Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenal incidentalomas are a heterogeneous group of pathological entities, including benign or malignant adrenocortical or medullary tumors, hormonally active or inactive lesions, which are identified incidentally during the examination of nonadrenal-related abdominal complaints. About 1.5% to 23% of adrenal incidentalomas are pheochromocytomas. Composite pheochromocytoma is a rare tumour of adrenal medulla with divergente clinical course. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether pheochromocytoma and nonpheochromocytoma components show the same embryologic origin. Nonpheochromocytoma components found in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The biologic behavior of composite pheochromocytomas may be as difficult to predict as more traditional pheochromocytomas; based on the number of cases reported to date the presence of areas resembling ganglioneuroblastoma or neuroblastoma does not necessary indicate a poor prognosis. Some may behave in a malignant fashion with metastasis by a component of the tumour which has neural features. Pheochromocytomas and paragangliomas are well-defined entities. Some of their nonsporadic associations and unusual morphological appearances are not universally appreciated. We report on a rare association of left adrenal CP, with typical right adrenal phochromocytoma and retroperitoneal paraganglioma, and a review of literature. We analyzed the clinical and immunohistochemical features in a 24-year-old woman with composite pheochromocytoma localized in the left adrenal gland and associated with blood pressure of 200/140 mmHg. Abdominal computed tomography and 131-J MIBG revealed a 65 x 60 mm mass in the right adrenal gland, but no revealed 45 x 40 mm retroperitoneal mass and 20 x 20 mm mass in the left adrenal region. Serum and urinary adrenaline levels were high, and catecholamine levels in the blood sample of the selective adrenal vein, were also high. Bilateral adrenalectomy and retroperitoneal mass were surgically removed without complications. Clinical symptoms were absent 6 years after surgery. After surgery the patient gave birth to two healthy babies. Immunohistochemical analyses revealed that tumour cells of right adrenal pheochromocytoma and retroperitoneal paraganglioma were strongly positive for neurone specific enolase, synaptophisin and chromogranin A. The left adrenal tumour showed pheochromocytoma, ganglioneuroma and neuroblastoma components. Immunoreactivity of this tumour added several features to the wide immunohistochemical spectrum. This case demonstrates the indolent behavior of sporadic-type CP and retroperitoneal paraganglioma in an adult patient. Unusual morphological features of CP occur in a substantial number of cases and may cause diagnostic problems.
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PMID:[Pheochromocytomas as adrenal gland incidentalomas]. 1258 98

The neuroendocrine secretory protein chromogranin A (CgA) is a precursor for various biologically active peptides. Several single and paired basic residues are present within its primary amino acid sequence comprising cleavage sites for prohormone convertases. In this study, SH-SY5Y human neuroblastoma cells were stably transfected with the prohormone convertase PC2 to analyse the proteolytic processing of endogenous chromogranin A and, in particular, the formation of the chromogranin-A-derived peptide GE-25. Our analyses revealed a significant change in the pattern of proteolytic conversion of chromogranin A in cells expressing PC2. Mock-transfected control cells contained mainly the intact chromogranin A molecule and hardly any shorter products were found. On the other hand, PC2-transfected cells showed extensive processing of chromogranin A, resulting in significantly lower amounts of the intact precursor and especially high levels of the free peptide GE-25.
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PMID:Proteolytic processing of chromogranin A by the prohormone convertase PC2. 1260 57

A 2-year-old Labrador Retriever developed atrophy of the right temporal muscle, subsequently showed generalized seizure and died 2 months after the clinical onset. Postmortem examination revealed the tumor masses in the right mandibulopharyngeal area, nasopharynx and intracranial space. Histopathologically, these tumor masses were composed of small round neoplastic cells and neuropil-like stroma separated by fibrovascular septa. In the neoplastic masses, small neoplastic cells with round to oval hyperchromatic nuclei and scanty cytoplasm predominated, and angulated neoplastic cells with larger nuclei and moderate cytoplasm were scattered. Immunohistochemically, neoplastic cells were positive for neuron specific enorase, neurofilament protein, chromogranin A, synaptophysin and tyrosine hydroxylase. Based on these findings, this case was diagnosed as peripheral neuroblastoma, presumably originated from the sympathetic ganglion, maybe right craninal cervical ganglion.
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PMID:Peripheral neuroblastoma in a young labrador retriever. 1265 27

Malignant rhabdoid tumors (MRT) show a multiphenotypic diversity, including a neural phenotype. To elucidate the difference in neural characteristics between MRT and neuroblastoma, we examined the expression of synapsin I, neuron-restrictive silencer factor (NRSF), neurofilament medium-size (NF-M) and chromogranin A (CGA) in five MRT cell lines (TM87-16, STM91-01, TTC549, TTC642 and YAM-RTK1) and five neuroblastoma cell lines under differentiation-induction with 12-O-tetradecanoylphorbol-13-acetate (TPA). Our results showed TM87-16 and TTC642 cells, expressed synapsin I and NF-M before TPA induction, had a neural phenotype. After differentiation-induction, only TM87-16 cells expressed CGA. Among all neuroblastoma cells, expression of NF-M and CGA was stable at a high level throughout TPA-induced differentiation. In TM87-16 and TTC642 MRT cells, synapsin I mRNA promptly increased after TPA differentiation, with the peak level at 6 h, and thereafter, synapsin I mRNA rapidly decreased in a time-dependent manner. The decreased expression of synapsin I correlated with an increased expression of NRSF during differentiation-induction. In contrast, in some neuroblastoma cells, a significant up-regulation of synapsin I was observed concurrently with a down-regulation of NRSF. The inverse relationship between NRSF and synapsin I expression in TM87-16 and TTC642 MRT cells was opposite to that of neuroblastoma cells. Our results showed that the neural characteristics of these MRT cells are fairly distinct from those of neuroblastoma cells. These MRT cells appeared to have only limited capability for neural differentiation, and were still in an extremely early stage of neural differentiation.
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PMID:Malignant rhabdoid tumor shows a unique neural differentiation as distinct from neuroblastoma. 1270 72

Central nervous system (CNS) neuroblastoma/ganglioneuroblastoma is one of the embryonal tumors with neuronal differentiation found in young adults, but it is most common in children, especially in those below the age of 5 years, whereas extraventricular neurocytoma, a rare neuroepithelial tumor with neuronal differentiation, mostly affects young adults. Here we present a rare case of cerebral ganglioneuronal tumor that occurred in a 32-year-old woman. The patient suffered from tonic convulsion, and computed tomography demonstrated a well-demarcated, round tumor 3.3 cm in size with marked calcification in the right parietal lobe. Histological analysis revealed diffuse infiltration of small, round cells with scattered large ganglion-like cells. Immunohistochemically, the tumor cells did not react with any neuronal molecules, except for chromogranin A in ganglion-like large tumor cells, but electron microscopy demonstrated the presence of synapse-like nerve terminal structures without mature postsynaptic density, suggesting the presence of neoplastic tumor components with neuronal differentiation; thus, this tumor was diagnosed as CNS ganglioneuroblastoma with possible low-grade malignancy because the Mib-1 labeling index was less than 3%-4%. Here we discuss the histological entity of cerebral ganglioneuronal tumors, including extraventricular neurocytoma.
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PMID:A case of cerebral ganglioneuronal tumor in the parietal lobe of an adult. 1841 66

A peculiar adrenal tumor was analyzed using immunohistochemistry, electron microscopy, and fluorescence in situ hybridization (FISH) with multiple bacterial artificial chromosome (BAC) probes. The patient was a 34-year-old woman with a mass above the left kidney and multiple metastases. Her serum and urine dopamine level were elevated, and a diagnosis of malignant pheochromocytoma was made. The patient died approximately 3 years after her first visit. On post-mortem an adrenal tumor composed of small round cells forming Homer Wright rosette-like structures, a feature rarely observed in pheochromocytoma, was found. Immunohistochemistry was positive for chromogranin A and synaptophysin, and negative for cytokeratin, vimentin and neurofilaments. Because these results did not rule out a diagnosis of neuroblastoma, the tumor was further characterized on FISH with multiple BAC probes for loci known to be altered in neuroblastoma or pheochromocytoma, according to information in the literature that was for the most part obtained using comparative genomic hybridization. FISH demonstrated loss of heterozygosity at 11p, and gains at 16p, 19p, and 19q, a profile that favored a diagnosis of malignant pheochromocytoma over neuroblastoma. This case demonstrates that repeating FISH is useful for differential diagnosis.
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PMID:Malignant pheochromocytoma in a young adult forming the structure simulating Homer Wright rosette: differentiation from neuroblastoma on repeating fluorescence in situ hybridization. 1870 73

To elucidate more precisely the biological characteristics of neuroblastomas, we examined four human neuroblastomas heterotransplanted into athymic nude mice NB-39 (undifferentiated type), NB-45 (poorly differentiated type with undifferentiated component), NB-52 (poorly differentiated type), and NB-726 (differentiating type) by electron microscopy, immunohistochemistry, and radioimmunoassay for the peptides in tumors. Ultrastructurally, NB-45, NB-52, and NB-726 contained more numerous and variously sized neurosecretory granules than did NB-39. Immunohistochemistry revealed neurofilament proteins, tyrosine hydroxylase, neuropeptide Y (NPY), and chromogranin A-positive cells in the four tumors in the following order of frequency: NB-726, NB-45, NB-52, and NB-39. NB-726, NB-45, and NB-52, but not NB-39, contained galanin-positive tumor cells. NB-45 and NB-726 harbored a few positive cells for calcitonin gene-related peptide. Furthermore, NB-726 exhibited positivity to leu-enkephalin, met-enkephalin, vasoactive intestinal peptide (VIP), and serotonin. Radioimmunoassay substantiated the results of immunohistochemistry, showing NPY in all tumors and either galanin or VIP in three tumors, excepting NB-39. Average doubling time of the tumor was as follows: 2 days in NB-39, 10 days in NB-45, 22 days in NB-52, and 45 days in NB-726. These results indicate that human neuroblastoma cells have different biological characteristics and reduced growth rate with differentiation in terms of ultrastructure and of peptide production abilities.
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PMID:Distinct morphological and immunohistochemical features and different growth rates among four human neuroblastomas heterotransplanted into nude mice. 1880 41

Neuroblastoma is the most common childhood cancer, which arises from sympathetic neural precursors. Because the prognosis of advanced neuroblastoma is known to be poor, developments of new anti-cancer drugs are desperately needed. For screening of therapeutic drugs for neuroblastoma, genetically engineered animal models would be useful. In an attempt to obtain transgenic mice carrying simian virus 40 T-antigen gene under control of tetracycline responsive elements with cytomegalovirus promoter, we found one line of mice exhibiting bilateral adrenal tumors by leakage expression of T-antigen in adrenal gland. These adrenal tumors contained small round tumor cells with increased N/C ratio, showing chromogranin A and neuron specific enolase-like immunoreactivity. By electron microscopy, tumor cells containing neuritic processes with synaptic vesicles surrounding them were observed. The plasma levels of dopamine were significantly elevated in these transgenic mice. MYCN expression levels were significantly elevated in these tumors. These findings indicated that the adrenal tumor was a neuroblastoma. This mouse model would be a useful tool for development of chemotherapeutic drugs and understanding the etiology of neuroblastoma.
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PMID:Establishment of a novel neuroblastoma mouse model. 1902 Jul 52

Neuroblastoma is one of the most common solid tumors occurring in childhood. Bone marrow evaluation is an important part of clinical staging in neuroblastoma patients. In view of the difficulty of detecting neuroblastoma cells with conventional bone marrow aspirate smears and biopsy (BMB) and specimens in cases in which metastasis is not prominent, we propose the use of immunohistochemistry (IHC) as a potential diagnostic tool. We examined 116 BMB and 115 bone marrow clot (BMC) specimens from 60 newly diagnosed neuroblastoma patients for tumor cells. Neuroendocrine IHC markers, such as CD56, chromogranin A, and synaptophysin were applied to diagnose neuroblastoma. Eighteen out of the 60 patients (25.4%) displayed BM metastasis, as observed with conventional hematoxylin and eosin staining. IHC staining of BMC sections was generally more sensitive than that of BMB sections for tumor cell detection. We detected tumors in 5 and 7 additional hematoxylin and eosin-negative BMB and BMC sections, respectively, using CD56. Overall, CD56 or a combination of CD56 and chromogranin A was effective in detecting neuroblastoma cells. IHC analysis of BMB and BMC sections is warranted as a routine component of the diagnostic work-up of neuroblastoma to overcome discrepancies between routine smears and IHC stains.
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PMID:Detection of bone marrow metastases of neuroblastoma with immunohistochemical staining of CD56, chromogranin A, and synaptophysin. 2021 6

A subcutaneous mass arising in the right gluteal area of an 11-year-old female shih tzu dog was surgically excised. Histologically, the mass was composed of small round or ovoid neoplastic cells that were arranged in nests of various sizes. The neoplastic cells generally had hyperchromatic nuclei and scanty eosinophilic cytoplasm, and were surrounded by a pale pink fibrillar area. Immunohistochemically, the neoplastic cells were positive for vimentin, S-100 protein, neurone-specific enolase and synaptophysin, but negative for cytokeratin, neurofilament protein, glial fibrillary acidic protein and chromogranin A. On ultrastructural observation, aggregates of thin cytoplasmic processes were frequently seen among the neoplastic cells. Based on these features, the tumour was diagnosed as a neuroblastoma. To the authors' knowledge, this is the first description of a neuroblastoma originating from the skin in an adult dog.
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PMID:Primary neuroblastoma in the skin of an adult shih tzu dog. 2023 May 84


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