2017
DOI: 10.3332/ecancer.2017.761
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Malignant extra-adrenal pelvic paraganglioma in a paediatric patient

Abstract: The extra-adrenal paraganglioma is a neoplasm originating in regional structures, uncommon in paediatrics. We report on a case of a 13-year-old patient who began with severe arterial hypertension, tachycardia, dilated cardiomyopathy and elevated levels of catecholamines in the blood and urine. The presence of a retrovesical pelvic mass in contact with the right vaginal dome was determined by imaging studies. A diagnosis of malignant extra-adrenal pelvic paraganglioma with lymph node metastases was reached thro… Show more

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Cited by 5 publications
(4 citation statements)
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“…The 2014 Endocrine Society clinical practice guidelines for pheochromocytoma and paraganglioma suggest that 10-17% of pheochromocytomas and paragangliomas are malignant, of which the proportion of paragangliomas is higher [5]. Malignant extra-adrenal paraganglioma is rare and reported to be less than 3%, of which nearly 75% are sporadic and 25% are hereditary [9]. Malignant tumors are mostly metastases or spread to organs without chromaffin tissue [10].…”
Section: Discussionmentioning
confidence: 99%
“…The 2014 Endocrine Society clinical practice guidelines for pheochromocytoma and paraganglioma suggest that 10-17% of pheochromocytomas and paragangliomas are malignant, of which the proportion of paragangliomas is higher [5]. Malignant extra-adrenal paraganglioma is rare and reported to be less than 3%, of which nearly 75% are sporadic and 25% are hereditary [9]. Malignant tumors are mostly metastases or spread to organs without chromaffin tissue [10].…”
Section: Discussionmentioning
confidence: 99%
“…In summary, our study improves upon previous reviews, which was dated and focalized mainly on bladder PGL . For example, Tsai et al includes one study dating back to 1911, and then eleven studies between 1989 and 2000 [17], while Beilan et al [85] included 80 studies between 1980 and 2012; therefore, we offer a more updated view for analyzing contemporary outcomes. Our study used a multitude of demographics to depict the disease process of pelvic and bladder PGL, including presenting signs and symptoms, tumor functionality and size, treatment modality, and outcomes.…”
Section: Discussionmentioning
confidence: 99%
“…In summary, our study improves upon previous reviews, which was dated and focalized mainly on bladder PGL . For example, Tsai et al includes one study dating back to 1911, and then eleven studies between 1989 and 2000 [17], while Beilan et al [85] included 80 studies between 1980 and 2012; therefore, we offer a more updated view for analysing contemporary outcomes. Our study used a multitude of demographics to depict the disease process of pelvic and bladder PGL, including presenting signs and symptoms, tumor functionality and size, treatment modality, and outcomes.…”
Section: Discussionmentioning
confidence: 99%