Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pheochromocytomas (PCCs) are rare catecholamine producing neuroendocrine tumors. The majority of these tumors (85 %) arise from the adrenal medulla. Those arising from the extra-adrenal neural ganglia are called paragangliomas (PGLs). Paroxysmal hypertension with sweating, headaches and palpitation are the usual presenting features of PCCs/ PGLs. Gene mutations are reported in 32-79 % of cases, making genetic screening mandatory in all the cases. The malignancy rates are 10-15 % for PCCs and 20-50 % for PGLs. Measurement of plasma or 24-hour urinary fractionated metanephrines is the best biochemical diagnostic test. Computed tomography or magnetic resonance imaging has high sensitivity (90-100 %) and reasonable specificity (70-90 %) for the anatomical localization. The functionality is assessed by different radionuclide imaging modalities such as metaiodobenzylguanidine (MIBG) scintigraphy, positron emission tomography or single photon emission computed tomography. The only modality of curative treatment is tumor excision. Proper peri-operative management improves the surgical outcomes. Annual follow up with clinical and biochemical assessment is recommended in all the cases after treatment. Children, pregnant women and older people have higher morbidity and mortality risk. De-bulking surgery, chemotherapy, radiotherapy, molecular agents like sunitinib and everolimus, radionuclide agents and different ablation procedures may be useful in the palliation of inoperable/metastatic disease. An update on the diagnostic evaluation and management of PCCs and PGLs is presented here.
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PMID:Diagnosis and management of pheochromocytoma: a practical guide to clinicians. 2479 93

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity that is manifested by characteristic magnetic resonance imaging (MRI) depictions of subcortical/cortical hyperintensities in the parieto-occipital lobes. Paroxysmal hypertension, headache, and palpitation are the most common clinical manifestations of pheochromocytoma, which are catecholamine-secreting enterochromaffin tumors. PRES is a rare complication of pheochromocytoma. Herein, we describe a 44-year-old woman who presented with postoperative confusion and headache. MRI images showed multiple asymmetrical hyperintensities with surrounding edema and contrast enhancement, predominantly in the right parietal lobe, left cerebellar hemisphere, and dentate nuclei, in favor of hemorrhagic metastases. The results of further investigations, including abdominopelvic computed tomography and the 24-hour urine test for metanephrine and normetanephrine, were in favor of a pheochromocytoma. The patient was scheduled for adrenalectomy and histopathologic examination of the tissue, which confirmed the diagnosis. Surprisingly, her symptoms and neuroimaging abnormalities improved significantly without any treatment during the follow-up period. Based on these findings, the diagnosis of PRES was considered, and the patient was followed. She was symptom-free at 3 years' follow-up. The literature contains only four case reports of PRES as a complication of pheochromocytoma; however, all these cases had bilateral symmetrical hemispheric involvement and occurred during childhood and adolescence.
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PMID:Atypical Posterior Reversible Encephalopathy Syndrome as the First Presentation of a Pheochromocytoma: A Case Report. 3328 Dec 66