Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three spindle cell neoplasms were encountered in a series of 46 FNA of the adrenal performed between 1984 and 1991. These neoplasms included a recurrent undifferentiated adrenal cortical carcinoma (ACC) with a predominant spindle cell pattern, a pheochromocytoma (PC), and a metastatic desmoplastic malignant melanoma (DMM). Cytologically, the ACC was characterized by the presence of numerous microtissue fragments composed of spindle-shaped malignant cells with oval to spindle-shaped nuclei, one or more nucleoli, and bipolar cytoplasmic processes. In some areas the tumor cells were dissected by vascular channels. The background contained abundant metachromatic stroma as well as individually scattered tumor cells. The PC was composed predominantly of loosely cohesive spindle-shaped cells along with more polygonal shaped cells with delicate faintly staining granular cytoplasm. The tumor cells exhibited mild anisonucleosis. The tumor fragments were well vascularized by arborizing delicate capillary channels. The DMM was composed of microtissue fragments, interlacing fascicles and loose aggregates of spindle-shaped malignant cells with hyperchromatic nuclei, small nucleoli, and an absence of cytoplasmic pigment. In each case ancillary studies including immunocytochemistry and electron microscopy (EM) were helpful in the differential diagnosis. The ACC was negative for cytokeratins, neuron-specific enolase (NSE), and muscle-specific actin (HHF), but displayed strong positivity for vimentin as well as characteristic whorls of smooth endoplasmic reticulum by EM. The PC was positive for NSE and chromogranin with no EM performed. The DMM stained for S-100 and vimentin but was negative for HMB-45, cytokeratin, and HHF. EM examination revealed rare atypical premelanosomes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Fine-needle aspiration cytology of spindle cell neoplasms of the adrenal gland. 160 81

Neuron-specific enolase (NSE) is the isoform of enolase, a glycolytic enzyme found in the neuroendocrine system. Neuropeptide Y (NPY) is a peptide recently discovered in the peripheral and central nervous systems. Serum NSE and plasma NPY levels have been reported to be increased in some patients with pheochromocytoma. The authors evaluated whether the measurement of these molecules could help to discriminate between benign and malignant forms of pheochromocytoma. The NSE levels were normal in all patients with benign pheochromocytoma (n = 13) and elevated in one half of those with malignant pheochromocytoma (n = 13). Plasma NPY levels were on the average significantly higher in the malignant (177.1 +/- 38.9 pmol/l, n = 16) than in the benign forms of the disease (15.7 +/- 389 pmol/l, n = 24). However, there was no difference in the percentage of patients with elevated NPY levels. These results show that determination of serum NSE may be useful for distinguishing between malignant and benign pheochromocytoma; the measurement of plasma NPY is not useful for differentiating the two kinds of tumors.
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PMID:Neuropeptide Y and neuron-specific enolase levels in benign and malignant pheochromocytomas. 220 39

A recurrent adrenal pheochromocytoma is reported in an 18-year-old woman with a family history of multiple endocrine neoplasia type IIA. The tumor was noteworthy for the presence of numerous fine intracytoplasmic lipid droplets confirmed by fat stain. Immunohistochemical studies revealed cytoplasmic positivity for chromogranin and neuron-specific enolase typical of pheochromocytoma. Electron microscopy demonstrated dense-core neurosecretory granules, in addition to the cytoplasmic lipid vacuoles. The presence of lipid within an adrenal pheochromocytoma is rare and, to our knowledge, has been documented in only one previous case report in the literature.
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PMID:Lipid degeneration in a pheochromocytoma histologically mimicking an adrenal cortical tumor. 237 65

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).
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PMID:Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. 257 47

A 58-year-old woman presented an unusual variant of bronchial carcinoid. A tumor measuring 20 x 25 mm was recognized upon gross examination in the upper lobe of the right lung. Microscopically, the tumor consisted of large polyhedral cells with a pseudoglandular arrangement similar to pheochromocytoma cells. Immunohistochemically, the tumor cells contained serotonin, S-100 protein and neuron-specific enolase. Thus, we consider this neoplasm to be a large cell variant of bronchial carcinoid which, to our knowledge, has not been described in the literature.
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PMID:An unusual case of bronchial carcinoid predominantly consisting of large cells. 260 43

Two cases of primary pheochromocytoma of the urinary bladder are reported. Ultrastructural demonstration of neuroendocrine granules and immunoreactivity for neuron-specific enolase and synoptophysin, and the absence of cytokeratin, an epithelial marker, are useful features for distinguishing pheochromocytomas from carcinomas of the bladder.
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PMID:Pheochromocytoma of the urinary bladder. Report of 2 cases with ultrastructural and immunohistochemical analyses. 266 23

Fine-needle aspiration (FNA) cytologic and immunocytochemical findings of a rare combined pheochromocytoma-ganglioneuroma developing in a 48-yr-old Japanese man in the organ of Zuckerkandl are described. This is the first report of a combined pheochromocytoma-ganglioneuroma of the organ of Zuckerkandl. FNA cytology showed typical cytologic findings of these two components similar to those described individually in fine-needle aspirates of these neoplasms. The neoplastic cells showed positive reactions for vasoactive intestinal polypeptide, neuron-specific enolase, and S-100.
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PMID:Fine-needle aspiration cytology of pheochromocytoma-ganglioneuroma of the organ of Zuckerkandl. 272 53

We have identified a new subline of PC12 pheochromocytoma cells (PC12D cells) in which neurites are extended within 24 hr in response to cAMP-enhancing reagents as well as in response to nerve growth factor (NGF), but not in response to epidermal growth factor or phorbol diester. Anti-NGF antiserum did not affect forskolin (FRK)-induced neuritic recruitment. FRK-induced neurites exhibited growth cones and contained secretion granules and many parallel arrays of microtubules as was the case with NGF-induced neurites. FRK, but not NGF, increased the levels of intracellular cAMP and activated adenylate cyclase in the membrane fraction. Both NGF and FRK enhanced the activities of tyrosine hydroxylase (TH), acetylcholinesterase (AchE), and ornithine decarboxylase (ODC), but not the levels of neuron-specific enolase. Enhanced levels of intracellular cAMP mimicked the effects of NGF on neuritic growth, TH, AchE, and ODC activities in PC12D cells, even though NGF does not act through elevation of levels of cAMP.
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PMID:Neuritic growth from a new subline of PC12 pheochromocytoma cells: cyclic AMP mimics the action of nerve growth factor. 303 56

Calmodulin is present in higher concentrations in brain tissues. The content rapidly increased during the 2nd postnatal week in rat brain. Although the protein is ubiquitous in all eukaryotic cells, immunohistochemical studies have revealed that calmodulin is mainly localized in the neurons, exhibiting a similar distribution to that of gamma-type neuron-specific enolase. In the mouse retina, both calmodulin and gamma-enolase were found to be localized in optic nerves, ganglion cells, and inner and outer plexiform layers. The development study showed that gamma-enolase increased in the 2nd postnatal week and that the levels of calmodulin did not significantly change in that stage. In the mouse retina with an inherited retinal dysplasia (C3H), in which all the photoreceptor cells degenerate during the 2nd and 3rd postnatal weeks, calmodulin-specific staining decreased in the residual layers. Calmodulin is also enriched in mammalian testes. In the mouse testis, levels of calmodulin were high in the spermatocytes and in the spermatids, as compared to the level in spermatozoa. This suggests that the large amount of calmodulin in the testis may be associated with miotic divisions and/or spermatogenesis. Immunocytochemical staining of calmodulin in C6 glioma cells and PC12 pheochromocytoma cells showed a high level of calmodulin to be localized on the half spindles between poles and chromosomes in mitotic cells. The protein was also shown to be localized on fibrous structures in the interphase of those cells.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Calmodulin and neuron: immunohistochemical studies]. 307 1

A 38-year-old woman was operated on to remove a large tumor that replaced the left adrenal gland. The tumor was encapsulated and showed small areas typical of pheochromocytoma, and spindle cell or undifferentiated round cell sarcoma in most areas. Metastases of primitive round cell appearance were operated from the abdominal cavity and abdominal wall shortly after the initial surgery. Eighteen months after the first operation, the patient was alive with metastases in liver and retroperitoneal space. The pheochromocytomalike component showed a typical ultrastructural and immunohistochemical profile of pheochromocytoma and was positive for neurofilaments, synaptophysin, neuron-specific enolase, and S-100 protein in the sustentacular cells. The sarcomatous areas showed fibroblastoid spindle cells that were often surrounded by a basal lamina. Immunohistochemistry revealed S-100 protein positivity in many spindle cells, but markers of pheochromocytoma or epithelial differentiation were absent. The metastases lacked all markers except for vimentin, and the cells were undifferentiated by electron microscopy. These findings suggest that the neoplasm was a compound tumor with a typical pheochromocytoma component and a sarcoma resembling a malignant schwannoma. Neoplastic proliferation of the S-100 protein-positive Schwann-cell-like sustentacular cells of the pheochromocytoma would be an explanation for the genesis of this sarcoma associated with pheochromocytoma.
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PMID:Pheochromocytoma combined with malignant schwannoma: unusual neoplasm of the adrenal medulla. 319 94


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