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

Five children with neural crest tumors (two ganglioneuromas, one ganglioneuroblastoma, and two neuroblastomas) were investigated regarding neuropeptide Y-like immunoreactivity (NPY-LI) in tumor tissue and plasma at diagnosis and during surgery. Radioimmunoassay of extracted plasma revealed higher NPY-LI at diagnosis of neuroblastoma (640 and 230 pmol/L resp) than ganglioneuroblastoma or ganglioneuroma (74, 45, and 26 pmol/L resp). During surgery of neuroblastoma plasma NPY-LI increased two- to four-fold while no peroperative increase was seen in the other children. NPY-LI was considerably higher in neuroblastoma tissue (220 pmol/g and 144 pmol/g) than in ganglioneuroblastoma (40.2 pmol/g), ganglioneuroma (0.6 and 4.4 pmol/g), or healthy adrenal tissue (5.5 pmol/g). The highest NPY-LI concentration was found in neuroblastoma metastasis, 3,091 pmol/g. Gel-permeation chromatography of a neuroblastoma tumor showed that a majority of NPY-LI was representing intact NPY (NPY 1-36) while metastasis and plasma from the same child mainly contained smaller immunoreactive fragments. High concentrations of systemic NPY in neuroblastoma patients are of tumoral origin. Plasma levels of NPY and its fragments can be useful in diagnosing and monitoring neuroblastoma, and for early detection of relapse or metastatic disease. A possible involvement of NPY in neuroblastoma tumor growth and spread deserves further investigation.
 ...
PMID:Neuropeptide Y in neuroblastoma: increased concentration in metastasis, release during surgery, and characterization of plasma and tumor extracts. 849 45

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.
 ...
PMID:Primary congenital cutaneous ganglioneuroma. 869 26

Neuroblastoma is a pediatric cancer for which a cure is elusive for most children with disseminated disease. Neuroblastomas possess receptors for somatostatin (SS). Some SS analogues can inhibit their proliferation. In addition, when SS analogues were used as agents for scintigraphy, neuroblastoma tumor sites can be localized with high efficiency. In this study, to better characterize the SS receptor subtype(s) (sst1-5) present in primary tumors and metastases of neuroblastoma, we show that: (1) The ligand 125I-Tyr11-SS-14 binding on membrane proteins from primary tumors and metastases of neuroblastoma cell line IGR-N-91 developed in nude mice shows similar values of Kd (in order of 0.1 nM) and Bmax (in order of fmol/mg) by filter-retention assay. These data are close to those measured on two other neuroblastoma cell lines: SK-N-SH and IGR-N-835 or to that measured on the rat cerebral cortex. (2) The IGR-N-91 sublines derived from primary tumor and metastases show one major complex of 57 kD by the chemical cross-linking assay using the ligands: 125I-SS-14 and 125I-BIM23014. One similar major complex of 57 kD was also detected in SK-N-SH and IGR-N-835 or in the cerebral cortex. (3) Addition of excess nonlabeled peptides selective for sst2 (BIM23014, BIM23060, BIM23068) suppressed the formation of the complex 57 kD whereas addition of BIM23052 or BIM23056 (sst5 and sst3 selective respectively) does not. This pharmacological profile corresponds to sst2. (4) Only RNA message of sst2 gene is detected in IGR-N-91 cells and its metastases derived sublines by reverse-transcription-polymerase chain reaction and Northern hybridization in keeping with the presence of sst2. (5) In human biopsies, the complex of 57 kD corresponding to sst2 is consistently detected in three samples of the histological subset of the disease: benign ganglioneuroma, ganglioneuroblastoma and immature neuroblastoma. Therefore, the sst2 should be considered as the primary target to develop more potent SS analogues for neuroblastoma therapy or/and scintigraphy.
 ...
PMID:Somatostatin-14 mainly binds the somatostatin receptor subtype 2 in human neuroblastoma tumors. 905 84

Ganglioneuroma is generally considered to be a benign tumor and potentially surgically curable. The authors present a case of a 21-year-old woman who underwent resection of a retroperitoneal ganglioneuroma and developed spinal neuroblastoma 11 years later. She has survived 10 more years with only recent development of metastases. To the authors' knowledge, this is the first report of malignant transformation of a ganglioneuroma into a neuroblastoma. Also, such long-term survival in an adult with spinal neuroblastoma has not been reported previously. This case raises the possibility of a dedifferentiating potential for ganglion cells in a ganglioneuroma or the presence of a long-term, quiescent form of neuroblastoma.
 ...
PMID:Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult. Case report. 945 44

In 1991, this prospectively designed study was started to assess the potentials of positron emission tomography with 18FDG in the diagnostic workup for the detection of lymph node metastases in testicular cancer, since there were no data available concerning this subject at this time. In 54 patients (27 patients with pure seminoma, 27 patients with non-seminomatous tumors) 18FDG-PET results were compared with the findings obtained with abdominal computed tomography, serum level of tumor markers (AFP, beta-HCG), and the histopathological findings after primary or post-chemotherapy retroperitoneal lymph node dissection. In 21 patients with pure seminoma (clinical stage I according to the Lugano classification) 18FDG-PET results were identical with those of the abdominal computed tomography, so PET does not add relevant informations in this group of patients. In 7 patients presenting with non-seminomatous testicular cancer (stage I), PET was not able to detect the existing micrometastases in 4 patients. In 1/7 case PET examination showed a suspicious focal lesion, this lymph node had 2 micrometastases within inflammatory changes. In 1/7 patient 18FDG-PET definitely revealed metastatic lesions, while the CT scans where judged to be unobtrusive and tumor marker levels were within the normal range. In the 4 patients with pure seminomas stage II B and II C (N = 6), that have undergone retroperitoneal lymph node dissection following chemotherapy, 18FDG-PET correctly predicted absence of tumor in 3 out of these 4, and in 1/4 patient the benign nature of a persistent large tumor after two cycles of polychemotherapy was correctly identified which eventually turned out to be a ganglioneuroma. This lesion falsely was classified as malignant tumor with abdominal computed tomography, and in 2/4 patients post-chemotherapy residual retroperitoneal lesions in the CT scans could not be assessed exactly whether or not malignant tumor was present. In 20 patients presenting with non-seminomatous testicular cancer (stage II and III) 18FDG-PET was able to demonstrate therapeutic effects of chemotherapy by showing decreasing tracer activity in those regions, that had hypermetabolic foci prior to chemotherapy. It became evident in testicular cancer that there is a single entity which is not characterized by increased glucose metabolism, the mature teratoma. In lesions detected by abdominal computed tomography which do not present increased 18FDG uptake, mature teratoma as well as scar/necrosis or rare other tumors with normal glucose metabolism can be supposed, but additional characteristics based on different 18FDG uptake were not observed. In 1/20 case post-chemotherapy PET scan detected a hypermetabolic lesion, which was suspicious for metastatic spread, but in the histopathological examination this lesion was identified as inflammatory tissue reaction. Based on the data reported here in 18FDG-PET cannot be considered a standard diagnostic tool in the staging examinations in testicular cancer. It is of clinical relevance in patients who present residual tumor after chemotherapy. In this situation 18FDG-PET is helpful in deciding whether or not a residual mass post-chemotherapy contains active tumor. 18FDG-PET can not replace retroperitoneal lymph node dissection for staging purposes.
 ...
PMID:[Positron emission tomography with [18 F]-2-fluoro-2-deoxy-D-glucose (18FDG-PET) in diagnosis of retroperitoneal lymph node metastases of testicular tumors]. 988 89

Recent reports have suggested that cyst formation accompanying astrocytomas in the central nervous system (CNS) is due to an edematous process caused by blood-brain barrier (BBB) disruption and not a result of necrosis. This study is performed to investigate whether the hypothesis of cyst formation based on BBB disruption also applies to various pathologically different intra-axial gliomatous tumors and metastases. By chemical analysis, using immunokinetic nephelometry, isoelectric focussing, cellulose acetate electrophoresis and a biuretic method, the concentrations of albumin, immunoglobulin G (IgG), IgA, alpha2-macroglobulin, IgM and total protein were measured and proportions of concentrations of these proteins were compared in cyst fluid, blood plasma and cerebrospinal fluid (CSF). Our data, based on the chemical analysis of cyst fluid and blood plasma of 37 patients, including 2 ependymomas (one cerebral; one thoracic), 3 oligodendrogliomas, 4 hemangioblastomas, 5 cerebellar astrocytomas and 1 cervical, 1 giant astrocytoma grade one, 1 gangliocytoma, 1 neuroblastoma and 19 metastases (five lung-; two renal-; three breast-; one melanoma-; one thyroid metastasis and seven metastases of unknown origin) present high protein concentrations in the cysts with a highly similar spectrum of proteins in the tumor cyst fluid and blood plasma, suggesting a BBB disruption followed by exudation of plasma proteins into the brain parenchyma with formation of edema and transition of edematous tissue into a cyst accompanying the tumor. Although histopathologically different types of tumor tissue are involved, data suggests that the pathogenesis of cysts accompanying gliomatous tumors and metastases in the CNS is based on BBB disruption and consequent edema, as is the case in the formation of cysts in anaplastic astrocytomas.
 ...
PMID:The pathogenesis of cysts accompanying intra-axial primary and metastatic tumors of the central nervous system. 1006 1

The authors analyse hormonal and morphological characteristics of adrenal incidentalomas, i.e. pathological adrenal masses accidentally found on CT scan performed due to extraadrenal causes of other causes of adrenal pathology. The group of patients was consisted by 42 patients at the age 24-79 years (27 females and 15 males). The most frequent clinical symptoms included arterial hypertension, diabetes mellitus and obesity. CT examinations revealed 36 cases of unilateral lesions (in 21 cases the lesions were localised on the right and in 15 cases on the left) and 6 bilateral lesions. The size of adrenal masses ranged from 7 mm to 12 cm. The CT examination helped in characterising myelolipomas in 3 cases, cysts in two cases, and pre-assuming malignity in 6 cases. Hormonal analyses have revealed primary aldosteronism in 2 cases, subclinical hypercortisolism in 1, steroid enzymopathy in 2 and secondary hyperaldosteronism in 2 patients. No patient had catecholamine overproduction. 19 patients were indicated for adrenalectomy with the following histological findings.: adenoma (n = 5), cyst (n = 2), myelolipoma (n = 3), carcinoma (n = 3), feochromocytoma, ganglioneuroma, metastases, lymphoma, sarcoidosis and pseudodrenal structure--Gravitz tumor (n = 1, respectively). The size of all neoplasms exceeded 3 cm, therefore the authors recommend adrenalectomy in incidentalomas with hormonal activity exceeding 3 cm in size. (Tab. 2, Fig. 1, Ref. 17.)
 ...
PMID:[Hormonal and morphologic characteristics of adrenal incidentalomas]. 1118 53

Background: Adrenal composite pheochromocytoma-ganglioneuroma is uncommon. The aim of this study is to investigate the characteristics of patients with this tumor. Methods: Histologic features of 46 pheochromocytomas diagnosed over a 28-year period (1971-1998) were reviewed. The clinical records, pathologic features, and ploidy patterns of patients with composite pheochromocytoma-ganglioneuroma were studied. Cases reported in the literature were also reviewed and compared with typical pheochromocytomas. Results: Four patients (two men; two women) with composite pheochromocytoma-ganglioneuromas were found. The tumors comprised 8.7% of pheochromocytomas. The two male patients with composite tumors were younger than the two female patients (age = 32, 52 vs 73, 75, respectively). The radiologic, gross, and microscopic appearances of the tumors were heterogeneous. One patient had malignant tumor with distant metastases. Flow cytometric analysis showed that the pheochromocytoma component was aneuploid and the ganglioneuroma portion was diploid. A review of the literature showed 31 adrenal composite pheochromocytoma-ganglioneuromas. Composite tumors were seen in older patients and were bigger than typical pheochromocytomas. Conclusion: Composite pheochromocytoma-ganglioneuromas had distinctive clinicopathologic features. Life-long clinical and biochemical follow-up of these patients is essential in view of the non-diploid DNA pattern.
 ...
PMID:Composite Pheochromocytoma-Ganglioneuroma of the Adrenal Gland: An Uncommon Entity with Distinctive Clinicopathologic Features. 1211 71

We investigated, morphologically and immunohistochemically, 74 medullary adrenal tumors, including 64 pheochromocytomas (14 malignant and 50 benign), 9 ganglioneuromas, and 1 malignant schwannoma. The tumors were detected in 2-year-old Wistar and Sprague-Dawley rats from carcinogenicity studies. Morphologically, benign pheochromocytomas were characterized by monomorphic, small, basophilic cells with almost absence of mitoses. Malignant pheochromocytomas presented a low grade of pleomorphism, higher rate of mitoses, necrosis, infiltrative growth and in 1 case metastases in the lung. Ganglioneuromas were characterized by ganglion and neuron-like cells embedded in an eosinophilic matrix containing neurites, Schwann cells, and scant fibrovascular elements. All pheochromocytomas were strongly immunoreactive for tyrosine hydroxylase (TH), the rate-limiting enzyme in catecholamine synthesis. Subpopulations of chromaffin cells expressed chromogranin A (CGA) positivity. Matrix and Schwann cells were positive for S-100 and for glial fibrillary acidic protein (GFAP). In focal areas of the tumors, ganglion cells and axons were positive for neurofilament proteins (NFP) and synaptophysin. Ganglion cells exhibited peripherin and beta-tubulin. Proliferative activity of the tumors was assessed by immunostaining the endogenous cell proliferation associated-antigen Ki-67 and the proliferating cell nuclear antigen (PCNA). As expected, cell proliferation indices were much higher in malignant pheochromocytomas than in benign, yet ganglioneuromas remained immunonegative. Considering that Ki-67 antigen is more specific for cell proliferation, it should be regarded as marker of choice for supporting the differential diagnosis between benign and malignant pheochromocytomas.
 ...
PMID:Pheochromocytomas and ganglioneuromas in the aging rats: morphological and immunohistochemical characterization. 1218 40

The aim of this retrospective study was to evaluate the diagnostic accuracy of nor-cholesterol and meta-iodobenzylguanidine radionuclide imaging in two separate groups of patients with adrenal tumours to characterize lesions as adenoma or pheochromocytoma. We studied 75 patients (22 male and 53 female, mean age 47 +/- 15 years) with hypersecreting (n = 32) or non-hypersecreting (n = 43) unilateral adrenal tumours detected by computerized tomography or magnetic resonance scans. 131I nor-cholesterol adrenal scintigraphy was performed in 41 patients. Meta-[131I]iodobenzylguanidine (131I-MIBG) imaging was acquired in the other 34 patients. Pathology examinations (n = 58) or computerized tomography follow-up studies (n = 17) were obtained. Adrenal lesions were represented by 44 adenomas, four cysts, one myelolipoma, one pseudotumour, one ganglioneuroma, 16 pheochromocytomas, three carcinomas, four metastases and one sarcoma. Radionuclide studies were qualitatively evaluated and the corresponding results were classified as true positive, true negative, false positive and false negative. Diagnostic sensitivity, specificity and accuracy as well as positive and negative predictive values were calculated. The diagnostic values of nor-cholesterol scintigraphy in identifying adrenal adenomas were sensitivity 100%, specificity 71%, accuracy 95%, positive predictive value 94% and negative predictive value 100%; of note, two false positive cases were observed represented by a pheochromocytoma and a myelolipoma. The diagnostic values of MIBG scintigraphy in recognizing pheochromocytoma were sensitivity 100%, specificity 95%, accuracy 97%, positive predictive value 94% and negative predictive value 100%; only one false positive case occurred consisting of a carcinoma. It is concluded that, in the large majority of cases, adrenal scintigraphy using nor-cholesterol or MIBG is able to characterize specific lesions such as adenoma and pheochromocytoma, respectively. These findings show relevant clinical impact, particularly in patients with non-hypersecreting adrenal lasions. Radiotracer selection depends on clinical patient history and department availability; since benign adenomas are the most common cause of non-hypersecreting tumours, nor-cholesterol should be the first choice followed by MIBG if nor-cholesterol shows normal images. However, rare as well as unusual findings may be observed; nor-cholesterol uptake may occasionally be also found in non-adenoma tumours such as myelolipoma and pheochromocytoma. Similarly, MIBG accumulation may occur not only in lesions arising from medullary chromaffin tissue, but also rarely in cortical adrenal carcinoma.
 ...
PMID:Diagnostic accuracy of radionuclide imaging using 131I nor-cholesterol or meta-iodobenzylguanidine in patients with hypersecreting or non-hypersecreting adrenal tumours. 1235 93


<< Previous 1 2 3 4 5 6 Next >>