[Middle mediastinal parathyroid adenoma of the aorto-pulmonary window. 2 cases].
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Biomedical subjects
Publications and source records attributed to C Proye.
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In 1985, 1409 consecutive patients underwent surgery in one surgical Professorial Unit of Lille (France). 45 (3.2%) died post-operatively: 28 (62%) were more than 70 years of age, 23 (50%) died after emergency procedure and 18 (40%) died in sepsis. Nothwerthy in retrospect, 20 (44%) died after surgical indication or procedure of questionable legitimacy. Preoperative appraisal of the surgical risk is still challenging nowadays but conclusions drawn from those charts should avoid identical outcome in similar patients.
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After having reported the case of a pheochromocytoma associated to Recklinghausen's disease, the authors define the best criteria of detection and localization of the pheochromocytoma and study its association to phacomatoses. Headaches, bouts of tachycardia and excessive inappropriate diuresis are the most evocative clinical signs of a pheochromocytoma. The different hormones and their urinary metabolites must be titrated separately and repeatedly. Two other examinations, scanner and scintigraphy with MIBG, visualize quite reliably the tumor foci. Calcium inhibitors are quite effective in sudden blood pressure rises. The association described here, may be explained by the fact that the two pathologies belong to the group of neurocristopathies.
Study of 20 pheochromocytomas in 19 patients confirmed the remarkable sensitivity of computed tomography (100%) for the detection of their adrenal and juxtarenal localizations. However, ultrasonography was as effective as the CT scan in these same patients, and even detected latent tumors in several cases. Certain pheochromocytomas in this series presented unusual appearances, particularly two large tumors which were manifest as abdominal masses and a malignant form which provoked a total infarct of the homolateral kidney.
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A pheochromocytoma that exclusively secretes dopamine (DA) rather than predominantly DA among a blend of catecholamines is as yet unreported. Of the 50 patients with pheochromocytoma who have undergone surgery, 32 underwent treatment within the last 5 years (when DA assay has been available). One half of these patients (15/32) exhibited DA secretion either in mixed catecholamines (12 patients) or exclusively (three patients). All three patients with exclusive DA-secreting tumors were normotensive. Without hypertension, the clinical investigation was a diagnostic challenge (unexplained cough or flank mass with inflammatory features). All three tumors were malignant and two were ectopic. Five of the 12 patients with mixed catecholamine-secreting tumors whose secretions included DA were hypertensive. Five other patients had flank mass and one had an unexplained cough. Tumors were rather large, and three of the tumors with mixed secretion were ectopic. Of the 12 patients, seven had tumors that were judged to be malignant. Three patients exhibited a dramatic decrease in blood pressure under alpha-blockade, which was not used in subsequent cases. Predominant or exclusive secretion of DA would explain the lack of hypertension due to its antiadrenergic action that inhibits the vasoconstrictive effects of other amines. Hypertension in patients with pheochromocytoma might depend on the ratio of DA/noradrenaline + adrenaline.
Studies were conducted in 14 patients with pheochromocytoma over a 3-year period. Circumstances of detection of these tumors varied greatly and were sometimes misleading, hypertension being an inconstant finding in the clinical history and was not always the predominant feature. Biologic exploration involved assay of excretion of free urinary noradrenaline (NA), adrenaline (AD) and dopamine (DA) using a HPLC technique as well as assay of total methoxy derivatives and urinary vanilmandelic acid. Validity of each assay in the diagnosis of pheochromocytoma could be evaluated and only the total free methoxy derivatives gave false negative results. Hormonal secretion of pheochromocytoma is often mixed, but sometimes predominant or exclusive for a single catecholamine. Relative increases of the different catecholamines, evaluated from the ratios DA/NA and DA/NA + AD, are an important factor since a relation exists between blood pressure induced symptomatology and equilibrium between hypotensive hormone (DA) and pressor amines (NA + AD); 3 types of pheochromocytoma can be described: NA-induced with paroxysmal or permanent hypertension but without typical metabolic and cardiac disorders, and with a very reduced DA/NA + AD ratio during hypertensive crises; AD-induced without permanent hypertension but with a mainly orthostatic hypotension and episodes of cardiovascular collapse following hypertensive attacks and with an AD/NA ratio greater than 1; finally the DA-induced lesion in which hypertension is never associated and manifestations are misleading and atypical with an elevated DA/NA + AD ratio.
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Diabetic ketoacidosis is an extremely rare manifestation of glucagonoma. We report such a case in a 72-year-old woman known to be diabetic for seven years. The patient was admitted with diabetic ketoacidosis and associated necrolytic migratory erythrema which suggested the diagnosis of glucagonoma. Plasma glucagon levels were increased (569 to 2298 pg/ml). A vascular tumor of the head of the pancreas without obvious hepatic metastases was visualised by angiography. Duodeno-pancreatectomy including the head of the pancreas led to complete recovery of the mucocutaneous lesions and the plasma glucagon level fell (229 pg/ml). The tumor had several histological characteristics suggesting malignancy and a high glucagon content on extraction. Electron microscopy showed multiple A cells and a few isolated B cells. Most of the cells showed immunoreactivity with anti-glucagon and anti-glicentine antibodies. Three months after surgery, the diabetes was again required treatment with insulin. Plasma glucagon level was again increased and chemotherapy with dimethyltriazenimidazolecarboxamide was undertaken.
Follow-up of 102 patients operated upon for autonomous thyroid nodules is reported. Retrospective study of operative findings and aftermath of surgery clearly demonstrates that the patho-physiological concept of autonomous nodule does not snugly fit practical problems experienced with the management of such patients. The autonomous nodule can be a malignancy. Ipsilateral thyroid lobectomy seems more suitable than nodulectomy. Gross appearance of the thyroid remnant makes sometimes post-operative hormonotherapy unexpectedly advisable. From a theoretical point of view, post-operative thyroid scan, TSH assay and TRH test are mandatory, to assess recovery.