Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixteen patients with abdominal neuroblastoma had 99m Technetium Phosphate Compounds (99m TC-PC) bone scans as a preoperative evaluation for metastatic disease. Ten patients (62%) had extraosseous tumor uptake while six patients (38%) did not. There was no difference in the incidence of tumor calcification, tumor necrosis or hydronephrosis in the two groups. However, VMA levels were significantly higher in the group with extraosseous tumor uptake. Various bone seeking radionuclides are compared to 99m TC-PC and possible mechanism for extraosseous uptake of such radionuclides are postulated. Awareness of the frequency of such uptake should reduce the possibility of errors in the interpretation of bone scans in patients with neuroblastoma.
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PMID:Extraosseous tumor uptake of 99m technetium phosphate compounds in children with abdominal neuroblastoma. 21 61

The X-ray findings in 51 cases of neuroblastoma were analysed. The aim of plain film of abdomen is to search for fine sandy calcifications in the abdominal mass, paravertebral widening at lower thoracic region, and bone metastases. Displacement and deformity of kidney by extrinsic pressure seen in IVU are useful in differentiating an extrarenal tumor from intrarenal one. Ultrasound and CT are superior to IVU in localization diagnosis and defining the extent of abdominal neuroblastoma. Thoracic neuroblastoma usually manifests as a posterior mediastinal tumor associated with rib changes and the chest film remains valuable in diagnosis of this condition. It is mandatory to screen the patient for skeletal metastases radiographically and to investigate the bone marrow, and urine VMA whenever abnormalities are found in abdominal or thoracic imaging. The significance of combined imaging techniques in staging of neuroblastoma was discussed, and a simple diagnostic approach was proposed.
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PMID:[Radiologic analysis of 51 cases of neuroblastoma]. 269 14

Pheochromocytoma, a chromaffin tumor of the sympathoadrenal nervous system, rarely exhibits direct extension into the inferior vena cava. We report a patient with a right adrenal pheochromocytoma that caused symptoms of recurrent pulmonary emboli. Intracaval extension of the tumor was detected during investigations for the source of the emboli by abdominal CT scan, ultrasonography, and venacavography. The biochemical diagnosis of pheochromocytoma was confirmed by sharply elevated catecholamine, metanephrine, and VMA urinary levels, which returned to normal upon resection of the tumor. The patient had no evidence of metastatic disease.
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PMID:Intracaval extension of pheochromocytoma simulating pulmonary embolism. 354 48

The clinical manifestations of 253 neuroblastoma cases in childhood, treated in Denmark from 1943 to 1980, were reviewed. Most striking was the vagueness of symptoms in the majority of patients, only a few of whom exhibited the symptoms strongly suggestive of a neuroblastoma (i.e. the Horner syndrome, the watery diarrhoea syndrome, the dancing eye syndrome). The vagueness of the symptoms might have led to fatal procrastination of the diagnosis. The diagnostic delay has, however, no independent prognostic significance for survival in our patients (p = 0.09). The maximal tumour spread was recorded for all 253 patients, and the distribution of metastases was in accordance with the "soil-seed" hypothesis. The tumour spreads with equal frequency by local growth, by lymphatic vessels to distant lymph nodes, and by blood to bone. Only in widely disseminated tumours are metastases to the lungs, the meninges, the brain, and the reproductive organs seen to occur. Eighty-five percent of the patients, for whom data were available, excreted VMA above the normal value for their age, and 43% excreted Norepinephrine + Epinephrine (N + E) above normal levels. The excretion of both VMA and N + E was significantly correlated to stage, and thus to prognosis. Neither the level of VMA excretion nor the level of N + E had any bearing on the survival when age and stage were adjusted for. Serial VMA and N + E determinations show that patients with normal values for these parameters had significantly better prognosis than patients with elevated values during the first, second, third and fourth trimesters after the initiation of treatment. Increasing values in the individual patient were associated with a poor prognosis. We found no correlation between the initial leucocyte count and survival when age and stage were adjusted for.
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PMID:Signs, symptoms, metastatic spread and metabolic behavior of neuroblastomas treated in Denmark during the period 1943-1980. 363 7

An 11-year-old white girl was admitted to The Children's Hospital of Philadelphia in July 1983 for evaluation of metastatic tumor. She had been well until July 1982, when a mass developed over the right scapula. Treatment with warm compresses and antibiotics resulted in no improvement, and incision and drainage were unproductive. A biopsy at another hospital was interpreted as showing a primitive neuroectodermal tumor. There was no evidence of metastatic disease. She then underwent excision of the tumor with the underlying scapula. No further treatment was administered. She remained well until February 1983, when she began to complain of occasional lower back and thigh pains. Her symptoms worsened over the succeeding 3 months, despite treatment with analgesics and physical therapy. By May 1983, she was no longer able to attend school because of weakness and pain, and had sustained a 10% weight loss during the previous 2 months. She was admitted to her original hospital, where bone scan and bone marrow biopsy showed disseminated tumor; she then came to The Children's Hospital of Philadelphia. On examination, she appeared acutely and chronically ill. It was very uncomfortable for her to move, and she walked with a slow, stooped, shuffling gait. She complained of tenderness in the lower back and both sides. There were no other abnormalities on examination. The hemoglobin level was 9.7 gm/dl, following transfusion at the other hospital; white blood cell count was 6,900/mm3 with a normal differential, and the platelet count was 480,000/mm3. A 24-hr urine test for VMA excretion was normal. She underwent bone marrow aspiration and biopsy, and the radiographs and pathology slides from the other hospital were reviewed.
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PMID:Extracranial primitive neuroectodermal tumor. 632 31

A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by "exclusion" a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.
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PMID:Esthesioneuroblastoma: ultrastructural, immunohistological and biochemical investigation of one case. 671 29

It was found a solid tumour of 24 x 28 x 31 mm size above the right kidney with ultrasound screening in a three days old newborn. After three days appeared cystic areas inside the tumour, so it was thought to adrenal haemorrhage. The tumour didn't decrease during two months and metastases developed in the liver. The urinary VMA level was normal, the HVA elevated. The MIBG scintigraphy was first negative, after a month pathognostic. The primer tumour and metastases disappeared after cyclophosphamid and Adriablastin treatment. The adrenal neuroblastoma can imitate the adrenal apoplexy, so it's important to control all neonatal adrenal haemorrhage until their disappearance.
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PMID:[Neonatal neuroblastoma detected by ultrasonic screening]. 823 51

Adrenal masses are more and more frequently detected by adrenal ultrasound, computed tomography or nuclear magnetic resonance carried out for a reason other than the suspicion of adrenal disease (incidentalomas). The findings of an incidentaloma still leaves many diagnostic and therapeutic questions open. We report the results of a multicentric retrospective evaluation of patients with adrenal incidentalomas, performed by a Study Group of the Italian Society of Endocrinology. According to the definition of incidentaloma, exclusion criteria a priori were: severe or paroxysmal hypertension, frank hypokalemia and clinical signs of hypercortisolism or hyperandrogenism. 29 centers participated in the study and the data obtained by questionnaire were collected in 2 centers for final elaboration. Center 1 carried out the epidemiological and clinical evaluation. Basal and dynamic hormonal evaluation of 786 among the 1013 cases recruited were performed in our center (center 2). Functional studies included: diurnal rhythm of cortisol, urinary free cortisol (UFC), ACTH, DHEAS, 17-OH progesterone, testosterone, androstenedione, supine and upright plasma renin activity (PRA) and aldosterone, urinary aldosterone, urinary catecholamines and VMA. The hormonal dynamic evaluation included the overnight dexamethasone suppression test (1 mg), CRH test and ACTH test. In our study, 89% (702 patients) of adrenal incidentalomas were non-hypersecretory masses; 6.2% (49 patients) showed a preclinical Cushing's syndrome (PCS) (at least two altered parameters of pituitary-adrenal axis); 3.4% (27 patients) were pheochromocytomas; 0.89% (7 patients) were aldosteronomas. One tumor was a masculinizing adrenocortical carcinoma. Two hundred sixty patients underwent surgical exploration and the histological diagnosis showed: 138 adenomas (53%), 32 carcinomas (12%), 26 pheochromocytomas (10%). 16 myelolipomas (8%), 13 cystic lesions (5.5%), 7 tumors of neuronal lineage (3%). 12 metastases (4%), 13 others (5%). The 138 patients with adenomas had the following hormonal diagnosis: 103 nonfunctional adenomas (74%), 31 PCS (23%) and 4 cases of hyperaldosteronism (3%). In the patients with PCS an abnormal dexamethasone suppression test was found in 86% of cases (37/41 patients). Values for ACTH were low in 78% (32/41 patients). UFC was elevated in 64% of patients, the diurnal rhythm of cortisol evaluated in 14 patients was absent in 7. Only in 50% of cases DHEAS values (12/24 patients) were decreased, whereas they were normal in the other 50%. Interestingly, 8 patients with normal DHEAS and normal UFC showed nonsuppressible cortisol by dexamethasone test (1 mg). Blunted ACTH response to CRH was detected in 9 of 14 patients (64%). Thus our data suggest that the best parameter for evaluating subclinical hypercortisolism seems to be the overnight dexamethasone suppression test. In 27 patients with pheochromocytoma 24-hour urinary catecholamine and VMA levels were elevated in 86 and 46% of cases respectively. In 7 patients with hyperaldosteronism upright PRA was suppressed in 100% of cases and aldosterone plasma levels were elevated in 6 patients (86%); serum potassium level was slightly decreased in 60% of cases. In 86 of 138 histologically proven adenomas, DHEAS levels were: normal in 59% of patients, decreased in 36% and elevated in 4.6%, whereas in 22 of 32 cortical carcinomas evaluated. DHEAS levels were normal in 63% of cases, decreased in 18% and elevated 18%. Post-ACTH 17-OH progesterone levels were elevated in 52% (62/118 patients) of non-functioning adenomas and in 2 of 4 carcinomas. Not enough data are yet available postoperatively. In summary, endocrine evaluation can lead to the identification of a nonnegligible number of cases of clinically unsuspected pheochromocytomas and subtle hypercortisolism (about 3.4 and 6.2%, respectively of all adrenal incidentalomas), while cases of primary subclinical aldosteronism are rarely found. (ABSTRACT TRUNCATED)
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PMID:Adrenal incidentaloma: an overview of hormonal data from the National Italian Study Group. 916 66

A 38 year-old man presented with upper abdominal mass and hypertension pointed out at a medical examination. Blood pressure was 170/90 under medication of an alpha-blocker. Abdominal CT scan showed an 8 x 8 cm inter-aortocaval mass displacing pancreas head ventrally, and further a 4 x 4 cm mass at the aortic bifurcation, but there was no tumorous lesion in bilateral adrenal glands. Plasma nor-epinephrine level and urinary VMA excretion were excessive but plasma adrenaline level was within normal limits. MIBG scintigram showed hot spots in the 4th and 9th thoracic vertebrae. The destructive change of the 9th vertebra on magnetic resonance imaging strongly suggested metastasis of the tumor. Histologic and immunohistochemical findings of the biopsy specimen taken from the lower abdominal tumor in addition to the above clinical data led to the diagnosis of extra-adrenal malignant pheochromocytoma with spinal metastases. Since 2 cycles of full dose CYVADIC chemotherapy had no effects on lowering the high blood pressure and reducing the tumor size, low dose (60% of the full dose) CVD (cyclophosphamide, vincristine and dacarbazine) was given as a palliative chemotherapy on an out-patient clinic approximately every 4 weeks. After 4 cycles of the chemotherapy, his backache due to spinal metastasis markedly improved, hypertension as well as the plasma dopamine level was normalized and nor-epinephrine level was markedly decreased, though the tumor size was not reduced. Thereafter, no medication for hypertension was necessary. During 3 years and 6 months until now, 36 cycles of the chemotherapy has been repeated with no significant side effects. He has been at full-time work with quality of life being well preserved. Low dose CVD regimen appears to be an effective tumor dormancy therapy for advanced extra-adrenal pheochromocytoma.
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PMID:[Low dose CVD chemotherapy as a tumor dormancy therapy for extra-adrenal malignant pheochromocytoma: a case report]. 1151 73

Thirty three patients with phaeochromocytomas had surgery in St. Vincent's Hospital between 1950 and 1997. 32 patients had hypertension, 32 had palpitations and 30 had excessive sweating. All patients had at least one of these cardinal symptoms. Diagnostic tests changed over this period. In the early cases the phentolamine test was used, later diagnosis was confirmed by measurement of urinary VMA and catecholamines. IVP, CT, ultrasound or MIBG scanning were used to locate the tumours. Before 1967, 3 of 17 patients died peri-operatively, 2 of these from strokes and one from pulmonary embolism. Since then, all patients have survived surgery. Four of the women had phaeochromocytomas diagnosed during pregnancy. Two of the 33 patients had tumours that were malignant, the remainder being benign. One patient has died from metastatic disease. This series illustrates the changing trends in the surgical management of phaeochromocytomas.
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PMID:47 years of phaeochromocytomas. 1169 8


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