamine metabolites

A 60 year old hypertensive patient suffered several cerebral infarctions. A phaeochromocytoma was suspected because the excretion rates of vanillylmandelic acid and its methoxy derivatives were raised and the patient had hypertensive crises. No tumour was found, however, by 131mI-iodobenzylguanidine scintigraphy and computed tomography of the abdomen. Moreover, the enhanced orthostatic plasma catecholamine response suggested that the high excretion rates of catecholamine metabolites were more likely to be caused by the syndrome of raised catecholamines after cerebrovascular accidents than a phaeochromocytoma. A phaeochromocytoma should not be diagnosed within several months of cerebral infarction without first excluding the possibility of a hyperadrenergic state induced by cerebral infarction.     

Neurogenic hypertension associated with an excessively high excretion rate of catecholamine metabolites.

A 60 year old hypertensive patient suffered several cerebral infarctions. A phaeochromocytoma was suspected because the excretion rates of vanillylmandelic acid and its methoxy derivatives were raised and the patient had hypertensive crises. No tumour was found, however, by 131mI-iodobenzylguanidine scintigraphy and computed tomography of the abdomen. Moreover, the enhanced orthostatic plasma catecholamine response suggested that the high excretion rates of catecholamine metabolites were more likely to be caused by the syndrome of raised catecholamines after cerebrovascular accidents than a phaeochromocytoma. A phaeochromocytoma should not be diagnosed within several months of cerebral infarction without first excluding the possibility of a hyperadrenergic state induced by cerebral infarction.     

Sequential ACTH and catecholamine secretion in a phaeochromocytoma

OBJECTIVE: We describe a patient with an ACTH-producing phaeochromocytoma who initially presented with hypercortisolism and normal catecholamine concentrations, followed by near-normalisation of ACTH secretion and massive catecholamine secretion. In vitro studies were carried out on the tumour to evaluate the interaction between the tumour cells and normal adrenal cortex. METHODS AND RESULTS: A 30-year-old man initially presented with severe hypercortisolism, biochemical evidence of ectopic ACTH production, a tumour in the right adrenal gland without a hyperintense signal on the T2-weighted images at magnetic resonance imaging (MRI) scanning, and normal urinary metanephrine concentrations. After 6 months, ACTH production had almost completely resolved, but the patient developed severe hypertension and excess catecholamines. At repeated MRI-scanning, the T2-weighted images showed a hyperintense signal, in agreement with the diagnosis of phaeochromocytoma. Although the initial T1-weighted images suggested bleeding in the adrenal tumour, no signs of bleeding were observed after surgical removal. The diagnosis of ACTH-producing phaeochromocytoma was histologically and immunohistochemically confirmed. Cultured cell suspensions of the tumour secreted ACTH, which stimulated cortisol production in the ipsilateral adrenocortical cells. CONCLUSION: This case demonstrates that the biological activity of an ACTH-producing phaeochromocytoma can vary significantly in time, which may be the consequence of different stages of tumour differentiation.     

Value of ultrasound in differentiating causes of persistent vomiting in infants.

A prospective study of abdominal ultrasound was undertaken in 100 consecutive infants who presented with a history of persistent vomiting aged 5 to 90 days. Each infant had a 'test feed' followed by an ultrasonographic scan of the pylorus at the cotside. On test feeding a palpable tumour was evident in 38 infants. On real time ultrasound using the criteria for diagnosing pyloric stenosis, these 38 infants as well as six others were documented as having pyloric stenosis. In the other 56 patients the vomiting settled in six and a barium examination was performed on the remaining 50. This confirmed gastro-oesophageal reflux in 46, two of whom had an associated hiatus hernia, one with a duodenal malrotation, and three were reported as normal. Ultrasound of the abdomen is an accurate, reliable, and rapid screening method to differentiate the causes of severe vomiting in infancy.     

Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma

Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma. HISTORY AND CLINICAL FINDINGS: A 39-year-old woman was admitted to our department of gastroenterology with recurrent epigastrical pain. Ten years previously the diagnosis of von Hippel-Lindau (VHL)-syndrome has been established. Two years before a germ line mutation in exon 3 of the VHL-tumour suppressor gene has been detected. The patient has a healthy son with a normal VHL-gene and four healthy siblings who had refused a genetic blood test. INVESTIGATIONS: At abdominal ultrasound at the head of the pancreas three 4 2 cm large cysts and in the region of the left adrenal gland a 2,9 2,7 cm large tumor were visible. MRI of the abdomen revealed in addition a 2,2 1,5 cm large tumour of the right adrenal gland. An asymptomatic biadrenal phaeochromocytoma was detected by elevated urine catecholamines and 123I-MIBG-scintigraphy. TREATMENT AND COURSE: The cysts of the pancreas were punctured under endosonographical control and analysis of the cyst fluid was not suspicious of a malignant cystic tumour. The patient had no further abdominal complaints. After oral treatment with the alpha-blocker phentolamine the biadrenal phaeochromocytoma was treated by retroperitoneal laparoscopic surgery in an organ-sparing fashion. Postoperatively ACTH-stimulating test revealed a normal cortisol response. CONCLUSION: Adrenocortical function can be preserved by la-paroscopic adrenal-sparing surgery in bilateral phaeochromocytoma.     

Cardiac phaeochromocytoma presenting with severe hypertension and chest pain

Cardiac phaeochromocytoma is a rare cause of endocrine hypertension. We report a case of a 25-year-old woman, who presented with severe hypertension and intermittent chest pain. The patient denied typical phaeochromocytoma spells of palpitation, headache, and diaphoresis. The 24-hr urinary excretion of norepinephrine was increased sevenfold above the upper limit of normal; however, the excretion of total metanephrines, epinephrine, and dopamine were normal. Computed tomography (CT) scan of the abdomen was normal. An 131I-labelled metaiodobenzylguanidine (MIBG) scan was falsely negative while the patient was taking labetalol. The cardiac phaeochromocytoma was localized with indium-111-pentetreotide scintigraphy and chest magnetic resonance imaging scan. Repeat 123I-MIBG scintigraphy was positive after discontinuing labetalol. The cardiac phaeochromocytoma was located in the right atrial groove, adjacent to the tricuspid valve, and contained multiple feeder arteries from the right coronary artery. After treatment with volume expansion, alpha-methyl-p-tyrosine, and alpha- and beta-adrenergic blockade, surgical resection was performed. While under cardiopulmonary bypass, coronary bypass grafting and tricuspid annuloplasty were performed to facilitate the complete surgical resection of the 4.5-cm tumour. The surgical course was uncomplicated, with complete cure of hypertension and normalization of catecholamine excretion. Post-operative cardiac function, as measured by echocardiogram, was normal. Although cardiac phaeochromocytoma may be highly vascular, invasive and difficult to resect, it can be cured.     

Vagolysis

We describe the case of a 75-year-old man presenting with labile hypertension and symptomatic postural hypotension 13 months following radiotherapy for squamous cell carcinoma of his external auditory canal. Magnetic resonance image (MRI) scan demonstrated scarring and a probable recurrence of his tumour. He underwent autonomic testing, including muscle sympathetic nerve activity (MSNA), heart rate (HR) and blood pressure (BP) responses to a variety of stimuli. Results were consistent with baroreflex failure. Urinary catecholamine levels were within the high normal range. We postulate that baroreflex failure was caused by vagal and glossopharyngeal nerve damage secondary to radiotherapy and tumour recurrence. This diagnosis is rare, but should be considered with pure autonomic failure and phaeochromocytoma in the presence of labile hypertension, especially in patients with a history of radiotherapy to the neck and high-normal catecholamine levels.     

Malignant Phaeochromocytoma with High Circulating DOPA, and Clonidine-Suppressible Noradrenaline

Phaeochromocytoma, “perhaps the most fascinating of all tumours” [1], can present with a broad range of clinical manifestations [2]. Once suspected, the biochemical diagnosis is straightforward in most patients since plasma and urinary levels of noradrenaline and/or adrenaline, and urinary metabolites are well above those encountered in healthy subjects or patients with essential hypertension [3,4]. Exceptions to this general rule are well known, however, hence suppression tests have found favour, particularly in cases where catecholamine levels are within, or close to, the normal range [5-7]. We present a unique patient with malignant phaeochromocytoma whose plasma noradrenaline levels were in the high-normal range, and suppressed normally with clonidine administration. He had extremely high circulating levels of dihydroxyphenylalanine (dopa) which were not affected by clonidine, and different patterns of catecholamine concentrations in tumour tissue and plasma.     

MALIGNANT PARAGANGLIOMA CAUSING BILATERAL PLEURAL EFFUSIONS

A male patient presented with dyspnea due to a large left pleural effusion, and pleural biopsy revealed a malignant paraganglioma. Raised urinary catecholamine levels confirmed a functioning tumour. Aggressive local spread occurred which did not respond to cytotoxic chemotherapy. The primary site of the tumour was most likely the aorticosympathetic chain. (Aust NZ J Med 1989; 19: 61–63).     

Effect of atrial natriuretic peptide on catecholamine release from human pheochromocytoma

The effect of synthetic alpha human atrial natriuretic peptide on catecholamine release from human pheochromocytomas was studied both in vivo and in vitro. Iv infusion of atrial natriuretic peptide at a rate of 0.1 microgram.kg-1.min-1 for 60 min into two normotensive patients with pheochromocytoma caused a small decrease in the mean blood pressure, increase in the heart rate, and marked increase in the plasma level of norepinephrine (2.08 to 6.83 nmol/l, and 1.15 to 2.83 nmol/l, respectively) compared with 0.60 +/- 0.10 to 1.19 +/- 0.20 nmol/l in normal subjects. Treatment with atrial natriuretic peptide also increased the plasma epinephrine level from 0.34 to 1.27 nmol/l, and from 0.67 to 0.79 nmol/l in the patients with pheochromocytoma, but not in the normal subjects (0.05 +/- 0.01 to 0.05 +/- 0.01 nmol/l). After removal of the tumour, the responses of the plasma norepinephrine and epinephrine to atrial natriuretic peptide infusion were normalized. There was no significant effect of 10(-8) to 10(-5) mol/l atrial natriuretic peptide on the basal release of catecholamines from isolated superfused pheochromocytoma tissue. Atrial natriuretic peptide (10(-7) mol/l) did not affect the increase in catecholamine release induced by glucagon (10(-5) mol/l). These results suggest that the exaggerated responses of plasma catecholamines to atrial natriuretic peptide in patients with pheochromocytoma may be due to a washout effect resulting from change in blood flow in the vessels feeding the tumour rather than increased sympathetic nerve activity induced by hypotension and hypovolemia. The results also suggest that atrial natriuretic peptide dose not have any direct action on pheochromocytoma tissue causing catecholamine release.     

Phaeochromocytoma and catecholamine induced cardiomyopathy presenting as heart failure

Phaeochromocytoma is rare and usually presents as paroxysmal or sustained hypertension; none the less, it can also cause severe acute pulmonary oedema in normotensive individuals. Six patients with phaeochromocytoma presenting in Cornwall and West Devon between 1982 and 1986 are described. Five of them died of pulmonary oedema within 24 hours of the onset of symptoms. At necropsy all five had normal sized hearts and in the four hearts examined by histology there was evidence of catecholamine induced heart disease in the form of focal myocardial necrosis. The sixth patient presented with arterial spasms and pulmonary oedema. Surgical removal of the causative tumour was successful in this patient.     

Phaeochromocytoma and catecholamine induced cardiomyopathy presenting as heart failure.

Phaeochromocytoma is rare and usually presents as paroxysmal or sustained hypertension; none the less, it can also cause severe acute pulmonary oedema in normotensive individuals. Six patients with phaeochromocytoma presenting in Cornwall and West Devon between 1982 and 1986 are described. Five of them died of pulmonary oedema within 24 hours of the onset of symptoms. At necropsy all five had normal sized hearts and in the four hearts examined by histology there was evidence of catecholamine induced heart disease in the form of focal myocardial necrosis. The sixth patient presented with arterial spasms and pulmonary oedema. Surgical removal of the causative tumour was successful in this patient.     

Successful catheter drainage of recurrent benign multicystic mesothelioma of the peritoneum

The case history of a patient with pulmonary embolism 4 months following a kidney transplantation is presented. Ultrasonography of the abdomen suggested a large ovarian tumour for which she had a laparotomy. Histological examination after resection of the tumour revealed a benign multicystic mesothelioma of the peritoneum. During the course of 1 year the tumour recurred 5 times and different attempts to aspirate and obliterate the cyst were ineffective. Eventually, cure was achieved by permanent transvaginal catheter drainage, which resulted in infection and obliteration of the cyst.     

Rezidivierende, stärkste Oberbauchschmerzen bei einem 45-jährigen Patienten

A 45-year-old patient presented with a history of recurrent abdominal pain of unknown origin. The CT scan of the abdomen demonstrated a thickened mesenteric root and a segmental ileus of the jejunum. Laparotomy revealed a neuroendocrine tumour of the small bowel. Such tumours are rare causes of recurrent abdominal pain. Especially when the CT scan of the abdomen reveals mesenteric abnormalities (desmoplastic reaction) and/or a segmental ileus, a neuroendocrine tumour of the small bowel should be considered. The therapy of choice is resection of the tumour followed by systemic therapy including somatostain analogues and chemotherapy in the case of a hormone secreting tumour or metastases.     

Selective intra-arterial calcium injection in the investigation of adult nesidioblastosis: a case report

A 69-year-old man with recurrent hypoglycaemia had inappropriately elevated plasma insulin level during a symptomatic hypoglycaemia, but had a negative prolonged fast. Computerized tomography (CT) of the abdomen revealed a nodular lesion over the body of pancreas, whereas pancreatic arteriography failed to show tumour blush. Hence, arterial stimulation (with calcium) and venous sampling (ASVS) was performed and a brisk response of plasma insulin level was found when calcium was injected both into the splenic and the superior mesenteric arteries. Since no tumour was found during the operation, the patient received subtotal distal pancreatectomy. Pathological examination of the resected tissue disclosed a typical finding of nesidioblastosis. We suggest that selective intra-arterial calcium injection with hepatic venous sampling for insulin gradients is useful for the diagnosis of adult nesidioblastosis. © 1997 John Wiley & Sons, Ltd.     

Development of anti-VWF antibody in a patient with severe haemophilia A following the development of high-grade non-Hodgkin's lymphoma

A 9-year-old-boy with severe haemophilia A (factor VIII < 1%) developed colicky abdominal pain with swelling in the left iliac fossa for 4 weeks. His LDH level was 1423 IU/l (normal range < 220 IU/l) and his uric acid, 6.8 mg/dl. A computerised tomography (CT) scan of the abdomen demonstrated a tumour of the terminal ileum and mild hepatosplenomegaly. Pre-operative screening for factor VIII inhibitor was negative. Post-operatively, the patient needed high doses of factor VIII to maintain haemostasis. The tumour was found to be a high-grade lymphoma of Burkitt's type. He recovered from his operation and chemotherapy was commenced. Investigations demonstrated an anti-von Willebrand factor (VWF) antibody. He subsequently relapsed and died of progressive disease. Development of anti-VWF antibody in lymphoma is well known, but development of this antibody in a haemophilia A patient developing lymphoma has not been reported. The present case shows that antibody to VWF should be considered as a possible reason for an increased factor VIII requirement in such patients.     

Plasma catecholamine levels in the acute and subacute stages of takotsubo syndrome: Results from the Stockholm myocardial infarction with normal coronaries 2 study

AimsIt is well‐accepted that takotsubo syndrome (TS) is characterized by a massive surge of plasma catecholamines despite lack of solid evidence. The objective of this study was to examine the hypothesis of a massive catecholamine elevation in TS by studying plasma‐free catecholamine metabolites in patients participating in the Stockholm myocardial infarction (MI) with normal coronaries 2 (SMINC‐2) study where TS constituted more than one third of the patients.Methods and resultsThe patients included in the SMINC‐2 study were classified, according to cardiac magnetic resonance (CMR) imaging findings (148 patients), which was performed at a median of 3 days after hospital admission. Plasma‐free catecholamine metabolites; metanephrine, normetanephrine, and methoxy‐tyramine were measured on day 2–4 after admission. Catecholamine metabolite levels were available in 125 patients. One hundred and ten (88%) of the 125 patients included in SMINC‐2 study, and 38 (86.4%) of the 44 patients with TS had completely normal plasma metanephrine and normetanephrine levels. All patients had normal plasma methoxy‐tyramine levels. Fourteen (11.2%) of the 125 patients included in SMINC‐2 study, and 5 (11.6%) of the 43 patients with TS had mild elevations (approximately 1.2 times the upper normal limits) of either plasma metanephrine or normetanephrine. One patient with pheochromocytoma‐triggered TS had marked elevation of plasma metanephrine and mild elevation of plasma normetanephrine. There were no significant differences between the number or degree of catecholamine metabolite elevations between the different groups of patients with CMR imaging diagnosis included in SMINC‐2 study.ConclusionThere was no evidence of massive catecholamine elevations in the acute and subacute stages of TS apart from one patient with pheochromocytoma‐induced TS. Most of the TS patients had normal catecholamine metabolites indicating that blood‐borne catecholamines do not play a direct role in the pathogenesis of TS.     

Hepatocellular carcinoma arising in an elderly male with primary biliary cirrhosis

We report the case of an elderly male with asymptomatic primary biliary cirrhosis (PBC) who developed a hepatocellular carcinoma (HCC). The 89-year-old man, who was otherwise healthy, was admitted for investigation of mild hepatic dysfunction, which had been detected during a routine physical check-up. Serum chemistry, positive anti-mitochondrial antibody (M2) and liver biopsy results led to a diagnosis of PBC. Three years later, at age 92, computed tomography (CT) and ultrasound scans of his abdomen revealed a large hepatic tumour, which was confirmed on liver biopsy to be HCC. The tumour ruptured 3 months after diagnosis and the patient was successfully stabilized by coil embolization of his right hepatic artery. We believe that, to date, this is the oldest reported patient to have had interventional radiology for the management of HCC.     

A Case of Multiple Extra-adrenal Pheochromocytomas

ABSTRACT A case report of a 37-year-old previously healthy man who contracted hypertension and insulin-dependent diabetes mellitus. Urinary catecholamine excretion was elevated. A total of nine benign pheochromocytomas with characteristic histological appearance were successfully removed. Most of the tumors were located extra-adrenally in the upper abdomen and in the pelvis. Two of the tumors were found in organ plexuses in the mesocolon sigmoideum and in the anterior wall of prostata. Because of asynchronous tumor growth a life-long follow-up is recommended.