Prognosis of malignant pheochromocytoma and paraganglioma (MAPP-Prono study): An ENS@T retrospective study
Journal of Clinical Endocrinology and Metabolism Feb 10, 2019
Hescot S, et al. - Authors investigated 169 subjects from 18 European centers in this retrospective multicentric study to assess prognostic parameters of overall survival in malignant pheochromocytoma and paraganglioma (MPP) cases. Following characterizations were presented in MPP cases: primary pheochromocytoma in 53% of cases, tumor or hormone-related symptoms in 57% or 58% of cases, positive plasma or urine hormones in 81% of candidates, identification of a mutation in SDHB in 42 % of cases. Bone (64%), lymph node (40%), lung (29%) and liver (26%) were the metastatic sites. They observed an association of better survival with head and neck paraganglioma, age <40 years, metanephrines <5-fold the upper limits of the normal range and low proliferative index. They concluded hypersecretion as an independent significant prognostic factor of worst overall survival.
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