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)

Ganglioneuroblastoma a transitional tumor of sympathetic origin has not yet been described as involving orbit. It is characterized by a mixture of cells ranging from primitive neuroblast to well differentiated ganglion cells within a neurofibromatous tissue. The prognosis is uncertain, as the tumor may either undergo maturation into a ganglioneuroma or may metastasize widely and rapidly as in neuroblastoma. We may postulate a relationship between ganglioneuroblastoma and Recklinghausen's neurofibromatosis in view of the development of the tumor in conjunction with the phacomatosis.
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PMID:[Ganglioneuroblastoma of the orbit]. 392 90

Over a 2 year period, 63 of 1,459 patients examined by computerized tomography were found to have adrenal masses. In 19 patients (1.3 percent of patients examined and 30 percent of patients with adrenal masses), they were unexpected and did not give rise to symptoms or signs. Three patients were explored. Two of the patients had adrenocortical adenomas and a third, a ganglioneuroma. Adrenal function tests were performed in 14 patients and showed evidence of Cushing's syndrome in 1 patients and revealed no abnormalities in 13. The lesions in 10 of 11 nonsurgical patients followed by computerized tomography for 11 to 36 months showed no change. One lesion became significantly smaller. In a review of 988 autopsy reports, grossly visible adrenal masses were present in 73 patients (7.3 percent), including 19 adrenocortical adenomas (1.9 percent) and 50 metastases (5 percent). We conclude that serendipitous adrenal masses are usually small, nonfunctioning, and benign, the most common lesion being adrenocortical adenoma. A protocol has been suggested for management to identify the minority of patients with functioning or malignant lesions and to avoid unnecessary surgery in the others who have benign disease.
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PMID:Serendipitous adrenal masses: prevalence, significance, and management. 401 56

Incidental finding of non-functioning adrenal masses is not uncommon in patients undergoing abdominal CT. In a 4-year period, adrenal masses were detected as an accessory finding in 38 patients. The diagnosis was confirmed either histologically or in follow-up studies. This global series consists of 6 adenomas, 4 carcinomas, 1 pheochromocytoma, 1 ganglioneuroma, 23 metastases, 1 lymphoma, 1 histiocytoma, 1 myelolipoma. The Authors point out the parameters for the pathologic assessment of adrenal masses: size, contours and fine-needle biopsy appear to be the most reliable features.
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PMID:[Computed tomography of non-functioning adrenal masses]. 404 55

Neuroblastoma, one of the commonest malignant childhood tumors, arises from the adrenal glands and/or sympathetic ganglia, and metastases into lymph nodes, bone marrow, bones, etc. Patients with advanced stages of neuroblastoma are poorly responsive to current combined chemo-radiotherapy. However, neuroblastoma is known to maturate into benign ganglioneuroma spontaneously or induced by certain chemicals. In our previous studies, adenosine 3', 5'-monophosphate (cAMP) was found to play an important role in this maturational process. It was also demonstrated that long-term continuous infusion of dibutyryl cAMP and papaverine caused significant growth-inhibition and tumor differentiation of C 1300 neuroblastoma in mice. These results prompted us to apply intra-aortic PGE1 infusion to two cases of abdominal neuroblastomas with stages III and IV.PGE1 infusion (0.25-3.0 ng/kg/min, over the period of 3 to 32 weeks), combined with oral papaverine and conventional chemotherapy, was effective in decreasing tumor size and promoting tumor maturation in the infused area. However, distant osseous metastases occurred in both cases, during and after the PGE1 administration. Similar modalities should be further evaluated in order to potentiate anti-metastatic effect on neuroblastoma cells.
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PMID:[Intra-aortic prostaglandin E1 infusion in the treatment of advanced neuroblastoma]. 631 Nov 12

Scintigraphy with 131I-m-BG was used in 4 children with a history of multiple pheochromocytomas in order to localize further catecholamine-producing tumors prior to surgery. We overlook 7 scintigraphies without any side effect. Phenoxybenzamine did not interfere with tracer uptake into tumors. Scintigraphic localization of even smallest extra-adrenal tumors was successful in all cases. In most cases we were dealing with benign pheochromocytomas, but also a ganglioneuroma and metastases of a malignant pheochromocytoma could be revealed. Scintigraphy is a reliable technique for tracing pheochromocytomas and catecholamine-producing ganglioneuromas. It seems to be superior to other non-invasive techniques, furthermore, invasive techniques bearing higher risks may be suspended.
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PMID:[Scintigraphic diagnosis of multiple pheochromocytomas in childhood with 131I-m-benzylguanidine]. 651 38

A case of primary ganglioneuroblastoma located in the anterior mediastinum is described. The tumour was found incidentally at autopsy in a 61-year-old woman who died of coronary artery thrombosis. There was no evidence of metastases. Histologically the tumour was a ganglioneuroblastoma of the diffuse type. There was no pure neuroblastomatous tissue present and maturation to ganglioneuroma was not observed. Regressive changes like fibrosis and calcification were present. Review of the literature failed to reveal any recorded cases of ganglioneuroblastoma occurring in the anterior mediastinum. Although ganglioneuroblastoma occurs predominantly in children, 24 cases occurring in adults have been reported in the literature.
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PMID:Primary ganglioneuroblastoma of the anterior mediastinum in a 61-year-old woman. 666 13

The authors report the case of a child with retroperitoneal ganglioneuroma and cytodifferentiated skeletal metastases. The primary tumor was surgically resected, and the child is alive and well 2 years later without additional therapy. This rarely documented phenomenon can be explained by spontaneous cytomaturation within both primary and metastatic tumor.
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PMID:Ganglioneuroma presenting with differentiated skeletal metastases. Report of a case. 672 50

At Cincinnati Childrens Hospital Medical Center, 19 cases of composite ganglioneuroblastoma (CGNB) comprise one third of the cases of neuroblastoma diagnosed after 1 year of age. We have not identified this condition in an infant. Usually located in the mediastinum, CGNB is typically resectable and has a favorable prognosis, but retains potential to metastasize until nests of neuroblasts have disappeared. Prognosis is independent of proportion of ganglioneuroma or neuroblastoma and is closely related to stage. The interval between diagnosis and death in fatal CGNB was typically prolonged. Survival of older children with poorly differentiated neuroblastoma was rare and neuromatous maturation was absent or trivial in unresected primary tumors studied at the time of autopsy. We conclude that favorable outcome in neuroblastoma diagnosed after 1 year of age is closely related to histological maturation as herein defined.
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PMID:Composite ganglioneuroblastoma. An assessment of the significance of histological maturation in neuroblastoma diagnosed beyond infancy. 689 29

Neuroblastomas are malignant tumors derived embryonically from the neural crest. Biological diagnosis relies on assay of urinary excretion of homovanillic acid (HVA), vanillylmandelic acid (VMA), and dopamine (DA). Spontaneous regression of these neoplasms has been reported by numerous investigators. The authors report the case of a child with neuroblastoma that illustrates the relationship between catecholamine metabolites and tumor maturation. At 1 month of age, this infant presented an adrenal neuroblastoma with multiple metastases (stage IV); the initial histological diagnosis based on examination of cutaneous metastases was neuroblastoma. At the age of 6 months, after chemotherapy, the primary tumor was resected; hepatic metastases were discovered at laparotomy. The histological diagnosis for all lesions was highly differentiated, mature ganglioneuroma-like tissue. The main biochemical abnormality at the time of diagnosis was an elevation in normetanephrine (NMN). HVA was only slightly increased but rose progressively during chemotherapy; it dropped back to normal levels after the sixth course. This case illustrates the potential benefits of separate assays of urinary methylated catecholamine metabolites for biochemical diagnosis and therapeutic management of neuroblastoma in addition to assays of HVA, VMA, and DA. Case findings suggest existence of a transformation process with maturation of the tumor involving enzymatic regulation and expression of MAO.
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PMID:Urinary excretion of methylated catecholamine metabolites in a child with neuroblastoma maturing into ganglioneuroma. 749 13

We reviewed the findings of adrenocortical scintigraphy with 131I-6-beta-iodomethyl-19-norcholesterol (NCL-6-131I) of 39 patients to clarify its role in the evaluation of unilateral adrenal or juxtaadrenal masses incidentally discovered by CT, ultrasonography or plain radiography. Twenty-seven benign adrenal masses showed various scintigraphic findings (hot nodule: 12 silent adenomas, warm nodule: one solid mass, normal appearance: one cyst and 2 solid masses, diffuse decrease: each one; solid mass, myelolipoma, ganglioneuroma and calcified adrenal and partial or complete defect: each one; solid mass, myelolipoma and ganglioneuroma and 2 cysts and 2 pheochromocytomas); while a partial or complete defect was shown in a nonfunctioning carcinoma and 3 metastases and a complete defect or inhomogeneous uptake without opposite adrenal visualization was shown in 2 patients with cortisol-producing carcinoma. Therefore a hot nodule and an inhomogeneous uptake or complete defect with nonvisualization of the opposite adrenal are specific to a benign tumor and a cortisol-producing carcinoma, respectively. The impaired tumor uptake of NCL-6-131I is a nonspecific finding. The scintigraphic findings of juxtaadrenal masses were normal in 4 and deviated adrenals in 2. Thus adrenocortical scintigraphy can identify silent adenomas and cortisol-producing carcinomas among the adrenal masses and may help to differentiate juxtaadrenal from adrenal masses.
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PMID:The role of adrenocortical scintigraphy in the evaluation of unilateral incidentally discovered adrenal and juxtaadrenal masses. 821 90


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