Metastatic Phaeochromocytoma: Spinning Towards More Promising Treatment Options.

ABSTRACT: Phaeochromocytomas (PCC) and paragangliomas (PGL) are rare tumours arising from the chromaffin cells of the adrenal medulla (PCC) or the paraganglia located outside the adrenal gland (PGL). However, their incidence is likely to be underestimated; around 10% of all PCC/PGL are metastatic, with higher metastatic potential of PGLs compared to PCCs. If benign, surgery is the treatment of choice, but if metastatic, therapy is challenging. Here we review the currently existing therapy options for metastatic PCCs/PGLs including conventional chemotherapy (the original Averbuch scheme, but updated), radiopharmaceutical treatments (¹³¹I-MIBG, ⁹⁰Y- and ¹⁷⁷Lu-DOTATATE) and novel targeted therapies (anti-angiogenic tyrosine kinase inhibitors and mTORC1 inhibitors), emph

SUBMITTER: Nolting S 

PROVIDER: S-EPMC7443617 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature