[Clinical studies of pheochromocytoma. (I) Circulating blood volume in pheochromocytoma (author's transl)].
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Adolescent↗
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Results for “Pheochromocytoma”
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[Pheochromocytoma and emergency resuscitation. Apropos of 2 cases of pheochromocytoma manifested by acute circulatory insufficiency and pulmonary edema and accompanied by hypoglycemia].
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Adrenal Gland Neoplasms↗
[Pheochromocytoma and multiple tumor formation. Ultrastructure of pheochromocytoma].
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Adrenal Gland Neoplasms↗
Adrenal medullary disease in multiple endocrine neoplasia, type 2: pheochromocytoma and its precursors.
Multiple endocrine neoplasia, type 2 (MEN-type 2), designates the syndrome of medullary thyroid carcinoma, pheochromocytoma, and occasional parathyroid hyperplasia. The thyroid carcinoma, which is usually bilateral and multicentric, is preceded by multifocal C-cell hyperplasia. The adrenal gland manifests pheochromocytoma, which is frequently bilateral and multicentric, and may be malignant. To test the hypothesis that diffuse adrenal medullary hyperplasia is a precursor of pheochromocytoma in this syndrome, we studied the adrenal glands of 19 patients who had MEN-type 2. The findings in the adrenal medulla in these 19 patients were: synchronous bilateral pheochromocytoma in 9 (metastatic in 3); asynchronous bilateral pheochromocytoma in 1 (metastatic); unilateral pheochromocytoma with contralateral diffuse and nodular hyperplasia in 2; unilateral pheochromocytoma with contralateral diffuse hyperplasia in 2; unilateral pheochromocytoma in 1; bilateral nodular hyperplasia in 1; bilateral diffuse hyperplasia in 1; and no abnormality in 2. This spectrum of adrenal medullary pathology suggests that diffuse and nodular medullary hyperplasia are precursors of pheochromocytoma in MEN-type 2.
Adrenal Gland Neoplasms↗
Pheochromocytoma--recognition and management.
Pheochromocytoma is an infrequent cause of hypertension. In spite of its rarity, pheochromocytoma has assumed notorious importance because or a wide variety of clinical features associated with this syndrome. Hypertension remains the most important clinical lead. The presence of other features, such as severe headache, perspiration, palpitations or orthostatic hypotension, makes the diagnosis of pheochromocytoma likely. The diagnosis of this condition can be made with greater certainty than that of any other form of secondary hypertension. Pharmacologic tests are no longer used for evaluation purposes. Biochemical tests are the most important aids to diagnosis, provided highly specific methods are used to determine the levels of urinary catecholamines or their metabolites. Interference by various drugs should be avoided. Most of the pheochromocytomas are found in the abdomen, predominantly in the adrenals. Successful outcome of surgery depends critically on adequate preoperative preparation of the patient with adrenergic blocking drugs and proper intraoperative care. Surgery should only be performed in an institution with experience in treating these tumors. Invasive localizing procedures could be dangerous in patients with pheochromocytoma and are best avoided. Medical therapy proves to be quite successful for those patients who are unable or unwilling to undergo surgery and for those with demonstrated malignant tumor. The postoperative course in most instances is uneventful. The physician should be aware of familial forms of pheochromocytomas and screen all the hypertensive members of the patient's family for the presence of this condition. Pheochromocytoma, with its multiple facets, presents a challenge to the clinician; however, with prompt diagnosis and proper treatment, pheochromocytoma can often be cured. The improved management of patients with this potentially lethal condition is the result of better knowledge of biochemical pharmacology, improved preoperative, surgical and postoperative care.
Adrenal Gland Neoplasms↗
Familial pheochromocytoma, hypercalcemia, and von Hippel-Lindau disease. A ten year study of a large family.
Long-term epidemiological and laboratory studies were carried out in a kindred with familial pheochromocytoma associated with von Hippel-Lindau disease. Thirteen members were affected by the syndrome and the trait appears to be transmitted in an autosomal dominant fashion. Of 13 patients, 7 had pheochromocytoma alone. Of the remaining six patients, one had pheochromocytoma combined with von Hippel-Lindau disease, four had pheochromocytoma with retinal disease only, and a single patient had a retinal lesion without pheochromocytoma. In four patients, pheochromocytoma antedated the development of retinal lesions. Ten members also had mild hypercalcemia without accompanying elevations of PTH in the 4 patients in whom this was determined. In all, hypercalcemia was corrected with removal of tumors, and no patient had a return of hypercalcemia in the absence of recurrent increases in urinary catecholamines. The clinical presentations in 12 patients varied markedly, as did their urinary excretion rates of norepinephrine, epinephrine and their metabolites. However, an analysis of the data revealed significant correlations not previously described between the urinary excretion of free catecholamines (norepinephrine plus epinephrine), blood pressure, the free catecholamine content of the tumor and the age of the patient. Urinary excretion of free norepinephrine plus epinephrine appear to be decreased with advancing age (p less than 0.001). Both systolic and diastolic blood pressures and the age of the patient were inversely correlated (p less than 0.01). A significant inverse relationship between the tumor content of free catecholamines and the age of the patients was, although to a lesser degree, also present (p less than 0.05). As a whole, the size of the tumors and their norepinephrine content were not correlated. We present a concept that, in familial pheochromocytoma, the metabolism of catecholamines is altered by the process of aging, and that this change modifies the clinical presentations of the disease.
Adolescent↗
[Incidence of medullary thyroid gland carcinoma in unilateral and bilateral pheochromocytoma].
In the autopsy material of the Departments of Pathology in Zürich and Winterthur the frequency of cases with unilateral or bilateral pheochromocytoma and combinations of pheochromocytoma with additional medullary thyroid carcinoma with amyloid has been studied. 56 cases (0.084 percent) of pheochromocytoma, 50 unilateral and 6 bilateral, were found in a material of 66782 autopsies. Three of the 6 patients with bilateral pheochromocytomas had an additional medullary thyroid carcinoma with amyloid deposits, while no patient with unilateral pheochromocytoma exhibited this type of thyroid tumor. In families with Sipple's syndrome, however, combinations of unilateral pheochromocytoma with medullary thyroid carcinoma do occur. No typical coincidence of pheochromocytoma with other malignant tumors could be demonstrated in our material, although this possibility is discussed in the literature.
Adolescent↗
Alterations in plasma norepinephrine concentration during surgical resection of pheochromocytoma.
Using a sensitive and specific radioenzymatic assay, the plasma norepinephrine (NE) concentration was measured in seven patients with pheochromocytoma, one patient with bilateral adrenal medullary hyperplasia, one patient with a retroperitoneal paraganglioma, and two patients undergoing bilateral adrenalectomies for palliation of metastatic breast carcinoma. Surgical manipulation of the pheochromocytomas resulted in striking increases in plasma NE concentration with concomitant increases in blood pressure. There were either small changes or no changes in the patients' plasma NE and blood pressure during resection of the normal adrenal glands, the adrenal glands with medullary hyperplasia, or the retroperitoneal paraganglioma. Plasma dopamine-beta-hydroxylase (DBH) was measured in one patient with pheochromocytomas and the patient with medullary hyperplasia. There was no change in plasma DBH in either patient, supporting the concept that exocytosis is not the primary mechanism for catecholamine secretion from pheochromocytomas. It was also noted that enflurane is an excellent general anesthetic for the resection of pheochromocytomas, and that sodium nitroprusside (rather than phentolamine) may be the agent of choice for the management of the hypertensive episodes that occur during surgical manipulation of pheochromocytomas.
Adolescent↗
Circulating and urinary catecholamines in pheochromocytoma. Diagnostic and pathophysiologic implications.
Three biochemical tests for the diagnosis of pheochromocytoma were evaluated in 24 patients with proved tumors and 40 patients whose clinical picture was suspect but who had no evidence of the disease. Measurement of resting, supine plasma catecholamines (by radioenzymatic assay) was more useful than either 24-hour urinary vanillylmandelic acid (VMA) or metanephrines or both. In only one of 23 patients with pheochromocytoma were plasma catecholamines within the range of those in patients without pheochromocytoma, as compared with urinary VMA in 11 of 22, urinary metanephrines in five of 22 and both metabolites in three of 22. These studies reaffirm the value of plasma catecholamines in the diagnosis of pheochromocytoma and indicate that urinary catecholamine metabolites are less useful. The poor correlation between the height of arterial pressure and circulating levels of catecholamines suggests that the regulation of arterial pressure in pheochromocytoma is complex.
Adrenal Gland Neoplasms↗
Simultaneous adrenal and cervical pheochromocytomas in childhood.
Pheochromocytoma is an uncommon tumor in childhood. The simultaneous occurrence of adrenal and cervical pheochromocytomas is a rare phenomenon; to our knowledge, this combination has been reported in the literature only once. Cervical pheochromocytomas are more accurately termed "aorticosympathetic paragangliomas." In children, 50% of pheochromocytomas are bilateral, multiple, or extra-adrenal. An increased familial incidence in the form of simple mendelian dominance is also noted in the pediatric age group. Bolus nephrotomography is extremely effective in identifying adrenal pheochromocytomas in children. To prepare the patient for surgery, and alpha-adrenergic blocking agent is administered from 7 to 10 days before operation and a beta-blocking agent is administered 3 days before. A transabdominal approach is essential because of the frequent extra-adrenal sites and multicentricity of the tumor in children.
Adolescent↗
[Biosynthesis of C19- and C21-steroids in tissue slices of paraganglioma and pheochromocytoma (author's transl)].
The biogenesis of C19- and C21-steroids has been studied in tissue slices of a paraganglioma, of a pheochromocytoma and of human adrenal cortex using radioactive steroids. Slices of paraganglioma as well as of pheochromocytoma metabolise 17-hydroxyprogesterone to cortisone, cortisol, 11-deoxycortisol and testosterone. The rate of formation of these steroids, however, by the two tumours is 8--15 times less than that in normal adrenal cortex tissue. After incubation of dehydroepiandrosterone with tissue slices of paraganglioma and pheochromocytoma, 7alpha-hydroxydehydroepiandrosterone, 11beta-hydroxy-4-androstene-3,17-dione and 4-androstene-3,17-dione were found as metabolites; testosterone was converted by both tissues to 4-androstene-3,17-dione. 17-Hydroxypregnenolone was converted to a small extent (1.7%) to dehydroepiandrosterone by slices of paranganglioma. These results show that enzymes of steroid biosynthesis (hydroxylases, oxidoreductases, delta4(-5)-isomerases, C17(-20)-desmolases) are present in both paraganglioma and pheochromocytoma.
Adrenal Cortex↗
Hypercalcitoninaemia in patients with pheochromocytoma.
As pheochromocytoma sometimes is accompanied by medullary thyroid carcinoma (in the sense of multiple endocrine adonomatosis type II = Sipple-Syndrome), serum calcitonin (CT) was measured by radioimmunoassay in 4 patients with pheochromocytoma. Before extirpation of the adreno-medullary tumor, serum CT was distinctly increased to 3 and 30 ng/ml in 2 of 4 patients, respectively. After removal of the tumor, serum CT was normal in the patients and pentagastrin stimulation produced no exaggerated CT response. In hydrochloric acid extracts from the two corresponding pheochromocytoma tissues, immunoreactive-Calcitonin (IR-CT) was detected, the concentrations amounting 1 and 4 ng/mg wet tissue. These findings suggest that hypercalcitonism in patients with pheochromocytoma cannot always be ascribed to the thyroid, i.e. increased calcitonin levels do not necessarily indicate a medullary carcinoma of this organ.
Adolescent↗
Cardiovascular complications of pheochromocytoma crisis.
Two patients with hypertensive crises due to pheochromocytoma presented with unusual features suggestive of cardiovascular disorders other than pheochromocytoma. These features included transient cortical blindness and other neurologic deficits, electrocardiographic changes indicative of transmural infarction and peripheral arterial spasm. In both patients the diagnosis of pheochromocytoma was made later; removal of the tumor was followed by ready reversal of the clinical and biochemical abnormalities. Management of such cases includes suspicion of the diagnosis in the presence of atypical features, early initiation of therapy with alpha and beta adrenergic blocking agents and a definitive surgical procedure before peripheral vascular changes become irreversible. When an apparent myocardial infarction occurs, the diagnosis of coronary artery disease cannot always be excluded, but rapid stabilization of the clinical and electrocardiographic changes after adrenergic blockade would favor the diagnosis of a catecholamine-induced myocarditis. The coexistence of coronary artery disease and uncontrollable arrhythmias presents an increased risk but, if pheochromocytoma is suspected, surgery may be necessary despite the increased risk.
Adrenal Gland Neoplasms↗
Diagnosis and localization of pheochromocytoma. Detection by measurement of urinary norepinephrine excretion during sleep, plasma norepinephrine concentration and computerized axial tomography (CT-scan).
The feasibility of differentiating patients with pheochromocytoma from other hypertensive patients by measuring urinary excretion rates of norepinephrine during sleep, a period of physiologic suppression of norepinephrine release, was investigated. The mean excretion rates of norepinephrine in 248 normal subjects and in 109 patients with essential hypertension were 1.03 +/- 0.03 and 1.12 +/- 0.06 (SEM) micrograms/hour, respectively, whereas the lowest excretion rate among the six patients with pheochromocytoma was about seven times higher. Plasma norepinephrine concentration in patients with pheochromocytoma was also consistently above the range observed in both normotensive and hypertensive subjects. CT scan correctly identified the same tumors visualized by selective arteriography. It is suggested that the usefulness of these approaches will provide simpler means of screening and detecting pheochromocytoma.
Adolescent↗
Diagnostic and operative problems in multiple pheochromocytomas.
Two children, aged 14 yr, with multiple pheochromocytomas are presented. Both patients had a positive family history. In the preoperative aortographies the intra-adrenal pheochromocytomas of both patients were well visualized, but not the extra-adrenal tumors of the first case. Chlorpromazine as an adrenergic blocking agent was successfully used in the preoperative treatment. Postoperative catecholamine excretion in the first case was repeatedly slightly increased indicating residual pheochromocytoma. In addition to the bilateral adrenal pheochromocytomas, multiple islet cell adrenomas and cholecystolithiasis were revealed at the operation of the second case. After bilateral adrenalectomy and total pancreaticoduodenectomy, regular follow-up examinations were carried out for 28 months. Hyperparathyroidism and signs of possible medullary thryoid carcinoma were discovered. Thus the patient had an unique pattern of MEA syndrome.
Adenoma, Islet Cell↗
Malignant pheochromocytoma arising in the organ of Zuckerkandl--a clinicopathological study.
An autopsy case of malignant pheochromocytoma arising in the organ of Zuckerkandl is reported, of which distant metastases were found in the liver and lymph nodes. The biochemical and electron microscopic findings from the tumor tissue showed noradrenaline-secreting type. It was revealed that sex ratio in malignant pheochromocytoma was more prominent in the female, and the frequency of extra-adrenal origin was higher in malignant pheochromocytoma than in benign one from comparative study of reported cases of malignant pheochromocytoma.
Catecholamines↗
Angiography in pheochromocytoma of the urinary bladder. Report of a case.
A case of pheochromocytoma of the urinary bladder in a 20-year-old female is reported. This is the 36th reported case in the English language literature. The patient had classical symptoms of vesical pheochromocytoma with sudden onset of headache, palpitations, and blood pressure elevation during voiding. The provisional diagnosis was arrived at on clinical history, laboratory investigations, including the estimation of catecholamines and vanil mandelic acid (VMA). The diagnosis was confirmed and the tumor was exactly localized by means of angiography. The angiographic appearance of pheochromocytoma of the urinary bladder is not pathognomonic and is similar to that previously described for adrenal pheochromocytoma. Angiography is, with adequate precautions, a safe and useful procedure for ruling out synchronous adrenal or extra-adrenal tumors and for exact localization of the bladder tumor.
Adult↗
Parathyroid function in patients with pheochromocytoma.
Serum calcium, serum immunoreactive parathyroid hormone (PTH), and plasma immunoreactive calcitonin were measured in 12 patients with catecholamine-secreting tumors. Only two patients had high serum calcium and immunoreactive PTH concentrations (one also had high basal immunoreactive calcitonin); in both, the hypercalcemia persisted after removal of their pheochromocytomas but was corrected by removal of hyperplastic parathyroid glands and medullary thyroid carcinomas. Of the 10 patients with normal serum calcium and immunoreactive PTH concentrations, 2 had high immunoreactive calcitonin concentrations and medullary thyroid carcinomas alone were found. Our results suggest that [1] hypercalcemia occurs infrequently in patients with pheochromocytoma, [2] chronic circulating catecholamine excess does not cause increased immunoreactive PTH or immunoreactive calcitonin secretion in patients with pheochromocytoma, and [3] the parathyroid disease in patients with pheochromocytoma is a genetically determined component of multiple endocrine neoplasia.
Adolescent↗